Archive for category: Case Studies

PELVIC HEMATOMA: A RARE CAUSE OF BLADDER PERFORATION AND GROSS HEMATURIA

In urological practice, bladder perforations are often seen in the context of trauma or as a complication of surgery. Here we report the second case of bladder perforation caused by a large pelvic hematoma without direct injury to the bladder.The patient was a 46-year-old woman. She was admitted to the hospital with cardiogenic shock. One week after a percutaneous coronary angiography performed through the right femoral artery, she presented with gross hematuria. A CT scan of her abdomen and pelvis revealed a large right pelvic hematoma compressing the bladder and producing bilateral hydronephrosis. A suspected extraperitoneal perforation of the right bladder wall was later confirmed by cystoscopy and cystography. Although conservative management is the standard recommendation for extraperitoneal bladder perforations, we performed a combined transvesical/extravesical primary open repair in two layers, as conservative management failed in the only similar case that had been previously reported in the literature.

Authors: Marchant, Fernando; Ossandón, Enrique; Fleck, Daniela; Sepúlveda, Francisco; Mercado, Alejandro

Corresponding Author: Alejandro Mercado. University of Chile Clinical Hospital. Department of Urology. Avenida Santos Dumont 999, Piso 5 Sector A. Independencia, Santiago, Chile. Phone: +5629788503. E-mail: [email protected]

Introduction
In urological practice, bladder perforations are often seen in the context of trauma or as a complication of surgery1. Here we report the second case of bladder perforation caused by a large pelvic hematoma without direct injury to the bladder, resulting in gross hematuria.

Case report

A 46-year-old woman with history of obesity, diabetes mellitus and chronic arterial hypertension presented to her local hospital with progressive dyspnea. Initial evaluation with a chest x-ray revealed bilateral pleural effusion and basal consolidation in the right lung. She developed respiratory, renal and heart failure, requiring management in an intensive care unit. Emergency percutaneous coronary angiography performed through the right femoral artery ruled out any coronary lesions, but the ventriculography revealed a dilated cardiopathy with an ejection fraction of 25%. After stabilization she was transferred to the intensive cardiology unit of our institution for further management. One week after the coronary angiography, a urologican consultation was requested as the patient developed gross hematuria. On physical examination she had a normal blood pressure and heart rate, her abdomen was soft and non tender, with no palpable masses, bruises or visible hematoma. In the right groin she had the scar from the percutaneous access for coronary angiography, with no sign of any complications. An urinary catheter was in place, with gross hematuria and abundant clots in the urine collection bag. A gentle bladder wash was performed, producing several clots, but not being able to recover clear urine. A three-way urinary catheter with continuous saline irrigation was left in place and an abdominal and pelvis CT scan was requested. The images revealed a large right pelvic hematoma of 15 x 9 cm compressing and deviating the bladder to the left, with secondary moderate bilateral hydronephrosis (Fig. 1). Active bleeding was ruled out with CT angiography. An extraperitoneal perforation of 2 x 3 cm at the right bladder wall was suspected and later confirmed by cystoscopy and cystography (Fig. 2). The case was discussed at the clinical meeting of the urology department. Based on the failure of conservative management reported in the only similar case published in the literature by Lane et al. (2004) (2), we attempted a primary open repair. A midline infraumbilical abdomen incision was used to access the Retzius space. A longitudinal anterior cystotomy was performed and the perforation was identified at the right bladder wall, close to the trigone area where the balloon of the urethral catheter rested. As the perforation was difficult to reach transvesically, we decided to change to a combined intravesical/extravesical approach, for which it was necessary to free the right bladder wall from, and remove, the pelvic hematoma (Fig. 3). The borders of the perforation were resected and the repair was performed in two layers. A suprapubic catheter and a perivesical drain were left in place upon closure of the bladder and abdomen. The patient did not develop any postoperative complications and was discharged a week after. Cystography performed 3 weeks after surgery showed integrity of the bladder with no extravasation of contrast medium (Fig. 4). The cystostomy catheter was removed after 4 weeks and to the date the patient is voiding normally and is free of any bladder-related symptoms.

Discussion
Gross hematuria in hospitalized patients can be explained by several medical conditions. Generally is related to traumatic catheterization or instrumentation. Other causes include urinary tract infections, bladder or renal tumors, blood dyscrasias and chemotherapy (2). Here we report the second case of bladder perforation produced by a large pelvic hematoma resulting in hematuria. The first case was reported by Lane et al. (2004) (2).
The recommended standard treatment of uncomplicated extraperitoneal bladder perforations, when conditions are ideal, is conservative management with catheter drainage alone (1). However, some authors (3,4) have reported fewer complications, such as fistula, failure to heal, clot retention, and sepsis, with open repair (5% overall) versus conservative management (12% overall). For this reason, extraperitoneal perforations with any complicating features should be managed with primary open repair to prevent complications.
The similarity of this case to the one reported by Lane et al. (2004) (2) is impressive. Both patients had a percutaneous coronary angiography with access through the right femoral artery. In both cases the pelvic hematoma was on the right side, correlating to the site of arterial puncture. In both cases the perforation occurred close to the bladder neck, in the right bladder wall. We agree with Lane et al. (2004) (2) in that the most likely cause of bladder perforation was pressure necrosis that developed between the hematoma and the balloon of the Foley catheter. Considering the failure of conservative management reported in the case of Lane et al. (2004)(2), we aimed to attempt a primary open repair for two reasons: first, as the perforation occurred at the site where the balloon of the catheter rests, the same balloon could prevent the closure of the wound; second, leaving this patient with a communication between the bladder and hematoma, and with an indwelling catheter, carried a high risk of complications, as infection could easily lead to cardiovascular decompensation in this particular patient.
This report supports pelvic hematomas as cause of bladder perforation, probably secondary to compression of the bladder wall against the balloon of a Foley catheter. Here we also suggest that in this kind of extraperitoneal bladder perforations, primary open repair could be a better option than conservative management.

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Figure 1 Pelvis CT scan. Large (9 x 15 cm) right pelvic hematoma (*) compressing the bladder (B). The arrow points the site of perforation, in direct relation to the balloon of the Foley catheter (f).

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Figure 2 Preoperative cystography. Retroperitoneal extravasation (E) of contrast from the bladder (B), which is deviated to the left by the pelvic hematoma.

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Figure 3 Postoperative cystography (3 weeks after surgery). No contrast extravasation was observed from the bladder (B), now on a central position. Cystography was performed through the suprapubic catheter.

References
1. Morey AF, Rozanski TA. Bladder Injuries. In Wein AJ, Kavoussi LR, Novick AC, Partin AW, Peters CA. (Editors): Campbell-Walsh Urology, 9th Edition. Philadelphia, WB Saunders, 2007, vol 3, 2655-2658.
2. Lane BR, Moy L, Frenkl T, Hijaz A, Daneshgari F. Pelvic hematomas as a cause of bladder perforation and gross hematuria. Urology 2004; 64: 1030.e7-1030.e8.
3. Cass AS. Diagnostic studies in bladder rupture. Urol Clin North Am 1989; 16: 267-273.
4. Kotkin L, Koch MO. Morbidity associated with nonoperative management of extraperitoneal bladder injuries. J Trauma 1995; 38: 895-898.

 

Date added to bjui.org: 13/12/2012

DOI: 10.1002/BJUIw-2012-077-web

 

Papillary Renal Cell Carcinoma and Clear Cell Renal Cell Carcinoma Arising in a Single Kidney

We report a case of clear cell RCC and papillary RCC arising in the same kidney.

Authors: Tait, Laura; Coleman, Pamela; Ahaghotu, Chiledum
Corresponding Author: Tait, Laura Department of Surgery-Division of Urology, Howard University Hospital Washington, DC

 

Introduction
Renal cell carcinoma (RCC) is responsible for 80 percent of all primary renal neoplasms and approximately 65,000 Americans are diagnosed with RCC each year (1). Clear cell RCC comprises a majority of all RCC tumors diagnosed histologically. They arise from the proximal tubule and macroscopically appearsolid or cystic (2). Clear cell RCC occurs most commonly as a sporadic disease. Papillary RCC are diagnosed in 10 to 15 percent of patients with RCC (3, 4, 5, 6). As with clear cell cancers, papillary RCCs originate from the proximal tubule, but they are morphologically and genetically distinct malignancies. Papillary carcinomas are frequently multifocal and bilateral, and commonly present as small, early stage tumors (7). For localized disease, radical nephrectomy has been the most widely used approach and remains the preferred procedure for cases suggestive of renal malignancy. The diagnosis of clear cell RCC has been established by identifying distinct morphological and histologic features of this tumor following nephrectomy. These unique qualities include neoplastic cells with clear cytoplasm in an acinar growth pattern. These findings separate it from papillary RCC in which psammoma bodies and a strong positive staining for CK7 are characteristic. This case is unique in that there were no prior available reports describing two RCC tumors of separate origin found within a single kidney. We report a case of clear cell RCC and papillary RCC arising in the same kidney.

Case report
A 55-year-old African American male presented to his PCP with a two-week history of hematuria. The patient’s past medical history included hypertension, hepatitis c, and a hernia repair. He was referred to urology and a computed tomography scan demonstrateda complex heterogenous cyst within the right kidney with characteristics suggestive of malignancy and the presence of a left pelvic kidney. Right nephrectomy was performed. Two renal tumors were noted upon gross examination. The main tumor mass was a well-circumscribed, lobulated, homogeneous yellow tumor at the inferior pole of the kidney measuring 4.2×4.0x3.6 cm and demonstrated microscopic invasion of the renal sinus. The satellite tumor was found to be compressing the renal parenchyma at the inferior pole and was a 0.6 cm, firm, well-circumscribed nodule with homogeneous, tan/grey cut surface with characteristic hilar vessel wall invasion. Renal tumor tissues obtained at operation wasfixed in formalin and embedded in paraffin. The specimens were serially sectioned using three-micrometer thick cuts. The sections were stained with hematoxylin-eosin and immunohistochemical studies were undertaken. CK7 staining was performed with the satellite papillary renal cell carcinoma showing strong positive staining. CK7 staining was negative within the main tumor mass. The pathology results provide evidence that clear cell RCC and papillary RCC can simultaneously arise within a single kidney.

Pathology
Macroscopically, the kidney showed no gross abnormalities. The tumor measuring 4.2 x 4.0 x 3.6 cm and the tumor measuring 0.6 cm were observed in the inferior pole. The cut surface of the main tumor showed a yellowish color while the satellite tumor had a tan/grey cut surface appearance. At the posterior/inferior pole it was noted that the renal capsule was expanded by a gelatinous blood clot. The cut surface is shown (Fig. 1).

Microscopically, the main tumor located at the inferior pole showed a proliferation of neoplastic cells with clear cytoplasm and an acinar growth pattern. The nuclear characteristics were a Fuhrman nuclear grade 4 (Fig. 2). Given this tumor’s microscopic characteristics, a diagnosis of clear cell carcinoma was made.

The satellite tumor showed a proliferation of neoplastic cells with foamy macrophages and psammoma bodies (Fig. 3). This satellite tumor also demonstrated strong positive staining for CK7, a well-known tumor marker for papillary renal cell carcinoma. Based on the microscopic and immunohistologic findings, a diagnosis of papillary renal cell carcinoma was made.

