Tag Archive for: nephrectomy

Posts

Article of the month: Understanding volume–outcome relationships in nephrectomy and cystectomy for cancer: evidence from the UK Getting it Right First Time programme

Every month, the Editor-in-Chief selects an Article of the Month from the current issue of BJUI. The abstract is reproduced below and you can click on the button to read the full article, which is freely available to all readers for at least 30 days from the time of this post.

In addition to the article itself, there is an editorial written by a prominent member of the urological community and a video prepared by the authors; we invite you to use the comment tools at the bottom of each post to join the conversation. 

If you only have time to read one article this month, we recommend this one. 

Understanding volume–outcome relationships in nephrectomy and cystectomy for cancer: evidence from the UK Getting it Right First Time programme

William K. Gray*, Jamie Day*, Tim W. R. Briggs* and Simon Harrison*

*Getting it Right First Time Programme, NHS England and NHS Improvement, London, UK and Pinderfields Hospital, Mid Yorkshire Hospitals NHS Trust, Wakefield, UK

Abstract

Objectives

To investigate volume–outcome relationships in nephrectomy and cystectomy for cancer.

Materials and Methods

Data were extracted from the UK Hospital Episodes Statistics database, which records data on all National Health Service (NHS) hospital admissions in England. Data were included for a 5‐year period (April 2013–March 2018 inclusive) and data on emergency and paediatric admissions were excluded. Data were extracted on the NHS trust and surgeon undertaking the procedure, the surgical technique used (open, laparoscopic or robot‐assisted) and length of hospital stay during the procedure. This dataset was supplemented by data on mortality from the UK Office for National Statistics. A number of volume thresholds and volume measures were investigated. Multilevel modelling was used to adjust for hierarchy and confounding factors.

Results

Data were available for 18 107 nephrectomy and 6762 cystectomy procedures for cancer. There was little evidence of trust or surgeon volume influencing readmission rates or mortality. There was some evidence of shorter length of hospital stay for high‐volume surgeons, although the volume measure and threshold used were important.

Conclusions

We found little evidence that further centralization of nephrectomy or cystectomy for cancer surgery will improve the patient outcomes investigated. It may be that length of stay can be optimized though training and support for lower‐volume centres, rather than further centralization.

 

Editorial: All for one, one for all: is centralisation the way to go?

The need to centralise complex surgical procedures in large centres remains at the core of many health policy discussions. Much of the debate is focussed on three main aspects: (i) outcomes, (ii) costs and (iii) accessibility. Gray et al. [1] recently noted that increasing centralisation may be unnecessary for invasive procedures such as nephrectomy and cystectomy. Specifically, they noted almost no difference in outcomes of high‐volume centralised centres and those with lower throughput. Their findings go against most of the current literature on the volume–outcomes relationship, which generally reports a correlation between a hospital’s volume of procedures and improved healthcare outcomes. One could ask what factors specific to their analysis could explain the different observations. For one, the healthcare system in the UK may (and likely) operate in ways different from other European and USA‐based healthcare systems, from which most of the current data are derived. Healthcare in the UK may already be organised in such a way that further centralisation may not improve outcomes, which the authors allude to in their conclusions. Differences in methodology may explain their findings, e.g. their use of multilevel modelling, testing specific incremental volume cutoffs, etc. Outcome selection may play a role as well; length of stay and re‐admissions may vary more according to organisational factors rather than individual surgeon expertise.

Regardless of their findings, we would argue that there are other tangible benefits to centralisation, which extend well beyond ‘better outcomes’. For instance, the management of the modern oncological, and urological, patient is critically dependent on a multidisciplinary team. The inherent multidisciplinary nature of large centres facilitates patients receiving their entire course of treatment at the same place. This enhances the continuity and efficiency of care, both of which are undoubtedly hampered in small peripheral centres that ultimately depend on referrals to larger facilities for advanced care for the most complex patients.

This ties into yet another major advantage of centralised centres, which is the ease of access to research. For instance, our affiliated cancer centre runs >1100 active clinical trials, 42 of which pertain to advanced urological diseases. Such trials provide access to otherwise unavailable therapies and enhance the production, diffusion, and application of knowledge.

In touting the many benefits of centralisation, one would imagine it comes at a significant cost. While this may have been true in the past, recent data comparing the higher‐volume teaching hospitals to lower‐volume non‐teaching centres suggest that centralisation actually decreases the 30‐day hospital costs and have similar costs at 90 days compared with non‐teaching hospitals [2]. Similar trends were also seen with radical cystectomies [3] and prostatectomies [4], showing that with the major urological procedures, centralisation is cost‐effective with at least the same outcomes as compared to peripheral centres.

