Figure 2 Histologic appearance of the tumour showing Ganglioneuroma of urinary bladder:
Figure (2A)- Hemotoxyline and eosin stain, 40X, Arrow showing muscularis propria infiltration by the tumour
Figure (2B)- Hemotoxyline and eosin stain, 40X, Arrow showing numerous ganglion cells in a fibrillary background
Figure (2C)- 200X, S 100, Immunohistochemistry, Arrow showing S100 positivity in nucleus of tumour tissue, indicating neuronal origin
Figure (2D)- 200X, SMA, Immunohistochemistry, SMA negative in tumour tissue, indicating no smooth muscle differentiation. SMA positive only in blood vessel walls (arrow)
Immunohistochemistry with S100 was positive, while SMA (smooth muscle antigen) was negative. The patient recovered well. Follow up CECT scans at 3, 6 and 12 months showed no recurrence of tumour in the urinary bladder.
Ganglioneuroma is a benign neurogenic tumor arising from sympathetic ganglia. They are rare tumors that most frequently start in the autonomic nerve cells, which may be in any part of the body. Ganglioneuromas are thought to be the fully differentiated counterpart of neuroblastomas. They are highly differentiated benign tumors and are compatible with long-term disease free survival, even though surgical treatments are unsatisfactory (1). About 60% of all patients with these tumors are younger than 20 years, most being less than 10 years of age. There is a slight female preponderance. Since ganglioneuromas may release catecholaminergic peptides, surgeons should be aware of the possibility of hypertensive crisis during the surgery (2). Ganglioneuromas most frequently occur in the posterior mediastinum 43%, followed by the retroperitoneum 32% and the neck 8% (3). Ganglioneuromas involving the genitourinary tract are extremely rare but are potentially serious tumors. It consists of spindle cells, fascicles composed of neuritic processes, Schwann cells, perineural cells and numerous ganglion cells. Immunohistochemically they are characterized by reactivity with S100 and neuronal markers such as NSE (neuron specific enolase) and synaptophysin4. According to many authors, surgical excision is sufficient for the treatment. Partial cystectomy or radical cystectomy is the recommended treatment modality. Preoperative or postoperative chemotherapy or radiotherapy have no value in the treatment except when it is associated with ganglioneuroblastoma changes when there might be some role for chemotherapy. Even with residual disease, cessation of all other treatments and a close follow-up may be adequate (4).
Five cases of composite paraganglioma-ganglioneuroma of the urinary bladder (CPGUB) have been reported in the English-language literature. These cases showed no malignant features, such as extra-bladder infiltration and metastasis, and no recurrence in the short period of follow up. A case of malignant peripheral nerve sheath tumor of the bladder in a 57-year-old man with multiple neurofibromatosis type 1 has been reported. The patient had a recent history of a transurethrally resected bladder ganglioneuroma. A probable histogenetic association between these two extremely rare neoplasms is proposed (5). Isolated Ganglioneuroma of urinary bladder without any other systemic disease has not been reported in literature so far.
Ganglioneuroma is a rare tumor of urinary bladder. TURBT with resection of full thickness of bladder wall provided a complete resection of tumor in this case. TURBT can be a treatment option in such tumours as an alternative to partial cystectomy.
1. Hayes FA, Green AA, Rao BN. Clinical manifestations of ganglioneuroma. Cancer 1989;63:1211-4.
2. Moriwaki Y, Miyaka M, Yamamoto T, Tsuchida T, Takahashi. S, Hada T et al. Retroperitoneal ganglioneuroma: a case report and review of the Japanese literature. Intern Med 1992;31:82-5.
3. Jasinki RW, Samuels BI, Silver TM. Sonographic features of retroperitoneal ganglioneuroma. J. Ultrasound Med 1984;3:413-5.
4. Kleihues P, Cavenee WK. World Health Organization Classification of tumors, pathology and genetics of tumors of the nervous system. Lyon 2000;153-61.
5. Kalafatis P, Kavantzas N, Pavlopoulos PM, Agapitos E, Politou M, Kranides A. Malignant Peripheral Nerve Sheath Tumor of the Urinary Bladder in von Recklinghausen Disease. Urol Int 2002;69:156-9.
Date added to bjui.org: 04/10/2010