Discussion
This case report describes a unique finding of clear cell and papillary RCC within a single kidney. The pathology of this conditionis described to illustrate how both tumors were morphologically different from one another. This particular anomaly has not been previously described in the literature.
The risk factors for RCC have been well-documented and include smoking, hypertension, occupational exposure to toxic compounds, obesity, acquired cystic disease of the kidney, analgesic abuse nephropathy, and genetic predisposition (8,9). These documented findings are similar to our case report in that the patient had a history of hypertension. The literature suggests that hypertension predisposes to the development of RCC. Although our patient had high blood pressure for many years, the incidence of RCC seems to be independent of anti-hypertensive medications. Therefore, hypertension may have played a role in the development of clear cell and papillary RCC seen in this patient.
Another risk factor our patient had for developing RCC waschronic hepatitis c infection. An epidemiologic study of over 67,000 patients found that chronic infection with the hepatitis C virus was associated with a significantly increased risk of RCC (10). This risk factor combined with hypertension may have put our patient at increased risk for RCC.
Although no clear cell RCC and papillary RCC have been found simultaneously before, a coexisting RCC can be identified in 10 to 32 percent of patients with oncocytoma (11). This evidence demonstrates that two unique RCC tumors can exist in a single kidney. Our findings of two RCC within the same kidney is consistent with past evidence of simultaneous tumors but are new in that none have reported clear cell and papillary RCC. Therefore, in our case, we were able to determine the possible risk factors predisposing our patient to RCC and report the new discovery of two RCC tumors within the same kidney.

Conclusions
We present a unique example of clear cell and papillary RCC within the same kidney. Histology of the tissues at the time of nephrectomy confirmed the diagnosis of two distinct renal tumors. Risk factors for RCC seen in this patient include hypertension and chronic hepatitis c. Although there are reports of oncomytoma and coexisting RCC, there have been no observed findings of clear cell and papillary RCC simultaneously.

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References
1. Siegel R, Naishadham D, Jemal A. Cancer statistics, 2012. CA Cancer J Clin 2012; 62:10.
2. Presti JC Jr, Rao PH, Chen Q, et al. Histopathological, cytogenetic, and molecular characterization of renal cortical tumors. Cancer Res 1991; 51:1544.
3. Tannenbaum M. Ultrastructural pathology of human renal cell tumors.PatholAnnu 1971; 6:249.
4. Thoenes W, Störkel S, Rumpelt HJ. Histopathology and classification of renal cell tumors (adenomas, oncocytomas and carcinomas).The basic cytological and histopathological elements and their use for diagnostics.Pathol Res Pract 1986; 181:125.
5. Störkel S, van den Berg E. Morphological classification of renal cancer. World J Urol 1995; 13:153.
6. Patard JJ, Leray E, Rioux-Leclercq N, et al. Prognostic value of histologic subtypes in renal cell carcinoma: a multicenter experience. J ClinOncol 2005; 23:2763.
7. Beck SD, Patel MI, Snyder ME, et al. Effect of papillary and chromophobe cell type on disease-free survival after nephrectomy for renal cell carcinoma. Ann SurgOncol 2004; 11:71.
8. Mandel JS, McLaughlin JK, Schlehofer B, et al. International renal-cell cancer study. IV. Occupation. Int J Cancer 1995; 61:601.
9. Wolk A, Gridley G, Niwa S, et al. International renal cell cancer study. VII. Role of diet. Int J Cancer 1996; 65:67.
10. Gordon SC, Moonka D, Brown KA, et al. Risk for renal cell carcinoma in chronic hepatitis C infection. Cancer Epidemiol Biomarkers Prev 2010; 19:1066.
11. Chao DH, Zisman A, Pantuck AJ, et al. Changing concepts in the management of renal oncocytoma. Urology 2002; 59:635.

 

Date added to bjui.org: 12/12/2012

DOI: 10.1002/BJUIw-2012-060-web

 

Massive urinary tract haemorrhage following bladder decompression by urethral catheterisation

The development of haematuria following bladder decompression is a well described phenomenon, but is usually transient, mild, and of little clinical consequence. We describe a case in which bladder decompression precipitated massive urinary tract haemorrhage requiring multiple blood transfusions and bilateral nephrostomy insertion

Authors: Spencer Chapman, Michael; Harber, Mark
Department of Nephrology, Royal Free Hampstead NHS Trust, London, UK
Corresponding Author: Spencer Chapman, Michael

 

Introduction
Urinary retention is a frequently encountered problem in everyday clinical practice. When chronic, the patient is able to urinate and may be asymptomatic, or may complain of lower urinary tract symptoms (LUTS) such as urinary frequency, urgency or hesitancy, poor urinary stream, post-micturition dribbling, nocturia or urinary incontinence. However the urinary tract is often subjected to high pressures which are required to allow voiding. Over time high pressures may damage the entire urinary tract: obstructive nephropathy resulting in renal failure and bladder changes such wall thickening, trabeculation, and loss of capillary and tissue integrity(1). The immediate management of urinary retention is urethral catheterisation to allow free drainage of urine from the bladder. The development of haematuria following bladder decompression is a well described phenomenon, but is usually transient, mild, and of little clinical consequence. We describe a case in which bladder decompression precipitated massive urinary tract haemorrhage requiring multiple blood transfusions and bilateral nephrostomy insertion.

Case report
A 58 year-old Caucasian man was transferred to our unit from a local hospital with massive urinary tract haemorrhage and renal failure.
He had presented to his GP five days previously complaining of dysuria. He was prescribed a course of antibiotics for a presumed urinary tract infection and blood taken for a full blood count and urea and electrolyte estimation. The results of his blood tests demonstrated a creatinine level of 958 μmol/l and haemoglobin of 5.2 g/dL. He was referred immediately to his local Accident & Emergency (A&E) department.
At his local A&E, he reported prostatic symptoms of urinary frequency, poor stream and nocturia. These had been present for the last twenty years but had worsened over the previous few weeks. The symptoms were accompanied by general lethargy, malaise and aching thoraco-lumbar back pain that radiated to his groin. In addition he had recently noticed bilateral numbness and dysaesthesiae in his fingertips. His past medical history was notable only for long-standing prostatic symptoms for which he had been investigated three years previously, at which time a serum prostate-specific antigen (PSA) level was measured at 5.4 μg/L. MRI of the prostate had showed an enlarged central lobe of the prostate and bladder changes consistent with chronic obstruction, but no evidence of malignancy (Figure 1a and Figure 1b). He had undergone urethroscopy and cystoscopy which were reportedly normal and he did not undergo any surgical intervention. He had been lost to follow up. There was no history of neurological disease. His medications included losartan, doxazosin and simvastatin. He reported no prescription or over-the-counter use of aspirin, clopidogrel or other anticoagulants. He had a 30 pack year smoking history, drank around 5 units of alcohol a week and did not use illicit drugs. He was afebrile and observations were within normal limits. General examination was reportedly unremarkable.
Initial blood tests showed a creatinine of 958 μmol/L, urea of 43.3 mmol/L, haemoglobin of 5.2 g/dL, corrected calcium of 2.06 mmol/L, phosphate of 3.29 mmol/L, and potassium of 5.8 mmol/L.
He was transfused and a urethral catheter was easily inserted which immediately drained 2.9L of clear urine. After a few hours the urine was noted to have become a pale red colour. Overnight this progressed to frank haematuria with clots. This persisted, and over the following 3 days he underwent bladder irrigation and required transfusion with 10 units of packed red blood cells, 7 units of fresh frozen plasma and 1 unit of platelets.
The patient’s haematuria and renal failure continued and on the fifth hospital day he was transferred to our unit. His haemoglobin was measured at 5.8 g/dL, urea 37.2 mmol/L, and creatinine 809 μmol/L. Bladder irrigation was continued and he was further transfused. CT of the abdomen and pelvis obtained with the administration of contrast material revealed hugely dilated renal pelvises bilaterally, measuring 10cm in maximal diameter on the right and 7cm on the left (Figure 2a). The catheterised bladder was diffusely thick-walled with a posterior diverticulum (Figure 2b). High attenuation material likely to represent haemorrhage was present throughout the bladder, ureters and renal pelvises. Both kidneys were hydronephrotic. No focal lesion or active bleeding was identified.
At this stage there was considerable discussion regarding the benefits of bilateral nephrostomy insertion to relieve back pressure on his kidneys. Renal failure was persisting with the need for dialysis under consideration, and imaging suggested obstruction at the level of the ureters due to insoluble blood clot. A consensus was reached that nephrostomy insertion would be likely to hasten renal recovery and potentially avoid the need for dialysis. On the eighth hospital day he underwent bilateral nephrostomy insertion. Serum creatinine levels fell and dialysis was not required.
Over the next two weeks he underwent bilateral antegrade stenting of his ureters, and by the twenty-fifth hospital day, both nephrostomies were removed. Shortly after he was discharged with a urethral catheter and right ureteric JJ stent in situ. His creatinine on discharge was 348 μmol/L. Two months later, at which time his creatinine was 241 μmol/L, he underwent successful transurethral resection of the prostate. Notably, no false passage was seen in the urethra. His LUTS were much improved and the urethral catheter was no longer required. He went on to have laparoscopic pyeloplasty of the right kidney and removal of the right JJ stent. Radioisotope renography with MAG-3 performed one month later demonstrated reasonable bladder emptying post-micturition, normal transit times, no evidence of obstruction, but ongoing dilatation of the right renal pelvis.

Discussion
Urinary retention is the inability to empty the bladder. When chronic, the patient is able to pass urine, but may experience voiding difficulties and bladder emptying is incomplete. The usual cause is bladder outlet obstruction, the aetiology of which may be enlargement of the prostate (benign or malignant), drugs (e.g. anticholinergics, antispasmodics), congenital deformities (e.g. meatal stenosis, posterior urethral valves) or urethral strictures (from trauma or infection). It largely affects men from middle-age onwards reflecting the increasing incidence of benign prostatic hypertrophy (BPH) in this group. Women are also affected however, around 50% of women are diagnosed with Fowler’s syndrome: a problem of inadequate urethral sphincter relaxation (2).
In order to facilitate emptying, the bladder is forced to hypertrophy so as to increase its contractile force. This results in bladder wall thickening, trabeculation and friability. It may also become increasingly sensitive (contributing to symptoms of urinary frequency, urgency and incontinence) and eventually become weakened thereby preventing complete bladder emptying. The structural changes within the bladder are thought to affect the integrity of capillaries and tissues within its wall.
Haematuria following bladder decompression by urethral catheterisation is well-recognized, and visible haematuria is estimated to occur in 2-16% of patients(3). However, though there are case reports of clinically significant haematuria, they are rare and we could find no reports in the literature of haemorrhage requiring multiple blood transfusions as in this case (4). Factors contributing to the severity of bleeding in this case include the significant structural damage to the urinary tract resulting from many years of urinary retention, and possible coagulopathy that is part of the “uraemic syndrome” associated with renal failure. Traditional teaching recommends a “clamping technique” to achieve step-wise gradual bladder decompression and supposedly reduce the risk of haematuria. Use of an IV giving set to achieve gradual decompression has also been advocated (5). However, these approaches are time consuming and presently there is scant evidence to support their use (8). We could find no randomized studies comparing gradual bladder decompression with quick, complete emptying, and other studies suggest that the risk of haematuria correlates with the severity of bladder wall damage prior to catheterisation, and not the rate of initial emptying (6). Furthermore, studies of changes in intravesical pressure following catheterisation for acute urinary retention show that the pressure drops to around 50% of the initial value after drainage of only 100mL (7)– hence clamping after 1000mL (a commonly adopted figure) might not be expected to significantly reduce risk of bleeding.
One question brought up by this case is whether the haemorrhage responsible for decompression haematuria is confined to the bladder, or if it occurs throughout the urinary tract. In our patient blood clot was seen to fill and distend both renal pelvises; did this blood track up from the bladder or did it originate from bleeding within the pelvis itself? A number of factors lead us to conjecture that the bleeding may well have originated from the wall of the renal pelvis itself. Firstly, the renal pelvises were filled with large volumes of clot in the absence of significant dilatation of the ureters (images not shown). Secondly, there is no reason to believe that the pathological process resulting from chronic urinary retention affecting the integrity of capillaries should be limited to the bladder. In the absence of a competent uretero-vesical valve, the ureters and collecting system of the kidneys are subjected to similarly high pressures and are likely to undergo similar pathological changes.
Given this, what can be done to minimise harm to the patient when considering urethral catheterisation for urinary retention? Most importantly, one must recognize patients who are at risk of clinically significant decompression haematuria. Risk correlates with the chronicity of symptoms and the degree of bladder wall damage. Hence, patients such as ours with a long history of LUTS are at increased risk and should have close monitoring post-catheterisation. It is currently unclear as to whether strategies facilitating “gradual decompression” should be advocated: further studies are required to assess the effectiveness and practicality of such strategies.