A common objection to centralisation is that it forces many patients to travel long distances and that this in turn could introduce or worsen discrepancies in accessibility to care. If true, this would have profound social and economic consequences for disadvantaged groups, as well as particularly fragile patients. Many centralised centres have developed approaches to ease the burdens of travelling from afar and, if patients can make the journey, the data suggest a survival advantage over those who are treated at peripheral centres. To this end, Vetterlein et al. [5] stratified >700 000 patients by risk class and demonstrated an overall survival benefit in those with all stages of prostate cancer. In the not‐too‐distant future, patient follow‐up can be shifted almost entirely to telemedicine, which can further alleviate travel burdens.

Our aim is not to promote a system of oncological care based solely at centralised hubs. However, to suggest that all care should be distributed equally across all centres seems unrealistic and may have devastating consequences, particularly for those with advanced disease. We strongly advocate the treatment of complex disease at high‐volume, centralised centres and suggest better use of an impartial classification of what constitutes a ‘complex’ disease. Therefore, one answer to this problem is broadly represented by the redistribution of the different surgical procedures amongst the hospitals.

by Daniele Modonutti, Venkat M. Ramakrishnan and Quoc‐Dien Trinh

 

References

  1. Gray WKDay JBriggs TWHarrison SUnderstanding volume‐outcome relationships in nephrectomy and cystectomy for cancer: evidence from the UK Getting it Right First Time programme. BJU Int 2020125234– 43
  2. Burke LGKhullar DZheng JFrakt ABOrav EJJha AKComparison of costs of care for medicare patients hospitalized in teaching and nonteaching hospitals. JAMA Netw Open 20192: e195229
  3. Leow JJReese STrinh QD et al. Impact of surgeon volume on the morbidity and costs of radical cystectomy in the USA: a contemporary population‐based analysis. BJU Int 2015115713– 21
  4. Gershman BMeier SKJeffery MM et al. Redefining and contextualizing the hospital volume‐outcome relationship for robot‐assisted radical prostatectomy: implications for centralization of care. J Urol 201719892– 9
  5. Vetterlein MWLöppenberg BKarabon P et al. Impact of travel distance to the treatment facility on overall mortality in US patients with prostate cancer. Cancer 20171233241– 52

 

 

 

Video: Understanding volume–outcome relationships in nephrectomy and cystectomy for cancer: evidence from the UK Getting it Right First Time programme

Understanding volume–outcome relationships in nephrectomy and cystectomy for cancer: evidence from the UK Getting it Right First Time programme

Abstract

Objectives

To investigate volume–outcome relationships in nephrectomy and cystectomy for cancer.

Materials and Methods

Data were extracted from the UK Hospital Episodes Statistics database, which records data on all National Health Service (NHS) hospital admissions in the England. Data were included for a 5‐year period (April 2013–March 2018 inclusive) and data on emergency and paediatric admissions were excluded. Data were extracted on the NHS trust and surgeon undertaking the procedure, the surgical technique used (open, laparoscopic or robot‐assisted) and length of hospital stay during the procedure. This dataset was supplemented by data on mortality from the UK Office for National Statistics. A number of volume thresholds and volume measures were investigated. Multilevel modelling was used to adjust for hierarchy and confounding factors.

Results

Data were available for 18 107 nephrectomy and 6762 cystectomy procedures for cancer. There was little evidence of trust or surgeon volume influencing readmission rates or mortality. There was some evidence of shorter length of hospital stay for high‐volume surgeons, although the volume measure and threshold used were important.

Conclusions

We found little evidence that further centralization of nephrectomy or cystectomy for cancer surgery will improve the patient outcomes investigated. It may be that length of stay can be optimized though training and support for lower‐volume centres, rather than further centralization.

View more videos

Visual abstract: Understanding volume–outcome relationships in nephrectomy and cystectomy for cancer: evidence from the UK Getting it Right First Time programme

See more infographics

Article of the week: Prognostic evaluation of perinephric fat, renal sinus fat, and renal vein invasion for patients with pathological stage T3a clear‐cell RCC

Every week, the Editor-in-Chief selects an Article of the Week from the current issue of BJUI. The abstract is reproduced below and you can click on the button to read the full article, which is freely available to all readers for at least 30 days from the time of this post.

In addition to the article itself, there is an editorial written by a prominent member of the urological community. These are intended to provoke comment and discussion and we invite you to use the comment tools at the bottom of each post to join the conversation. 