Teaching points

  • Chronic urinary retention should be suspected in patients with a dilated upper urinary tract and/ or a history of lower urinary tract symptoms such as urinary frequency, urgency, hesitancy, straining, incomplete bladder emptying, poor flow and dribbling.
  • “Decompression haematuria” can commonly occur following urethral catheterisation, and is the result of the sudden drop of pressure in a damaged bladder.
  • Rarely, such bleeding may be severe and require blood transfusion – therefore close monitoring is required in at-risk patient groups.

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References
1. High pressure chronic retention. George NJ, O’Reilly PH, Barnard RJ, Blacklock NJ. Br Med J (Clin Res Ed). 1983 Jun 4;286(6380):1780-3.
2. Urinary retention in women: its causes and management. Kavia RB, Datta SN, Dasgupta R, et al; BJU Int. 2006 Feb;97(2):281-7.
3. Management of urinary retention: rapid versus gradual decompression and risk of complications. Nyman MA, Schwenk NM, Silverstein MD . Mayo Clin Proc. 1997 Oct;72(10):951-6.
4. Hematuria as a complication of sudden decompression of chronically distended bladder in a child with neurogenic bladder dysfunction. Soylu A, Kavukçu S, Türkmen M, Arici A, Aktud T. Pediatr Emerg Care. 2000 Jun;16(3):221.
5. Slow decompression of the bladder using an intravenous giving set. Perry A, Maharaj D, Ramdass MJ, Naraynsingh V. Int J Clin Pract. 2002 Oct;56(8):619.
6. Comparison of rapid versus slow decompression of the distended urinary bladder. Gould F, Cheng CY, Lapides J. Invest Urol. 1976 Sep;14(2):156-8.
7. Intravesical pressure changes during bladder drainage in patients with acute urinary retention. Christensen J, Ostri P, Frimodt-Møller C, Juul C. Urol Int. 1987;42(3):181-4.
8. Practical management of patients with dilated upper tracts and chronic retention of urine. George NJ, O’Reilly PH, Barnard RJ, Blacklock NJ. Br J Urol. 1984 Feb;56(1):9-12.

 

Date added to bjui.org: 11/12/2012

DOI: 10.1002/BJUIw-2012-092-web

 

Right Inguinal Ectopic Scrotum With Ipsilateral Renal Agenesis And Left Foot CTEV (Congenital Talipes Equinovarus ) Deformity: A Rare Congenital Anomaly

A case of an ectopic scrotum located in the right inguinal area is described. The left hemiscrotum was in the normal location and each hemiscrotum contained a testis. The patient also had a left foot CTEV (congenital talipes equinovarus) deformity, right renal agenesis and bladder outlet obstruction with recurrent bladder stone formation. Patient was treated for recurrent bladder stone formation by cystolithotomy and reconstructive surgery.

Authors: Kumar, Amarendra; Singh, Rajendra; Kumar, Satish; Kumar, Rajnish
1.    Associate Professor, Department Of Surgery, Nalanda Medical College, Patna, Bihar India- 800007
2.    Assistant Professor, Department Of Surgery, Nalanda Medical College, Patna, Bihar India- 800007
3.    Senior Resident, Department Of Surgery, Nalanda Medical College, Patna, Bihar India- 800007
4.    Post Graduate Trainee, Department Of Surgery, Nalanda Medical College, Patna, Bihar India- 800007
Corresponding Author: Kumar, Rajnish Post Graduate Trainee, Department Of Surgery, Nalanda Medical College, Patna, Bihar India- 800007. E-mail:[email protected]

 

ABSTRACT
A case of an ectopic scrotum located in the right inguinal area is described. The left hemiscrotum was in the normal location and each hemiscrotum contained a testis. The patient also had a left foot CTEV (congenital talipes equinovarus) deformity, right renal agenesis and bladder outlet obstruction with recurrent bladder stone formation. Patient was treated for recurrent bladder stone formation by cystolithotomy and reconstructive surgery.

CASE REPORT
A 60 yrs old male was admitted in the Department of Surgery, Nalanda Medical College and Hospital, with complaints of dysuria, severe pain at the end of micturition, hematuria and fever. The patient had a history of two previous operations for bladder stone disease. He had also limped on his left foot since childhood.
On physical examination carried out, there was an ectopic scrotum in the right inguinal area with the left hemiscrotum in its normal position. [Figure-1]
The right testis was present in the right ectopic scrotum, the left was normally sited. The median raphe was not developed and the phallus was normal.
The patient’s previous clinical records were reviewed, ultrasound revealed right renal agenesis and bladder calculi. Cystolithotomy had been performed twice for recurrent bladder stones. [Figure-1] shows the ectopic scrotum with a scar from previous cystolithotomy. The patient underwent investigation; routine examination of urine showed many RBCs. Plain x ray of the abdomen showed multiple large stones in the urinary bladder [Figure-2].
The patient had limped on his left limb since childhood. On examination, equino- varus deformity of his left foot [Figure- 3] was detected, no abnormality of his hip or knee was noted.
The patient was prepared for operation after informed consent was taken. Under a neuriaxial block through a lower midline incision, the bladder approached extraperitoneally after excising the scar of previous surgery. The bladder was opened in an inverted Y fashion. There were three stones in the bladder [Figure-4] and a stricture of the bladder neck. All stones were retrieved and a Y-V plasty of bladder neck performed. Postoperatively, the patient developed a minor wound infection, but his recovery was otherwise uneventful..

DISCUSSION
Ectopic scrotum is rare and refers to the anomalous position of one hemiscrotum along the inguinal canal. Most commonly it is supra inguinal [figure-1], although it may be infrainguinal or perineal (1,2). In this case the ectopic scrotum was found in right inguinal region.
This anomaly has been associated with cryptorchidism, inguinal hernia & exostrophy as well as popliteal pterygium syndrome (3). In one review, 70% of patients with a suprainguinal ectopic scrotum exhibited ipsilateral upper urinary tract anomalies, including renal agenesis, renal dysplasia and ectopic ureter (1). In this case, among above mentioned associated anomalies only ipsilateral renal agenesis was noted.
Another study indicated that an associated perineal lipoma was found in 83% of these children; 68% of those with a lipoma had no associated anomalies, whereas 100% of those without a lipoma had associated genital or renal malformations (4). This patient has a distinct left hemiscrotum and right ectopic scrotum situated in the inguinal region associated with right renal agenesis and bladder outlet obstruction due to bladder neck contracture.
The embryology of the gubernaculum and scrotum is intimately related chronologically and anatomically. It seems likely that the ectopic scrotum results from a defect in distal gubernaculum formation that prevents migration of the labioscrotal swelling (5). In this patient, probably the ectopic attachment of the gubernaculum to the right groin has resulted in formation of another hemiscrotum separated from its native place.
This case is peculiar in its presentation in that there is a musculoskeletal defect “left foot CTEV” which has not previously been reported in the literature.

075Figure-1 Right ectopic scrotum in right inguinal region

 

 

 

 

 

 

 

 

 

 

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075Figure-2 X-Ray shows multiple bladder stones

 

 

 

 

 

 

 

 

 

 

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075Figure-4 Retrived bladder stones

 

 

 

 

 

 

 

 

 

 

Fig.4

REFERENCES
1. Elder and Jeffs, 1982. Elder JS, Jeffs RD: Suprainguinal ectopic scrotum and associated anomalies. J Urol 1982; 127:336-338
2. Lamm DL, Kaplan GW Urology.1977 Feb, 9(2);149-53
3. Cunningham LN, Keating MA, Snyder HM, Duckett JW: Urological manifestations of the popliteal pterygium syndrome. J Urol 1989; 141:910-912.
4. Sule JD, Skoog SJ, Tank ES: Perineal lipoma and the accessory labioscrotal fold: An etiological relationship. J Urol 1994; 151:475-477.
5. Hoar RM, Calvano CJ, Reddy PP, Bauer SB, Mandell J.Unilateral suprainguinal ectopic scrotum: The role of the gubernaculum in the formation of an ectopic scrotum. Teratology 1998, feb; 57(2):64-9.

 

Date added to bjui.org: 10/12/2012

DOI: 10.1002/BJUIw-2012-075-web

 

Hydronephrosis of Pregnancy in an Ectopic Pelvic Kidney

We present a case of a woman with an ectopic pelvic kidney and symptomatic hydronephrosis who failed conservative treatment and required ureteric stent placement.

Authors: Toepfer, Nicholas; Parikh, Ankur; Bhangdia, Darshan
Corresponding Author: Toepfer, Nicholas J

 

Introduction
Hydronephrosis during pregnancy is common and has a reported occurrence between 43% and 100% (1).  Both mechanical and hormonal changes during pregnancy are thought to contribute to its etiology, but evidence suggests it is mainly due to compression of the ureters between the pregnant uterus and the linea terminalis (2,3). It has been postulated that this phenomenon does not affect women whose ureters do not cross the pelvic brim. The incidence of an ectopic pelvic kidney is approximately 1 in 2200 to 3000 patients (4,5). Ectopic kidneys occur due to failure of the kidney to ascend during weeks 6-9 of fetal development. Although ectopic pelvic kidneys are relatively common, only one case of hydronephrosis during pregnancy in a pelvic kidney is described in the literature (6). This supports the notion that hydronephrosis during pregnancy in pelvic kidneys is exceedingly rare. We present a case of a woman with an ectopic pelvic kidney and symptomatic hydronephrosis who failed conservative treatment and required ureteric stent placement.

Case Presentation
A 31 year old pregnant female at 22 weeks gestation presented with complaints of sharp right lower quadrant pain, nausea, and vomiting. She denied any lower urinary tract symptoms. On physical examination, she was afebrile and haemodynamically stable. The gravid abdomen was soft, non-tender and no pain could be elicited on palpation. Her urinalysis was negative for leukocyte esterase and nitrite. She demonstrated a leukocytosis of 12.7 K/µL with 88% segmented neutrophils and 7% lymphocytes. A CT scan of the abdomen and pelvis with intravenous contrast was performed for suspected acute appendicitis. The CT scan showed an ectopic right kidney located in the pelvis with mild hydronephrosis of an anteriorly oriented collecting system (Figure 1a-b). The ectopic kidney was malrotated, medially deviated, and located anterior to the iliac bifurcation and immediately posterior to the gravid uterus. The left kidney was unremarkable and located in the orthotopic location in the left retroperitoneum. The patient’s severe right lower quadrant pain persisted despite intravenous analgesia. The patient was transferred to a tertiary medical center for further care.
The patient was counseled on the options regarding symptomatic hydronephrosis during pregnancy and she opted for conservative management with intravenous and oral analgesia. She continued to experience intractable abdominal pain and after 48 hours of medical management, she decided to undergo a cystoscopy with retrograde ureteric stent placement.
At operation, there were no abnormal findings on cystoscopy. A retrograde pyelogram was performed. The ureter had no evidence of filling defects as it coursed to a midline kidney located at the level of the L4 vertebral body. An 18 cm long 4.7 Fr diameter stent was placed and the location was confirmed using fluoroscopy. Postoperatively, the patient’s pain completely resolved but she did report mild bladder irritation, frequency and urgency from the ureteric stent.
The patient underwent exchange of her stent 12 weeks later. She eventually underwent a caesarean section for a breech presentation at 39 weeks. This was completed without complications and the newborn baby was without any identifiable abnormalities. The ureteric stent was removed 12 days later and the patient remained asymptomatic afterwards. A renal ultrasound one month after stent removal demonstrated resolution of the hydronephrosis (Figure 2).

Discussion
Hydronephrosis can manifest at different stages during pregnancy. A large prospective study found hydronephrosis present in 15%, 20% and 50% of women during the first, second, and third trimesters respectively.1 The development of dilatation at various stages of pregnancy may represent different etiologies of hydronephrosis in pregnancy including mechanical and hormonal effects.
The 15% incidence of ureteric dilation on ultrasound during the first trimester occurs before the uterus reaches the pelvic brim. This supports a non-mechanical etiology for the development of hydronephrosis. Increased levels of progesterone during pregnancy have been hypothesized to promote ureteric dilatation and inhibit peristalsis which causes subsequent dilation of the upper urinary tract (1,7). In addition, progesterone has been shown to slow the rate of disappearance of hydroureter in postpartum women (8).
While hormonal changes may contribute to hydronephrosis of pregnancy, the most plausible etiologic factor is mechanical obstruction of the ureter by the gravid uterus. Studies have shown normal ureteral contractile pressures in pregnant women suggesting that hormonal induced atony is not the primary factor in ureteric dilation (2). Additionally, women whose ureters do not cross the pelvic brim, such as those associated with ectopic and pelvic kidneys or non-orthotopic urinary diversion, have been shown not to develop ureteric dilation of pregnancy (9-11). Despite this evidence, our patient demonstrates that women with pelvic kidneys can develop hydronephrosis during pregnancy.
Pelvic kidneys present challenges in treatment for a variety of reasons including tortuosity of the ureters limiting endoscopic access, as well as a greater risk of injuring aberrant vessels or overlying abdominal viscera. Management of symptomatic hydronephrosis in pregnancy is often treated with ureteric stent placement (12-15). Advantages of treating an obstructed kidney with a percutaneous nephrostomy rather than an indwelling ureteric stent are the lower cost and ease of changes during pregnancy (16). In addition, percutaneous nephrostomies have a lower incidence of irritative LUTS, reduced analgesic requirements and patients are thought to have a better quality of life as compared to those patients with indwelling stents (17,18). In this particular patient, the location of the ectopic kidney was such that a percutaneous nephrostomy could not be placed safely under ultrasound guidance due to proximity of the iliac artery. In order to minimize the risk of radiation exposure to the fetus, the ALARA principle was applied. The fluoroscopy settings during stent placement were set to low dose with collimation and the patient’s gravid abdomen was shielded using a lead gown. Fluoroscopy time was limited as much as possible and there was only a total accumulated dose area product to the patient of 0.7 Gy/cm2.
Any surgery during pregnancy has increased risks. Pregnancy is a hypercoagulable state associated with an increased risk of deep venous thrombosis and thromboembolism and there is also an increased risk of aspiration during general anesthesia. Regarding potential risks to the fetus during surgery, a review of 5,405 patients found no increased incidence of congenital abnormalities, premature labor or stillbirths during non-obstetrical operations (19). Rapid encrustation of ureteric stents as a result of the hypercalciuria and hyperuricosuria during pregnancy must be considered when deciding when to exchange stents (20). This patient had no prior history of urolithiasis so it was decided to extend the time to stent exchange closer to her due date at 34 weeks gestation in case the endoscopic manipulation induced premature labor.
Resolution of the hydronephrosis following delivery confirmed that the obstruction was secondary to the pregnancy and not a previously undiagnosed chronic obstruction such as pelviureteric junction obstruction which is common in ectopic kidneys (4).

Conclusion
Hydronephrosis of pregnancy occurs in most women but it has been found not to affect those patients with pelvic kidneys. This patient illustrates that it is possible for women with ectopic pelvic kidneys to develop hydronephrosis during pregnancy. While conservative management is successful in controlling symptoms in most patients with hydronephrosis during pregnancy, a patient with a pelvic kidney such as this may require more aggressive drainage of the kidney with a ureteric stent or nephrostomy.

 

Toepfer-Fig1a

Fig.1a

Toepfer-Fig1b

Fig1.b

Toepfer-Fig1b

Fig.2

Acknowledgments
None

Disclosure Statement
No competing financial interests exist.

References
1. Faundes A, Bricola-Filho M, Pinto et al. Dilatation of the urinary tract during pregnancy: Proposal of a curve of maximal caliceal diameter by gestational age. Am J Obstet Gynecol 1998; 178(5):1082-1086.
2. Roberts JA. Hydronephrosis of pregnancy. Urology 1976; 8(1):1-4.
3. Rasmussen PE, Nielsen FR. Hydronephrosis during pregnancy: a literature survey. Eur J Obstet Gynecol Reprod Biol 1988; 27(3):249-259.
4. Cinman NM, Okeke Z, Smith AD. Pelvic Kidney: Associated Diseases and Treatment. J Endourology 2007; 21(8):836-842.
5. Zafar FS, Lingeman JE. Value of laparoscopy in the management of calculi complicating renal malformations. J Endourol 199; 10(4):379-383.
6. Malhorta N, Roy KK, Garg PK, et al. Ectopic hydronephrotic kidney masquerading as an ovarian cyst during pregnancy. Eur J Obstet Gynecol Reprod Biol 2001; 97(2):239-240.
7. van Wagenen G, Jenkins RH. An experimental examination of factors causing ureteral dilation of pregnancy. J Urol 1939; 42:1010-1020.
8. Lubin S, Drexler LS, Bilotta WA. Post-partum pyeloureteral changes following hormone administration. Surg Gynecol Obstet 1941; 73:391.
9. Swanson SK, Heilman RL, Eversman WG. Urinary tract stones in pregnancy. Surg Clin North Am 1995; 75:123-142.
10. McAleer SJ, Loughlin KR. Nephrolithiasis and pregnancy. Curr Opin Urol 2004; 14:123-127.
11. Harrow BR, Sloane JA, Salhanith L. Etiology of hydronephrosis of pregnancy. Surg Gynecol Obstet 1964; 119:1042-1048.
12. Sadan O, Berar M, Sagiv R, et al. Ureteric stent in severe hydronephrosis of pregnancy. Eur J Obstet Gynecol Reprod Biol 1994; 56(2):79-81.
13. Delakas D, Karyotis I, Loumbakis P, et al. Ureteral drainage by Double-J-catheters during pregnancy. Clin Exp Obstet Gynecol 2000; 27(3-4):200-202.
14. Fainaru O, Almog B, Gamzu R, et al. The Management of Symptomatic Hydronephrosis in pregnancy. BJOG 2002; 109:1385-1387.
15. Vendola N, Giumelli P, Galdini R, et al. Ureteral Drainage with Double-J Catheters in Obstructive Uropathy during Pregnancy. Gynecol Obstet Invest 1995; 40:274-275.
16. Kavoussi LR, Albala DM, Basler JW, et al. Percutaneous Management of Urolithiasis During Pregnancy. J Urol 1992; 148(3):1069-1071.
17. Joshi HB, Adams S, Obadeyi OO, et al. Nephrostomy tube or “JJ” ureteric stent in ureteric obstruction: assessment of patient perspectives using quality of life survey and utility analysis. Eur Urol 2001; 39:695-701.
18. Mokhmalji H, Braun PM, Portillo JM, et al. Percutaneous nephrostomy versus ureteral stents for diversion of hydronephrosis caused by stones: a prospective randomized clinical trial. J Urol 2001; 165:1088-1092.
19. Mazze RI, Kallen B. Reproductive outcome after anesthesia and operation during pregnancy: a registry study of 5405 cases. Amer J Obst Gynec 1989, 161:1178-1185.
20. Goldfarb R, Nerrhut G, Lederer E. Management of acute hydronephrosis of pregnancy by ureteral stenting: risk of stone formation. J Urol 1989; 141:921-922.

 

Date added to bjui.org: 08/12/2012

DOI: 10.1002/BJUIw-2012-058-web

 

Primary small cell carcinoma of the ureter with hydronephrosis

We report a case of primary small cell carcinoma of the ureter with hydronephrosis.

Authors: Zhao Zhenhua (1), Wang Boyin (1), Yang Jianfeng (1), Wang Ning (2), Pan Shouhua (3)
1. Department of Radiology, Shaoxing People’s Hospital (Zhejiang University Shaoxing Hospital) Shaoxing, Zhejiang, China
2. Department of Pathology, Shaoxing People’s Hospital (Zhejiang University Shaoxing Hospital) Shaoxing, Zhejiang, China
3. Department of Urology, Shaoxing People’s Hospital (Zhejiang University Shaoxing Hospital) Shaoxing, Zhejiang, China

Corresponding Author: Yang Jianfeng Department of Radiology, Shaoxing People’s Hospital (Zhejiang University Shaoxing Hospital) 568 Zhongxing North Rd, Shaoxing, Zhejiang, China

 

Introduction
Small cell carcinoma (SCC) is usually found in the lungs, and its extrapulmonary counterpart is rarely encountered. Primary small cell carcinoma (PSCC) of the ureter with hydronephrosis is extremely rare. We report a 70-year-old woman who presented with left-sided flank pain. The clinical impression and diagnosis following renal ultrasound was of a calculus in the distal left ureter. Abdominal and pelvic CT indicated a mass near the distal ureter with pronounced hydronephrosis. The patient underwent left nephroureterectomy. Histological and immunohistochemical staining confirmed PSCC of the ureter. After 9 months, the patient was found to have massive metastases in the liver and lungs, lymphadenopathy in the retroperitoneum, and para-aortic region, and several implantation metastases in the bladder and left psoas major. Radiologists and clinicians should be aware of the possibility and severity of malignant PSCC of the ureter in patients with hydronephrosis.

Case report
A 70-year-old woman presented with left-sided flank pain without the symptoms of bladder irritation and gross hematuria. The pain was continuous with paroxysmal irritation radiating to the hypogastrium and groin. It was accompanied by nausea but no vomiting. Physical examination was unremarkable except for sensitivity to percussion in her left renal region and tenderness over the course of the left ureter. Urinalysis indicated leukocytes (+) in her urine specimen; her other laboratory data were within normal limits. Ultrasonography revealed one 6-mm diameter calculus in the distal left ureter with hydronephrosis. The pain did not improve after several days’ treatment with anti-inflammatory agents and analgesia. The patient then underwent intravenous urography (IVU), abdominal and pelvic computed tomography (CT), and chest radiography.

The IVU showed a left hydroureter (Fig. 1A). The CT indicated pronounced left hydronephrosis and thickened perirenal tissue within the wall of the left upper ureter. Abdominal multiplanar reconstruction (MPR) demonstrated a mass near the end of the ureter and the thickened, irregular wall of the upper ureter with pronounced hydronephrosis (Fig. 1B). We did not observe any lymphadenopathy in the pelvis, retroperitoneum, or para-aortic regions. A chest radiograph was reviewed, and no abnormality was seen.