If you only have time to read one article this week, it should be this one.

Prognostic evaluation of perinephric fat, renal sinus fat, and renal vein invasion for patients with pathological stage T3a clear‐cell renal cell carcinoma

Paras H. Shah*, Timothy D. Lyon*, Christine M. Lohse, John C. Cheville,
Bradley C. Leibovich*, Stephen A. Boorjian* and R. Houston Thompson*
 
*Department of Urology, Department of Health Sciences Research, and
Department of Pathology and Laboratory Medicine, Mayo Clinic, Rochester, MN, USA
 

 

Read the full article

Abstract

Objective

To investigate the prognostic significance of various patterns of extrarenal extension that comprise pathological stage T3a clear‐cell renal cell carcinoma (ccRCC) amongst patients undergoing nephrectomy for non‐metastatic disease.

Patients and Methods

A retrospective review of 563 patients who underwent radical nephrectomy for pathologically confirmed T3aN0/NxM0 ccRCC between 1970 and 2011 was performed. All pathological slides were re‐reviewed by one urological pathologist. Associations of patterns of extrarenal extension (perinephric fat [PF], renal sinus fat [SF], and renal vein [RV], in isolation or in any combination) with disease progression, cancer‐specific mortality (CSM), and all‐cause mortality were evaluated on multivariable analyses.

Fig. 1. Progression-free survival stratified by type of extrarenal extension

Results

Overall, PF invasion, renal SF invasion, and RV tumour thrombus were present in 144 (26%), 51 (9%), and 163 (29%) patients, respectively, with multiple patterns of extrarenal extension identified in 205 (36%) patients. There were no significant differences in survival outcomes for isolated involvement of PF, renal SF, or RV. However, patients with multiple patterns of extrarenal extension were at significantly increased risk of disease progression (hazard ratio [HR] 1.31, 95% confidence interval [CI] 1.04–1.65; P = 0.020), CSM (HR 1.64, 95% CI 1.27–2.12; P < 0.001), and all‐cause mortality (HR 1.32, 95% CI 1.08–1.61; P = 0.008).

Conclusions

The presence of multiple patterns of extrarenal extension is associated with a higher risk of disease progression and cancer‐related death after radical nephrectomy compared to isolated involvement of the PF, renal SF, or RV, which carry similar prognostic weight. If validated, these findings may help refine risk stratification of non‐metastatic T3a RCC by distinguishing patients with multiple vs one pattern of extrarenal extension.

 

Editorial: Does knowing the risk of relapse in localized renal cell carcinoma matter?

Shah et al. [1] report a retrospective analysis from the Mayo Clinic investigating the prognostic significance of different patterns of pathological T3a clear‐cell RCC in patients who underwent radical nephrectomy for localized disease. There was no difference in disease progression, cancer‐specific mortality or all‐cause mortality when comparing isolated perinephric fat invasion vs isolated renal sinus fat invasion vs isolated renal vein invasion. Multiple sites of extra‐renal extension compared with one site, however, was independently associated with an increased risk of disease progression (hazard ratio [HR] 1.31, P = 0.02), death from RCC (HR 1.64, P < 0.001) and all‐cause mortality (HR 1.32, P = 0.008) when adjusting for multiple key variables including age, tumour size, grade, presence of coagulative tumour necrosis and sarcomatoid differentiation. The authors incorporated multiple sites of extra‐renal extension vs one site into three RCC prognostic models: SSIGN score, UISS and MSKCC nomogram. After controlling for these three predictive tools independently, multiple sites of extra‐renal disease predicted progression, death from RCC and all‐cause death. These data suggest that risk stratification for pT3aN0MO clear‐cell RCC is improved by differentiating multiple vs one site of extra‐renal extension.