The patient underwent left nephroureterectomy. Macroscopically, a 1.6 × 1.2 × 0.5–cm grayish white lesion was identified.. Histological examination revealed that the mass was composed of small cells with hyperchromatic nuclei, round to fusiform in shape, exhibiting a high level of mitotic activity but little cytoplasm and an absence of nucleoli [Fig. 2A]. Immunohistochemical staining of the specimen was positive for chromogranin A (CgA) [Fig. 2B], CD56, synaptophysin (Syn), neuron-specific enolase, and Ki-67 80%, confirming the neuroendocrine origin of the tumor.

The abdominal and pelvic CT and chest radiography performed 9 months after the operation indicated massive metastases in the liver and lungs [Fig. 3A], lymphadenopathy involving the retroperitoneum, and para-aortic regions, and some implantation metastases in the bladder and left psoas major [Fig. 3B]. This patient died 10 months after operation due to multiple organ failure.

Discussion
Primary small cell carcinoma of the ureter is an extremely malignant and rare disease; only several cases worldwide have been reported so far [1-4]. To our knowledge, PSCC with hydronephrosis has never been reported. The cause of SCC of the ureter may be related to smoking, but it remains unclear [2]. There are two hypotheses regarding the histopathogenesis of urinary tract small cell carcinoma. One indicates that it originates from intrinsic neuroendocrine cells within the normal genitourinary tract derived from the neural crest during embryogenesis [5], whereas the other hypothesis suggests that it results from a transformation of pluripotent epithelial reserve cells in the genitourinary tract that exhibit the ability to generate any cell type [6]. According to Kim Ts [2], SCC of the ureter combined with other components, such as transitional cell carcinoma, adenocarcinoma, squamous cell carcinoma, and carcinoma sarcomatodes, supports the second hypothesis. However, in this case, there are no other components; thus, it is compatible with the first hypothesis.
Because of high mitotic activity [1, 4, 7], small cell carcinoma of the ureter usually obstructs the ureter completely. The ureter above the obstruction or renal pelvis exhibits severe dilation. To our knowledge, severe and long-standing obstruction can give rise to the renal insufficiency or renal failure; the excretion of contrast medium is then not visualized on IVU or CT after contrast administration. In this patient, we did not find these changes. We presume that this was due to the following two reasons: the pressure in the left renal pelvis decreased after the development of the hydronephrosis, and secondly that the function of the right kidney improved to compensate.

The clinical features of PSCC of the ureter are mostly hematuria and flank pain [4]. Hematuria, usually gross, is due to vascular invasion, whereas pain is secondary to hydronephrosis after obstruction of the ureter. However, in this case, the patient only presented with left-sided flank pain and radiating pain in both the hypogastrium and groin. This clinical feature was similar to the presentation of a ureteric calculus. Ultrasonography is a commonly performed examination for urogenital diseases, but its resolution is inferior to CT. We can differentiate a calculus from a mass in the ureter by CT scanning, and demonstrate the development of hydronephrosis and its relationship with surrounding tissues through MPR.

Nephroureterectomy is the primary treatment in the majority of patients with PSCC of the ureter but usually cannot achieve adequate control of the disease; cisplatin-based chemotherapy has been frequently combined with surgery, but the overall outcome is poor [1]. The lymphatics, the lung, and the liver are common metastatic sites for small cell carcinoma of the ureter, and the majority of patients with small cell carcinoma die of the disease within a year [2]. This PSCC of the ureter caused hydronephrosis, and subsequently extensive metastases involving the liver and lungs and lymphadenopathy in the retroperitoneum, and para-aortic region. A the metastasis in the psoas major muscle also occurred 9 months following the surgical procedure.

Conclusion

Primary small cell carcinoma of the ureter is rare and often misdiagnosed as a ureteric calculus or other disease. CT assisted in identifying both the lesion and subsequent metastases, but the final diagnosis depended on pathological confirmation. During operation, surgeons must be mindful of the possibility of implantation metastasis in the perirenal tissue in patients with PSCC of the ureter with hydronephrosis. Radioactive implants may be considered to help prevent metastases. A patient with an early diagnosis and comprehensive therapy may achieve a long-term relative survival
.
Conflict of interest: There are no conflicts of interest in this study.

References
1. Kozyrakis D, Papadaniil P, Stefanakis S, et al. Small cell carcinoma of the urinary tract: a case report. Cases J. 2009;2:7743.
2. Kim TS, Seong DH, Ro JY. Small cell carcinoma of the ureter with squamous cell and transitional cell carcinomatous components associated with ureteral stone.J Korean Med Sci. 2001;16:796-800.
3. Ishikawa S, Koyama T, Kumagai A, Takeuchi I, Ogawa D. A case of small cell carcinoma of the ureter with SIADH-like symptoms. Nippon Hinyokika Gakkai Zasshi. 2004;95:725-8.
4. Kho VK, Chan PH. Primary small cell carcinoma of the upper urinary tract. J Chin Med Asso. 2010;73:173-6.
5. Fetissof F, Dubois MP, Lanson Y, Jobard P. Endocrine cells in renal pelvis and ureter: an immunohistochemical analysis. J Urol. 1986;135:420-1.
6. Christopher ME, Seftel AD, Sorenson K, Resnick MI. Small cell carcinoma of the genitourinary tract: an immunohistochemical, electron microscopic and clinicopathological study. J Urol.1991; 146:382-8.
7. Sved P, Gomez P, Manoharan M, Civantos F, Soloway MS. Small cell carcinoma of the bladder. BJU Int. 2004;94:12-7.

Fig1a040Fig1b040

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Fig 1
The IVU shows the dilated ureter (arrow) [A]. The sagittal oblique reconstruction of the left ureter shows the lesion at the end of the ureter (arrow) and hydronephrosis (asterisk) [B]

Fig2a040Fig2b040

 

 

 

 

Fig 2
Light microscopy shows small cells that exhibit hyperchromatic nuclei, round to fusiform in shape, and high mitotic activity with little cytoplasm and an absence of nucleoli (H&E, 400×) [A]. The immunohistochemical staining of the specimen is positive for CgA (100×) [B]

Fig3a040Fig3b040

 

 

 

 

 

 

 

 

 

 

 

 

 

Fig 3
Many metastases scatter over the liver [A] and one of implantation metastases are in the left psoas major (arrow) [B] 9 months postoperatively

Date added to bjui.org: 06/12/2012
DOI: 10.1002/BJUIw-2012-040-web

 

Port–Site recurrence following Laparoscopic Radical Nephrectomy for Chromophobe Renal Cell Carcinoma

Port-site metastasis following laparoscopic radical nephrectomy is being increasingly recognized as a complication following laparoscopic surgery, especially when correct surgical principles are violated. All previously reported cases have been of either the clear cell or papillary variant of renal cell carcinoma. Herein we report a case of chromophobe renal cell carcinoma with port-site recurrence 10 months after laparoscopic radical nephrectomy.

Authors: Javali, Tarun; Dogra, Premnath; Singh, Prabhjot
Corresponding Author: Javali, Tarun

 

Introduction
Port-site metastasis following laparoscopic radical nephrectomy is being increasingly recognized as a complication following laparoscopic surgery, especially when correct surgical principles are violated. All previously reported cases have been of either the clear cell or papillary variant of renal cell carcinoma. Herein we report a case of chromophobe renal cell carcinoma with port-site recurrence 10 months after laparoscopic radical nephrectomy.

Case History
A 44 year old lady presented with a 6 month history of left flank pain and hematuria. Computed tomography revealed a 12×5×5 cms heterogeneous enhancing left renal upper pole mass. [Fig.1]. There was no evidence of lymph node enlargement or liver metastasis, and no tumor thrombus. The CT scan also revealed a multiloculated right adnexal cyst of size 6×4×4 cms. The patient underwent laparoscopic transperitoneal left radical nephrectomy and right oophorectomy. Additional ports were placed for the oophorectomy. The radical nephrectomy was performed first because it was deemed to be more technically challenging than the oophorectomy. Both the specimens were entrapped separately in retrieval bags and removed through a Pfannensteil incision. The specimen was not morcellated.
Histopathology revealed a chromophobe renal cell carcinoma. On immunohistochemical analysis, the tumor was positive for cytokeratin and negative for CD10 and vimentin. There was no evidence of any capsular breach. The tumor had reached the renal sinus but had not infiltrated it. Histopathology of the right oophorectomy specimen showed an endometrial cyst.
The patient was reviewed on a three monthly basis with a chest x-ray, liver and kidney function tests and a full blood count. No abdominal imaging was done at this time. Ten months after surgery, the patient presented with pain and swelling at one of the port sites. Examination revealed a hard subcutaneous nodule of size 2×1.5 cm at the 12mm working port site above and medial to the left anterior superior iliac spine [Fig 2]. The surgical incision site for specimen removal had no evidence of recurrence. The patient had already had fine needle aspiration cytology (F.N.A.C.) performed, which was reported as showing ‘malignant cells suspicious for renal cell carcinoma’. A contrast enhanced CT scan of her abdomen revealed an irregular enhancing subcutaneous soft tissue lesion in left anterior abdominal wall measuring 1.8 cms, thought likely to be a metastasis. The liver and right kidney were normal and there were no retroperitoneal nodes or masses. Positron emission tomography-computed tomography (PET-CT) revealed increased 18-fluro deoxy glucose (18FDG) uptake in the soft tissue density lesion in left lower abdominal wall [Fig 3]. The patient underwent wide local excision of the port-site nodule. Histology revealed chromophobe renal cell carcinoma with the same histomorphology as the primary tumour [Fig 4,5].
At one year post excision of the port-site nodule, patient is well, and has had no recurrences, either local or systemic.

Discussion
Factors which have been implicated in port-site recurrence include natural tumor factors, local wound factors, immune response and laparoscopic related factors such as direct wound contamination, either instrument contamination or during specimen extraction, specimen morcellation, use of specimen retrieval bags and pneumoperitoneum pressure [1,2]. Poorly differentiated transitional cell carcinomas have accounted for most of the cases of port site recurrence in laparoscopic uro-oncologic case series [1,2,3]. Only a few cases of port site recurrence following laparoscopic nephrectomy for renal cell carcinoma have been reported [4,5,6,7,8,9,10]. In all of these cases the histopathology was clear cell carcinoma. Russo et al reported a case of papillary renal cell carcinoma with port site metastasis following laparoscopic partial nephrectomy [11]. To the best of our knowledge this the first case of chromophobe renal cell carcinoma with port site recurrence. In the present case the tumor was organ confined (pT2) and well differentiated. Most studies report more favorable prognosis for chromophobe compared to conventional renal cell carcinoma. This goes against the commonly held notion that adverse tumor biology or aggressive nature of the tumor is the most important causative factor in port site recurrence [1]. Greco et al have further elaborated on the factors that accelerate the development of port-site metastasis [12]. These conditions include performing laparoscopic surgery in the presence of ascites, lack of trocar fixation to prevent dislodgement and gas leakage around the trocars, inadequate laparoscopic equipment and technique and tumour boundary violation. Factors which have deemed to reduce the incidence of port-site metastases include use of a bag for specimen retrieval, placement of drainage before desufflation, povidone-iodine irrigation of instruments, trocars and port-site wounds and suturing trocar wounds ≥10mm in size.
In the present case, all possible precautions were taken during the course of surgery, including changing the instruments, once the radical nephrectomy was over. A probable etiologic factor in this case may have been the prolonged operating time as laparoscopic nephrectomy was followed by laparoscopic oophorectomy and the gynaecologists also used the left sided 12mm working port. Microleakage around ports (“chimney effect”) has been postulated to play a role in port site metastasis [13]. Ports used by the main operating surgeon have been proved to have more contamination by tumor cells than either those used by the assistants or the camera port [14]. It is a matter of conjecture that whether performing the oophorectomy first followed by radical nephrectomy could have altered the result.
We wish to highlight two main points through this case report. Firstly, that tumor histology may not be predictive of port site recurrence. Chromophobe renal cell carcinoma is biologically a tumor of low malignant potential. Metastasis of chromophobe tumour constitute less than 1% of all metastatic renal cell carcinoma [15]. Advanced pathological T stage, tumor necrosis and sarcomatoid change have been purported to predict an aggressive phenotype of chromophobe renal cell carcinoma [16]. However none of these features were present in this case. Hence following laparoscopic radical nephrectomy patients need to be carefully examined at each visit with particular attention to the port sites. This should be done regardless of the grade, stage or histology of the tumor.
The second point we wish to highlight is that PET-CT could be a useful adjunct in diagnosing port-site recurrence in equivocal cases. This may be especially relevant in patients who return within a short span of time following laparoscopic radical nephrectomy, wherein induration due to surgical factors at scar site may be confused vis-a vis port site recurrence.

035fig1

 

 

 

 

 

 

 

 

 

 

 

 

 

Fig. 1 – CT KUB (Kidney-ureter-bladder) showing left upper pole tumor.

035fig2

 

 

 

 

 

 

 

 

 

 

 

 

Fig. 2 – Port-site nodule

035fig3

 

Fig. 3 – PET-CT showing port-site metastasis

035fig4

 

 

 

 

 

 

 

 

 

 

Fig. 4 – Hematoxylin and Eosin staining of excised port-site specimen. Sheets of cells with round to oval nuclei and perinuclear halo with prominent cell membranes.

035fig5

 

 

 

 

 

 

 

 

 

 

Fig. 5 – Immunohistochemistry. Negative for CD10 and vimentin.

References
1. Rassweiler J, Tsivian A, Kumar AV, Lymberakis C, Schulze M, Seeman O, et al. Oncological safety of laparoscopic surgery for urological malignancy: experience with more than 1,000 operations. J Urol 2003; 169:2072–5.
2. Tsivian A, Sidi AA. Port site metastases in urological laparoscopic surgery. J Urol 2003; 169:1213–8.
3. Micali S, Celia A, Bove P, et al: Tumor seeding in urological laparoscopy: an international survey. J Urol 2004; 171: 2151–4.
4. Fentie DD, Barret PH, and Taranger LA: Metastatic renal cell carcinoma after laparoscopic radical nephrectomy: long term follow-up. J Endourol 2000; 14: 407–11.
5. Landman J, and Clayman R: Re: port site tumor recurrences of renal cell carcinoma after videolaparoscopic radical nephrectomy (letter). J Urol 2001; 166: 629–30..
6. Castilho LN, Fugita OEH, Mitre AI, et al: Port site tumor recurrences of renal cell carcinoma after videolaparoscopic radical nephrectomy. J Urol 2001; 165: 519.
7. Chen YT, Yang SSD, Hsieh CH, et al: Hand port-site metastasis of renal-cell carcinoma following hand-assisted laparoscopic radical nephrectomy: case report. J Endourol 2003; 17: 771–4.
8. Iwamura M, Tsumura H, Matsuda D, et al: Port site recurrence of renal cell carcinoma following retroperitoneoscopic radical nephrectomy with manual extraction without using entrapment sac or wound protector. J Urol 2004; 171: 1234–5.
9. Dhobada S, Patankar S, Fais F, et al: Port-site metastasis after laparoscopic radical nephrectomy for renal-cell carcinoma: case report. J Endourol 2006; 20: 119–22.
10. Goyal R., Sing P., Mandhani A. et al. Port-site metastatis of renal cell carcinoma after laparoscopic transperitoneal radical nephrectomy. Ind J of Urol 2006; 22:150-1.
11. Masterson TA, Russo P. A case of port-site recurrence and loco-regional metastasis after laparoscopic partial nephrectomy. Nature Reviews Urology 2008; 5:345-9.
12. Greco F, Wagner S, Reichelt O, Inferrera A, Lupo A, Hoda MR, Hamza A, Fornara P
Huge port-site metastasis after laparoscopic partial nephrectomy: a case report. Eur Urol 2009; 56:737-39.
13. Curet MJ: Port site metastases. Am J Surg 2004; 187: 705-12.
14. Ramirez PT, Wolf JK, and Levenback C: Laparoscopic port-site metastases: etiology and prevention. Gynecol Oncol 2003; 91: 179–89.
15. Choueiri TK, Plantade A, Elson P et al. Efficacy of sunitinib and sorafenib in metastatic papillary and chromophobe renal cell carcinoma. J Clin Oncol 2008; 26:127-31.
16. Amin MB, Paner GP, Alvarado-Cabrero I et al. Chromophobe renal cell carcinoma: histomorphologic characteristics and evaluation of conventional pathologic prognostic parameters in 145 cases. Am J Surg Pathol 2008; 32:1822-34.

 

Date added to bjui.org: 05/12/2012

DOI: 10.1002/BJUIw-2012-035-web

 

Renal cell carcinoma in a setting of chronic lithium toxicity

A case of renal cell carcinoma on a background of acquired cystic disease due to chronic lithium toxicity is described. The possible pathogenetic mechanisms that may have lead to the development of the renal cell carcinoma in this setting are explored.

Authors: Zardawi, Ibrahim; Nagonkar, Santoshi; Patel, Purvish
Corresponding Author: Zardawi, Ibrahim

 

Lithium salts are widely used in the treatment of affective disorders of the bipolar type. Lithium is a nephrotoxic substance and patients undergoing long-term lithium treatment can present with acute lithium intoxication, chronic renal disease or nephrogenic diabetes insipidus.1,2 Lithium causes structural renal damage with tubular loss, fibrosis, inflammation, glomerular sclerosis and cyst formation.3 Cysts appear to predispose the kidney to renal cell carcinoma and an association between the two conditions has been well documented.4 A case of renal cell carcinoma on a background of acquired cystic disease due to chronic lithium toxicity is described. The possible pathogenetic mechanisms that may have lead to the development of the renal cell carcinoma in this setting are explored.

A 72 year old obese female with an 18 year history of a schizoaffective disorder, presented in March 2011 with painless haematuria. She had been on Lithium for approximately 12 years, from 1993 to 2005. She was diagnosed in 2005 with Type 2 Diabetes Mellitus and in 2008 with chronic kidney disease thought to be due to chronic interstitial nephritis from long term Lithium use. She has been a heavy user of non-steroidal anti-inflammatory drugs but is a non-smoker. Her past medical history included hysterectomy and appendectomy. On examination, she was found to be mildly hypertensive with a blood pressure of 155/90 mm Hg. She was haemodynamically stable with no pallor, bruising, petechiae or purpura and no signs of mucosal bleeding. Her abdomen was soft and non-tender and there were no masses or organomegaly. Her medications at presentation included Aripiprazole 10mg daily, Sodium Valproate 500mg bd, Atorvastatin 20mg daily, Thyroxin 50mcg daily and Telmisartan Hydrochlorothiazide 80/12.5 mg daily.

Investigations revealed a raised blood urea at 16.5 mmol/L (reference range (3.0-10.0 mmol/L) and serum Creatinine of 205 umol/L (reference range 30-100 umol/L). Renal ultrasound showed a mass (4.5x 4.7x 4.5 cm) with cystic and solid components and increased vascularity in the upper pole of the left kidney. No other renal masses were detected but both kidneys contained multiple cysts. A mobile echogenic mass (3.4cm X 2.8 cm in size), thought to be a blood clot, was detected in the bladder. No significant abnormalities were detected on cystoscopy and retrograde ureteroscopy, except for a blood clot which was removed during the procedure. No bladder or ureteric masses or stones were identified. Bladder biopsy showed active (acute and chronic) inflammation but no urothelial dysplasia or malignancy. A left renal tumour, indenting the renal sinus and extending into the renal vein with the presence of thrombosis was detected on Magnetic Resonance Imaging (MRI). Numerous cysts, mostly less that 5mm in diameter were identified in both kidneys. There was no evidence of metastatic disease in the abdomen or pelvis. A left laparoscopic nephrectomy was performed. The patient has been well post nephrectomy with no clinical or radiological evidence of tumour recurrence.

The macroscopic specimen consisted of a kidney with attached peri-nephric fat. The kidney (140x85x40mm) contained numerous cysts up to 30mm in maximum diameter (Fig. 1). A relatively circumscribed, partly necrotic tumour with haemorrhage necrosis, 60 x 55mm was noted in the upper part of the kidney with extension into the renal vein and peri-hilar fat (Fig. 1). Microscopically, the renal tumour was a Fuhrman grade 2 clear cell carcinoma. The tumour had extended into the peri-hilar adipose tissue and the renal vein. The remainder of the kidney showed numerous simple cysts, each lined with a layer of flattened cuboidal cells (Fig. 2). Cysts were present throughout the renal parenchyma with involvement of cortical and medullary tissues.

Lithium salts are widely used in the treatment of affective disorders of the bipolar type. A frequent side effect of long-standing lithium treatment is kidney damage. Compared with the general population, patients on long-term Lithium salts are 6 times more likely to develop chronic renal failure. In a recent Swedish study, plasma creatinine levels of >150 µmol/L (normal range 30-100 µmol/L) were detected in 12% of patients on long-term lithium treatment and renal replacement therapy was required in 5.3% of the lithium treated population.5 Patients undergoing long-term lithium treatment can present with acute lithium intoxication, chronic renal disease or nephrogenic diabetes insipidus.1,2 Lithium causes structural renal damage with tubular loss, fibrosis, inflammation, glomerular sclerosis and cyst formation.3,4

Cysts appear to predispose the kidney to renal cell carcinoma and an association between the two conditions has been well documented.5 The patient presented here, is obese, had been on lithium for 12 years and has misused non-steroidal anti-inflammatories for a long period of time. The above combination is important when considering renal cell carcinoma in this setting because a relationship, albeit indirect, can be established between these medications and renal cell carcinoma. Renal biopsy from patients on long-term lithium treatment often shows chronic interstitial nephritis with associated tubular atrophy, cortical and medullary fibrosis, glomerular sclerosis, tubular dilatation and cyst formation.6,7 This patient had numerous renal cysts and macroscopically these cysts were indistinguishable from the cysts of adult polycystic kidney disease. These cysts, which were deemed to be acquired (acquired cystic disease), were attributed to lithium toxicity.

Renal cell carcinoma is a group of malignancies that arise from the renal tubular epithelium. Although smoking, obesity and hypertension are the main risk factors for renal cell cancer in both sexes, exposure to environmental chemicals and prolonged intake of phenacetin-containing analgesics also play a role in the aetiology of renal cell carcinoma.8 An association between renal cell carcinoma and acquired and hereditary cysts has been well documented.9 The majority of renal neoplasms in the setting of acquired cystic kidney disease have been in patients on chronic haemodialysis for several years. Tumours also occur in patients with chronic renal failure who have not been dialysed. The number of cysts is important for establishing a diagnosis of renal cell carcinoma associated with acquired cystic disease. At least 5 cysts are necessary for a diagnosis of acquired cystic disease.8 This patient, who had not been dialysed, fulfils the criteria for acquired renal disease. Although renal tumours are found in 25% of patients with acquired cystic disease, not all lesions are histologically malignant and only 5% metastasise. This observation is different to the findings in this case, which is a high stage tumour with a large tongue of tumour extending into the main renal vein. In some cases of acquired cystic disease, atypical proliferative epithelial changes, harbouring cytogenetic abnormalities, representing early neoplastic transformation have been found. Some clear cell renal cell carcinomata arising in the setting of acquired cystic disease has been shown to have von Hipple-Lindau (VHL) gene mutations.8 The cysts in this patient are simple and there is no epithelial hyperplasia or atypia, therefore no precancerous proliferative changes are demonstrable morphologically. It is worthwhile remembering that renal cell carcinomas are rare in adult polycystic kidneys and probably occur with no more greater frequency than might be expected based on the prevalence of the two conditions.8

A small but consistent excess of renal cell carcinoma has been established with exposure to phenacetin-containing analgesics which also cause cancer of the renal pelvis.8 This patient has abused many types of analgesics for a long period of time and had often consumed large quantities of these analgesics.