Does an improved ability to predict recurrence and mortality increase the likelihood of cure in high‐risk localized RCC patients in 2018? Unfortunately, the answer is no. Ideally, prognostic models would identify patients at sufficient risk to consider adjuvant therapy, which would increase cure rates by eradicating micro‐metastatic disease with an acceptable toxicity. Regrettably, in RCC management there are no well‐established post‐surgical therapies that improve cure rates. The deficiency of established adjuvant therapies is not attributable to a lack of investigative trials. In the era before vascular endothelial growth factor receptor (VEGFR) targeting, adjuvant vaccines, immunotherapies and other systemic therapies failed to demonstrate improved recurrence‐free (RFS) or overall survival (OS) [2]. The efficacy of VEGFR‐targeted therapies in the metastatic setting re‐energized the hope for adjuvant therapy in patients with high‐risk localized RCC after surgical resection in the past two decades. The results to date have been disappointing. To date, three trials (ASSURE, PROTECT and S‐TRAC) have been completed, comparing oral VEGFR tyrosine kinase inhibitors with placebo in high‐risk localized clear‐cell RCC, with disease‐free survival (DFS) as the primary endpoint [3,4,5]. ASSURE and PROTECT showed no difference in RFS or OS [3,4,5]. S‐TRACT demonstrated an improvement in DFS but not in OS [4]. A pooled analysis of these three trials also failed to demonstrate improved DFS or OS with adjuvant VEGFR‐targeted therapy [6]. Significant side effects with discontinuation of adjuvant therapy occurred in 28–45% of patients as a result of drug‐related toxicity [6]. Trials investigating immune checkpoint inhibitors have yet to be published and, with the established efficacy of these drugs in the metastatic setting, hope still remains for adjuvant therapy in resected high‐risk localized RCC.

If the current literature does not support adjuvant therapy for resected high‐risk RCC, does knowing the risk of relapse alter surveillance? National Comprehensive Cancer Network guidelines for resected stage III RCC recommend chest and abdominal imaging within 3–6 months, along with subsequent chest and abdominal imaging every 3–6 months for 3 years, and then annually up to 5 years. Although the ideal schedule for surveillance imaging is unknown, further characterizing of the risk of relapse in high‐risk localized RCC would not be likely to affect this schedule significantly.

Although knowing the risk of relapse in high‐risk localized RCC does not help management in 2018, there is still a value to enhancing our prognostic tools. For one, our prognostic tools help clinicians counsel patients appropriately about their risk of recurrence. In addition, enhanced prognostic tools will assist in selecting appropriate patients with high‐risk localized RCC for future clinical trials of adjuvant therapy and also help us understand the results when comparing cohorts within and between trials.

References

  1. Shah PH, Lyon TD, Lohse CM. Prognostic evaluation of perinephric fat, renal sinus fat, and renal vein invasion for patients with pathologic stage T3a clear cell renal cell carcinoma. BJU Int 2019; 123: 270–6
  2. Scherr AJO, Lima JPSN, Sasse EC et al. Adjuvant therapy for locally advanced renal cell cancer: a systematic review with meta‐analysis. BMC Cancer 2011; 11: 115–21
  3. Haas N, Manola J, Uzzo R et al. Adjuvant sunitinib or sorafenib for high‐risk, non‐metastatic renal‐cell carcinoma (ECOG‐ACRIN E2805): a double‐blind, placebo‐controlled, randomised, phase 3 trial. Lancet 2016; 387: 2008–16
  4. Ravaud A, Motzer RJ, Pandha HS et al. Adjuvant sunitinib in high‐ risk renal‐cell carcinoma after nephrectomy. N Engl J Med 2016; 375: 2246–54
  5. Motzer RJ, Haas NB, Donskov F et al. Randomized phase III trial of adjuvant pazopanib versus placebo after nephrectomy in patients with localized or locally advanced renal cell carcinoma. J Clin Oncol 2017; 35: 3916–23
  6. Sun M, Marconi L, Eisen T et al. Adjuvant vascular endothelial growth factore‐targeted therapy in renal cell carcinoma. Eur Urol 2018; 74: 611–20

 

Article of the Week: Management and Outcomes of RMC

Every Week the Editor-in-Chief selects an Article of the Week from the current issue of BJUI. The abstract is reproduced below and you can click on the button to read the full article, which is freely available to all readers for at least 30 days from the time of this post.

In addition to the article itself, there is an accompanying editorial written by a prominent member of the urological community. This blog is intended to provoke comment and discussion and we invite you to use the comment tools at the bottom of each post to join the conversation.

Finally, the third post under the Article of the Week heading on the homepage will consist of additional material or media. This week we feature a video discussing the paper.

If you only have time to read one article this week, it should be this one.