The risk of renal cell carcinoma increases steadily with increasing body mass index (BMI).10 The incidence of renal cell carcinoma in obese people with a BMI of >29 kg/m2 is double that of normal individuals. In Europe one quarter of kidney cancers in both sexes are attributable to excess weight, particularly in women8. A real association would be supported by oestrogen-mediated carcinogenesis that is documented in animal models. Conversely, it could be a confounding effect of excess body weight that is often increased in women who have had many children. Our patient’s BMI is close to 30 kg/m2. The mechanism by which obesity predisposes to renal cell cancer in humans is unclear, although hormonal factors are thought to play a role. An alternative explanation is that hypertension or metabolic complications of obesity may result in kidney damage that increases susceptibility to carcinogens or promoting agents. The association with obesity persists after controlling for other risk factors, such as a history of hypertension or kidney disease and intake of meat, fat or protein.10

The incidence of renal cell carcinoma is significantly increased in people with a history of hypertension that is independent of obesity and tobacco smoking.9 The association with the use of diuretics instead is likely to be due to the associated hypertension.

Lithium is commonly used in the treatment of bipolar mental illness. It is nephrotoxic and long-term use of lithium can lead to chronic renal failure through tubular damage with cyst formation. Kidneys with multiple cysts are at risk of renal cell carcinoma. In this case, it is difficult to determine if long term Lithium use is responsible for the renal cell carcinoma as other contributing factures to renal cell carcinoma, such hypertension, obesity and analgesic abuse are also present. Despite this, patients on long-term lithium therapy should undergo regular renal function and imaging tests.

References
1. Hansen HE. Renal toxicity of lithium. Drugs 1981; 22: 461–476.
2. Timmer RT, Sands JM: Lithium intoxication. J Am Soc Nephrol. 1999;10: 666–674.
3. Hansen HE, Hestbech J, Olsen S, Amdisen A. Renal function and renal pathology in patients with lithium-induced impairment of renal concentrating ability. Proc Eur Dial Transplant Assoc. 1977;14:518-527.
4. Bendz H, Schön S, Attman PO, Aurell M. Renal failure occurs in chronic lithium treatment but is uncommon. Kidney Int. 2010;77:219-224.
4. Hurst FP, Jindal RM, Fletcher JJ, Dharnidharka V, Gorman G, Lechner B, Nee R, Agodoa LY, Abbott KC. Incidence, predictors and associated outcomes of renal cell carcinoma in long-term dialysis patients. Urology. 2011;77:1271-1276.
6. Markovitz GS, Radhakrishnan J, Kammbham N, Valeri AM, Hines WH, D’Agati VD. Lithium nephrotoxicity: a progressive combined glomerular and tubulointerstitial nephropathy. J Am Soc Nephrol 2000; 11:1439–1448.
7. Farres MT, Ronco P, Saadoun D, Remy P, Vincent F, Khalil A, Le Blanche AF. Chronic Lithium Nephropathy: MR Imaging for Diagnosis. Radiology 2003; 229:570–574.
8. WHO classification of tumours: Pathology and genetics of tumours of the urinary system and male genital organs. Edited by Eble JN, Sauter G, Epstein JI, Sesterhenn IA. IARC press, Lyon 2004.
9. McCredie M, Stewart JH, Day NE. Different roles for phenacetin and paracetamol in cancer of the kidney and renal pelvis. Int J Cancer. 1993;53:245-249
10. Chow WH, McLaughlin JK, Mandel JS, Wacholder S, Niwa S, Fraumeni JF (Jr). Obesity and risk of renal cell cancer. Cancer Epidemiol Biomarkers Prev 1996;5:17-21.
11. Setiawan VW, Daniel O. Stram DO, Nomura AMY, Kolonel LN and Brian E. Henderson BE. Risk Factors for Renal Cell Cancer: The Multiethnic Cohort. Am. J. Epidemiol. 2007; 166: 932-940.

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Figure 1. Numerous cysts in the kidney (left panel) with part of a tumour in the right upper corner of the kidney. A large haemorrhagic tumour in the upper pole of the kidney (right panel) with renal vein invasion by tumour (arrow).

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Figure 2. Kidney with simple cysts, tubular atrophy and interstitial fibrosis with mild inflammation (left panel) and clear cell renal cell carcinoma within the renal vein, as arrowed in the right panel.

 

Date added to bjui.org: 02/12/2012

DOI: 10.1002/BJUIw-2012-062-web

 

Megameatus Intact Prepuce revisited

We present the case of a 41 year old male who was referred to the outpatient department with microscopic hematuria

Authors: Bourdoumis, Andreas; Kapoor, Sona; Bhanot, Shiv
Corresponding Author: Bourdoumis, Andreas

 

Introduction
Hypospadias is an abnormality in which the urethral opening is ectopically located on the ventrum of the penis as far down as in the scrotum or perineum. It occurs in 1 to 300 live births and presents with a 3 fold increasing incidence during the last 30 years, with recent reports linking the increased rate of hypospadias in boys born prematurely, small for gestational age, and boys with low birth weight.
The earliest medical text describing hypospadias dates back to the second century AD and was the work of Galen, the first to use the term. Failure of fusion of the urethral folds is thought to be primarily responsible. The urethral folds coalesce in the midline from base to tip, forming a tubularized penile urethra and median scrotal raphe. The prepuce normally forms as a ridge of skin from the corona that grows circumferentially, fusing with the glans. Failure of fusion of the urethral folds in hypospadias impedes this process, and a dorsal hooded prepuce results. The glans penis is splayed open, while the shaft is associated with ventral shortening and curvature, called chordee. On rare occasions, a glanular cleft with intact prepuce may occur, which is termed the megameatus intact prepuce (MIP) variant.

Case Description
A 41 year old male was referred to the outpatient department with microscopic hematuria. He denied any lower urinary tract symptoms. During the physical examination, a small, benign prostate was identified, as well as a sub-coronal, wide external urethral meatus with a cleft deep granular groove (Figure 1). The patient, a father of two, denied ever having any symptoms from his apparent anatomic variant, hence he never sought medical advice. He denied any symptoms relating to urination, erection or ejaculation. Visual assessment of urinary stream showed no obstruction or spraying (Figure 2). Uroflowmetry revealed a Qmax of 16 ml/sec. His microscopic haematuria resolved without any intervention. The patient was reassured and discharged.

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Figure 1. Megameatus Intact Prepuce (MIP) variant of hypospadias in a 42 year old patient, a father of two, with the characteristic wide mouth sub-coronal meatus and intact, retracted foreskin.

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Figure 2. Good urine stream with intact prepuce covering the glans completely.

Discussion
As a rule of thumb, diagnosis and classification of hypospadias is based on the location of the abnormal urethral meatus. Megameatus intact prepuce variant is reported at a prevalence of 1 in 10000 patients, or 1% to 3% of hypospadias.1 Embryological origins are unclear, but it appears to be related to megalourethra.1, 2, 4

One has to be aware of the variant in order to avoid confusion. The features of the variant include a spatulated glans with a distal, wide patulous meatus at the deep subcoronal groove, an intact foreskin, a very thin corpus spongiosum and no ventral chordee. If curvature is present, it is invariably dorsal.1 Classic hypospadias is characterized by the triad of ventral urethra defect, absence of dorsal prepuce and chordee.

Clinical examination and prompt identification of MIP cannot be overemphasised. As recommended, before any circumcision, the foreskin should be fully retracted and the glans and urethral meatus inspected. This is especially true for populations were circumcision is considered as religious practice. Litigation often follows for what appeared initially to be iatrogenic hypospadias after circumcision. 2 A recommendation was thus made for circumcision to take place only after correction of hypospadias. 2

Nonomura et al.3reported excellent cosmetic and functional results using perimeatal-based flaps in previously circumcised patients. Similarly, others have used the MAGPI technique in distal glanular MIP, and also with the GAP technique for the remaining glanular defects with equally satisfactory results.4 Bar-Yosef et al. 5 used both the GAP and TIP urethroplasty with an 83% satisfactory cosmetic and functional result in selected cases. Prior circumcision did not negatively affect the results of subsequent urethroplasty in patients with anterior, distal penile, and the MIP variant of hypospadias. 4

Our case represents one of five patients, who have been identified in the outpatient department during the past 8 years. They constitute, to our knowledge, the first report in the literature of satisfactory functional outcome without the need for surgery. Preputial development seems to be independent from the formation of the glandular urethra. Based on this fact, we propose that the intact prepuce plays the role of a funnel, directing urine as well as semen appropriately. The absence of chordee precludes painful erections, difficulty in vaginal insertion as well as deflection of ejaculate. Given the successful parity and lack of urinary symptoms and in the absence of psychological stress of having a genital anomaly on behalf of the patient, one is tempted to question the value of reconstructive surgery in this subset of patients. As mentioned previously, reconstruction is feasible even in the event of prior circumcision. It is imperative to consider a patient’s journey through these procedures which are complex plastic surgery reconstructions of the urinary tract, highly demanding in skill and expertise, with serious complications, including fistula, diverticulum, meatal stenosis and stricture. Patients with MIP variant of hypospadias should receive an extensive consultation focusing on appropriate indications for treatment based on clinical and functional findings. Active participation of the patient in the decision making process is essential. The desired surgical reconstruction should take place in centers of excellence by dedicated reconstructive urological surgeons.

Conclusion
Diagnosis and management of MIP can be particularly challenging. Timely identification of the variant provides for appropriate treatment and planning in order to achieve the desirable effect for the patient. Prospective data, although challenging to collect, could provide more robust evidence regarding the real need for surgical reconstruction of this rare form of hypospadias.

References
1. Duckett JW, Keating MA. Technical challenge of the megameatus intact prepuce hypospadias variant: the pyramid procedure. J Urol. 1989 Jun;141(6):1407-9.
2. Peretz D, Westreich M. Pseudo-iatrogenic hypospadias: the megameatus intact-prepuce hypospadias variant. Plast Reconstr Surg. 2003 Mar;111(3):1182-5.
3. Nonomura K, Kakizaki H, Shimoda N, Koyama T, Murakumo M, Koyanagi T.
Surgical repair of anterior hypospadias with fish-mouth meatus and intact prepuce based on anatomical characteristics. Eur Urol. 1998 Oct;34(4):368-71
4. Pieretti RV, Pieretti A, Pieretti-Vanmarcke R. Circumcised hypospadias. Pediatr Surg Int. 2009 Jan;25(1):53-5. Epub 2008 Oct 11.
5. Bar-Yosef Y, Binyamini J, Mullerad M, Matzkin H, Ben-Chaim J.Megameatus intact prepuce hypospadias variant: application of tubularized incised plate urethroplasty. Urology. 2005 Oct;66(4):861-4

 

Date added to bjui.org: 30/11/2012
DOI: 10.1002/BJUIw-2012-084-web

 

Intra-operative pulmonary embolism secondary to dislodged tumor thrombus from synovial sarcoma of the kidney

Primary synovial sarcoma (PSS) of the kidney is a rare occurrence, with preoperative diagnosis difficult to distinguish from other renal neoplasms. Presented here is the case of a patient who underwent elective left laparoscopic nephrectomy for a 6-cm renal mass. Intra-operatively, she decompensated into PEA from multiple pulmonary emboli secondary to dislodged tumor thrombus requiring emergent sternotomy and embolectomy after placing the patient on cardiopulmonary bypass. Patient survived the surgery and has been placed on adriamycin, ifosfomide, mesna (AIM) chemotherapy. However, no definitive treatment of renal synovial sarcoma exists and prognosis is bleak despite primary surgical resection, chemotherapy, or radiotherapy.

Authors: Zhao, Philip;  Mikkilineni, Nina; Johnson, Kelly; Ankem, Murali
Corresponding Author: Philip Zhao MD, Division of Urology, Department of Surgery, UMDNJ-Robert Wood Johnson Medical School, New Brunswick, NJ 08901, USA. Email: [email protected]

 


Abstract

Primary synovial sarcoma (PSS) of the kidney is a rare occurrence, with preoperative diagnosis difficult to distinguish from other renal neoplasms. Presented here is the case of a patient who underwent elective left laparoscopic nephrectomy for a 6-cm renal mass. Intra-operatively, she decompensated into PEA from multiple pulmonary emboli secondary to dislodged tumor thrombus requiring emergent sternotomy and embolectomy after placing the patient on cardiopulmonary bypass. Patient survived the surgery and has been placed on adriamycin, ifosfomide, mesna (AIM) chemotherapy. However, no definitive treatment of renal synovial sarcoma exists and prognosis is bleak despite primary surgical resection, chemotherapy, or radiotherapy.

Introduction

Synovial sarcoma of the kidney was reported first by Faria et al. and Argani et al. in the beginning of the twenty-first century as a distinct subset of the embryonal sarcoma of the kidney [1,2]. Although synovial tumors occur primarily in the para-articular joints of extremities, they have been encountered in the lungs, mediastinum, heart, pharynx/larynx, kidneys, and other organs. Primary renal synovial sarcoma is extremely rare, with less than thirty-five cases reported in the literature [3, 4]. Its diagnosis cannot be based on imaging studies alone – it looks similar to other renal neoplasms on CT or MRI scans, but must be confirmed by molecular and cytogenetic analysis demonstrating the chromosomal translocation of t(X;18)(p11;q11) [2]. The disease is clinically aggressive and because of the paucity of cases, there are no optimal treatment plans except complete resection and a combination of adjuvant chemotherapy or radiotherapy [5]. We present a case of PSS of the kidney that, during nephrectomy, embolized the pulmonary arterial system, causing catastrophic decompensation, requiring the patient to undergo cardiopulmonary bypass with emergency embolectomy.

Case Report

A 44-year-old female initially presented in March 2011 with gross haematuria and left flank pain, and workup revealed a heterogeneous 6 x 5 cm left upper pole renal mass. A metastatic evaluation was negative. The patient had no prior medical or surgical history and was a non-smoker. Anxious with her diagnosis, she elected to have an elective laparoscopic nephrectomy performed at the earliest available time. An abdominopelvic CT scan with IV contrast (Figure 1) done two weeks prior to date of surgery showed the tumor without any extensive invasion outside of the kidney and with only an element of left renal vein involvement, that id extend into the vena cava. In fact, the edge of the tumor thrombus was about 1.5 cm from the IVC.
The patient underwent a left laparoscopic radical nephrectomy in April 2011 in the standard manner. After placing the patient in the right lateral decubitus position and creating the pneumoperitoneum, surgery proceeded uneventfully until dissection of the renal hilum. The left renal artery was identified and stapled without difficulty. The left gonadal, adrenal, and lumbar veins were all identified and ligated. The left renal vein was then dissected and a vascular stapler was slowly closed flush to the IVC and then pushed laterally towards the kidney before closing completely and firing. Immediately after firing the vascular stapler, the patient became bradycardic, hypotensive, and hypoxic, eventually leading to PEA. A catastrophic event was suspected and the surgery immediately ceased with deflation of the pneumoperitoneum, removal of all laparoscopic ports, and placement of the patient in the supine position. Although a tension pneumothorax was initially suspected, TEE confirmed significant right ventricular (RV) distension and signs of pulmonary embolism. Aggressive cardiopulmonary resuscitation began and cardiothoracic surgery was emergently consulted for cardiopulmonary bypass and embolectomy.
A femoral-femoral bypass was established and a standard sternotomy was performed with the pericardium opened. The RV was significantly dilated and there was high pulmonary arterial pressure by palpation. The right atrium (RA) and ascending aorta were both cannulated and the femoral lines were switched to the aortic arterial line and venous outflow from the RA to reestablish bypass. The patient was then systemically cooled to approximately 31 degrees Celsius. The pulmonary artery was then opened in a longitudinal direction and multiple clots as well as tumor thrombi were then removed from all the pulmonary arterial segmental branches on both the left and right sides (Figure 2). A frozen section sent off revealed spindle cell tumor. After all clots and tumors had been removed, the pulmonary artery was closed along with the chest, while keeping a chest tube in place. The nephrectomy was completed by making a left subcostal incision and removing the entire left kidney. A JP drain was left in place and the abdomen was then closed.
The patient remained intubated in the ICU and was weaned off the ventilator on post-operative day three. The rest of her hospitalization was complicated by acute renal failure, from which she was able to recover. Upper extremity DVT also developed, for which she was placed on therapeutic enoxaparin. The patient was ultimately discharged from the hospital to begin outpatient chemotherapy with adriamycin, ifosfomide, mesna (AIM). Pathological confirmation of primary monophasic synovial sarcoma of the kidney was obtained, with molecular studies detecting SYT-SSX2 fusion transcripts consistent with t(X,18) translocation. Although the fascial margins of the tumor were negative, there was extensive lymphovascular invasion and extension into the renal sinus. Final pathology of the pulmonary thrombi demonstrated metastatic synovial sarcoma.
A PET scan done a month after patient’s operation revealed extensive areas of hypermetabolic nodules and soft tissue densities in the left renal bed and significant lymphadenopathy in the retroperitoneum coursing down the left ureter consistent with metastatic spread of the cancer. A repeat CT scan of the chest also revealed a non-occlusive filing defect at the bifurcation of the right main pulmonary artery suggesting in situ thrombus, as well as new lytic osseous lesions in the intervertebral bodies suspicious for osseous metastases.

Discussion

Primary synovial sarcoma of the kidney is rare and tumors presenting with IVC thrombus are fewer still-there are only four cases reported in the literature [4]. Our case is the first report of a PSS thrombus dislodging and embolizing the pulmonary arterial tree, causing immediate decompensation and requiring emergency sternotomy and embolectomy after placing the patient on cardiopulmonary bypass. The case is especially interesting and highlights the aggressiveness of this tumor variant because only two weeks prior to surgery, a CT scan showed a grossly patent IVC with minimal tumor invasion of the left renal vein. There was a wide discrepancy between the amount of tumor burden removed from the pulmonary arteries and the small tumor thrombus in the renal vein identified on CT scan. A case may have been made for additional imaging (MRI) to better define the level of tumor thrombus. However, recent literature shows that CT and MRI both detect and assess caval thrombus with similar sensitivity and specificity –78% and 72%, 88% and 76%, respectively [6]. In retrospect, we could have used an intra-operative ultrasound to better delineate the exact edge of the tumor thrombus in the renal vein prior to ligation. We did not because of the grossly patent IVC on recent imaging and because the attending surgeon felt confident in his technique of closing the stapler partway and then “milking” any thrombus away from the IVC before firing.
There is no preoperative method to diagnose PSS without tissue samples for analysis. Differential diagnosis includes adult Wilms tumor, renal cell carcinomas, mesoblastic nephromas, primitive neuroectodermal tumors, primary renal rhabdosarcomas, and transitional cell carcinomas. Using reverse transcriptase polymerase chain reaction (RT-PCR), Argani et al. were able to identify the presence of SYT-SSX gene fusion from t(X; 18) [2]. Although five different variants of the SSX gene have been identified, only SSX1 and SSX2 have been shown to fuse with the SYT gene to create the biphasic and monophasic forms of PSS, respectively. Biphasic PSS contains both glandular and spindle epithelial elements while the monophasic form is only composed of spindle cells [4, 7]. The only consistent immunoreactive marker for PSS is vimentin, which was markedly positive in our patient’s tumor.
Although surgery is the mainstay of therapy, it is not enough alone to augment the already poor prognosis associated with renal PSS. Although there have been limited success with stereotactic body radiotherapy and ifosamide and doxorubicin-based chemotherapy with case reports showing complete remission after post-nephrectomy metastasis to the lung, most patients do not live beyond two years [8, 9, 10].

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Fig. 1

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Fig. 2

References

1. Faria P, Argani P, Epstein J. Primary synovial sarcoma of the kidney: A molecular subset of so called embryonal renal sarcoma. Mod Pathol Am. 1999;12:94.
2. Argani P, Faria PA, Epstein JI, Reuter VE, Perlman EJ, Beckwith JB, Ladanyi M. Primary renal synovial sarcoma: molecular and morphologic delineation of an entity previously included among embryonal sarcomas of the kidney. Am J Surg Pathol. 2000 Aug;24(8):1087-96.
3. Gabilondo F, Rodríguez F, Mohar A, Nuovo GJ, Domínguez-Malagón H. Primary synovial sarcoma of the kidney: corroboration with in situ polymerase chain reaction. Ann Diagn Pathol. 2008;12:134-7.
4. Dassi V, Das K, Singh BP, Swain SK. Primary synovial sarcoma of kidney: A rare tumor with an atypical presentation. Indian J Urol. 2009 Apr-Jun;25(2): 269-271.
5. Kataria T, Janardhan N, Abhishek A, Sharan GK, Mitra S. Pulmonary metastasis from renal synovial sarcoma treated by stereotactic body radiotherapy: a case report and review of the literature. J Cancer Res Ther. 2010 Jan-Mar;6(1):75-9.
6. Hallscheidt PJ, Fink C, Haferkamp A, Bock M, Luburic A, Zuna I, Noeldge G, Kauffmann G. Preoperative staging of renal cell carcinoma with inferior vena cava thrombus using multidetector CT and MRI: prospective study with histopathological correlation. J Comput Assist Tomogr. 2005 Jan-Feb;29(1):64-8.
7. Skytting B, Nilsson G, Brodin B, Xie Y, Lundeberg J, Uhlen M, et al. A novel fusion gene, SYT-SSX4: In synovial sarcoma. J Natl Cancer Inst. 1999;91:974-5.
8. Kataria T, Janardhan N, Abhishek A, Sharan GK, Mitra S. Pulmonary metastasis from renal synovial sarcoma treated by stereotactic body radiotherapy: a case report and review of the literature. J Cancer Res Ther. 2010 Jan-Mar;6(1):75-9.
9. Park SJ, Kim HK, Kim CK, Park SK, Go ES, Kim ME, et al. A case of renal synovial sarcoma: Complete remission was induced by chemotherapy with Doxorubicin and Ifosamide. Korean J Intern Med. 2004;19:62-5.
10. Long JA, Dinia EM, Saada-Sebag G, Cyprien J, Pasquier D, Thuillier C, Terrier N, Boillot B, Descotes JL, Rambeaud JJ. Primitive renal synovial sarcoma: a cystic tumor in young patients. Prog Urol. 2009 Jul;19(7):474-8.

 

Date added to bjui.org: 26/11/2012

DOI: 10.1002/BJUIw-2012-070-web

 

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