Management and outcomes of patients with renal medullary carcinoma: a multicentre collaborative study

Amishi Y. Shah*, Jose A. Karam*, Gabriel G. Malouf, Priya Rao*, Zita D. Lim*, Eric Jonasch*, Lianchun Xiao*, Jianjun Gao*, Ulka N. VaishampayanDaniel Y. Heng§, Elizabeth R. Plimack, Elizabeth A. Guancial**, Chunkit Fung**, Stefanie R. Lowas
††, Pheroze Tamboli*, Kanishka Sircar*, Surena F. Matin*, W. Kimryn Rathmell§§, Christopher G. Wood* and Nizar M. Tannir*

 

*MD Anderson Cancer Center, Houston, TX, USA, Groupe Hospitalier Pitie-Salpetriere, University Pierre and Marie Curie,
Paris, France, Karmanos Cancer Center, Detroit, MI, USA, §Tom Baker Cancer Center, Calgary, Canada, Fox Chase Cancer Center, Philadelphia, PA, **University of Rochester, Rochester, NY, ††University of Nebraska Medical Center and
Childrens Hospital and Medical Center, Omaha, NE, and §§Vanderbilt-Ingram Cancer Center, Nashville, TN, US

 

Read the full article

Abstract

Objective

To describe the management strategies and outcomes of patients with renal medullary carcinoma (RMC) and characterise predictors of overall survival (OS).

Patients and Methods

RMC is a rare and aggressive malignancy that afflicts young patients with sickle cell trait; there are limited data on management to date. This is a study of patients with RMC who were treated in 2000–2015 at eight academic institutions in North America and France. The Kaplan–Meier method was used to estimate OS, measured from initial RMC diagnosis to date of death. Cox regression analysis was used to determine predictors of OS.

Results

In all, 52 patients (37 males) were identified. The median (range) age at diagnosis was 28 (9–48) years and 49 patients (94%) had stage III/IV. The median OS for all patients was 13.0 months and 38 patients (75%) had nephrectomy. Patients who underwent nephrectomy had superior OS compared to patients who were treated with systemic therapy only (median OS 16.4 vs 7.0 months, P < 0.001). In all, 45 patients received chemotherapy and 13 (29%) had an objective response; 28 patients received targeted therapies, with 8-week median therapy duration and no objective responses. Only seven patients (13%) survived for >24 months.

Conclusions

RMC carries a poor prognosis. Chemotherapy provides palliation and remains the mainstay of therapy, but <20% of patients survive for >24 months, underscoring the need to develop more effective therapy for this rare tumour. In this study, nephrectomy was associated with improved OS.

Read more articles of the week

Editorial: New Strategies for Treating RMC

In the current issue of BJUI, Shah et al. [1] present a multi-institutional study of 52 patients with renal medullary carcinoma (RMC) collected over a 15-year period. This notoriously lethal and rare form of kidney cancer, associated with sickle cell trait and disease, usually affects young adults. In the study, the median age was 28 years, 94% of the patients presented with stage 3 or 4 disease, and the median overall survival was only 13 months. Nephrectomy, performed in 75% of patients, as opposed to systemic therapy alone, was associated with longer survival (16.4 vs 7.0 months). Of the 45 patients who received platinum- or carboplatinum-based systemic chemotherapy, 13 (29%) had an objective response, while there was no objective response in 28 patients treated with vascular endothelial growth factor-targeted agents, which are highly effective in conventional clear-cell carcinoma of the kidney. Only seven patients survived >2 years, with two long-term survivors at 5 and 9 years after nephrectomy and various combinations of systemic therapy.

Recent reports suggest new insight into this lethal form of kidney cancer, raising hope about the development of effective systemic agents. Calderaro et al. [2] used gene expression profiling, array genomic hybridization, and RNA and whole-exome sequencing to study frozen tissue in five patients with RMC. They reported an interchromosomal balanced translocation that disrupts the SMARCB1 gene, a tumour suppressor on chromosome 22 encoding BAF47 protein, which impairs the SWI/SNF complex regulating chromatin remodelling, which, in turn, leads to increased cyclin D transcription and downstream over-expression of the transcriptional regulator EZH2. EZH2 is the enzymatic subunit of the PRC2 complex and its histone methylation function. EZH2 also has a PRC2-independent role in transcriptional activation and can methylate a number of non-histone proteins. Over-expression of EZH2 can lead to cancer by changing expression of tumour suppressor (pRB) and DNA-damage repair genes. EZH2 over-expression and loss of function mutations are associated with a diverse group of cancers including liver, breast, prostate, endometrial, melanoma, bladder and lymphoma, none of which are as rare as RMC but in which targetable agents can be tested for their ability to disrupt this pathway [3]. Interestingly, SMARCB1 gene truncating and or deletion mutations have been reported in the equally rare and lethal paediatric rhabdoid tumour of the kidney [4] and loss of immunoexpression of SMARCB1 reported in the clinically aggressive collecting duct renal cancer, which is morphologically similar to and often difficult to distinguish from RMC [5].

A number of small-molecule compounds able to target both EZH2 and PRC2 complex are currently undergoing preclinical testing (i.e. DZNEP, E11, EP2005687), phase I trials (GSK126), and phase II trials (EPZ-648, Tazemetostat) [3]. A phase II multicentre study of tazemetostat, a selective small-molecule inhibitor of EZH2, is underway and accruing patients with rare tumours with abnormalities in this pathway, including synovial-cell sarcoma, RMC and rhabdoid tumour of the kidney, for which there are no standard therapies [6]. Contemporary genomic research has great potential to identify such critical oncogenic pathways, shared in both rare and more common malignancies, with the potential for effective drugs to be designed to improve the grave prognosis of RMC and related cancers.

Paul Russo
Weill Cornell School of Medicine Memorial Sloan Kettering Cancer Center New York NY USA

 

Read the full article

 

References

 

1 Shah AYKaram JAMalouf GG et al. Management and outcomes of patients with renal medullary carcinoma: a multicentre collaborative study. BJU Int2017; 120: 78292.

 

2 Calderaro JMasliah-Planchon JRicher W et al. Balanced translocations disrupting SMARCB1 are hallmark recurrent genetic alterations in renal medullary carcinomas. Eur Urol2016; 69: 105561.

 

3 Kim KHRoberts CWM. Targeting EZH2 in cancer. Nat Med 2016; 22: 128– 34

 

4 Versteege I, Sevenet NLange J et al. Truncating mutations of hSNF5/INI1 in aggressive paediatric cancer. Nature 1998; 394:2036

 

5 Elwood H1Chaux ASchultz L et al. Immunohistochemical analysis of SMARCB1/INI-1 expression in collecting duct carcinoma. Urology 2011;78: 474

 

 

Video: Management and Outcomes of RMC

Management and outcomes of patients with renal medullary carcinoma: a multicentre collaborative study

Amishi Y. Shah*, Jose A. Karam*, Gabriel G. Malouf, Priya Rao*, Zita D. Lim*, Eric Jonasch*, Lianchun Xiao*, Jianjun Gao*, Ulka N. VaishampayanDaniel Y. Heng§, Elizabeth R. Plimack, Elizabeth A. Guancial**, Chunkit Fung**, Stefanie R. Lowas
††, Pheroze Tamboli*, Kanishka Sircar*, Surena F. Matin*, W. Kimryn Rathmell§§, Christopher G. Wood* and Nizar M. Tannir*

 

*MD Anderson Cancer Center, Houston, TX, USA, Groupe Hospitalier Pitie-Salpetriere, University Pierre and Marie Curie,
Paris, France, Karmanos Cancer Center, Detroit, MI, USA, §Tom Baker Cancer Center, Calgary, Canada, Fox Chase Cancer Center, Philadelphia, PA, **University of Rochester, Rochester, NY, ††University of Nebraska Medical Center and
Childrens Hospital and Medical Center, Omaha, NE, and §§Vanderbilt-Ingram Cancer Center, Nashville, TN, US

 

Read the full article

Abstract

Objective

To describe the management strategies and outcomes of patients with renal medullary carcinoma (RMC) and characterise predictors of overall survival (OS).

Patients and Methods

RMC is a rare and aggressive malignancy that afflicts young patients with sickle cell trait; there are limited data on management to date. This is a study of patients with RMC who were treated in 2000–2015 at eight academic institutions in North America and France. The Kaplan–Meier method was used to estimate OS, measured from initial RMC diagnosis to date of death. Cox regression analysis was used to determine predictors of OS.

Results

In all, 52 patients (37 males) were identified. The median (range) age at diagnosis was 28 (9–48) years and 49 patients (94%) had stage III/IV. The median OS for all patients was 13.0 months and 38 patients (75%) had nephrectomy. Patients who underwent nephrectomy had superior OS compared to patients who were treated with systemic therapy only (median OS 16.4 vs 7.0 months, P < 0.001). In all, 45 patients received chemotherapy and 13 (29%) had an objective response; 28 patients received targeted therapies, with 8-week median therapy duration and no objective responses. Only seven patients (13%) survived for >24 months.

Conclusions

RMC carries a poor prognosis. Chemotherapy provides palliation and remains the mainstay of therapy, but <20% of patients survive for >24 months, underscoring the need to develop more effective therapy for this rare tumour. In this study, nephrectomy was associated with improved OS.

Read more articles of the week

© 2022 BJU International. All Rights Reserved.