CASE REPORT Primary Retroperitoneal Transitional Cell Carcinoma – A Rare Clinical Entity Pramod Kumar Sharma, Rajan Kumar Sinha*, Subhabrata Mukherjee Keywords: primary; retroperitoneum; urothelial neoplasm; transitional cell carcinoma INTRODUCTION Transitional Cell Carcinoma (TCC) most commonly arises from the urinary bladder and less frequently from the upper urinary tract. We report a case of primary retroperitoneal TCC in a male patient. The entire urinary tract did not have any evidence of tumour. As far as we are concerned, only four such cases have been reported in the literature previously(1) and all of them were female. CASE DESCRIPTION A 30 year-old male patient belonging to Asian-Indian race presented with intermittent left flank pain for 2 months with normal abdominal examination findings. The patient was a farmer by occupation and did not have any his- tory of radiation exposure or any known family history of malignancy. Ultrasonography along with intravenous urography (IVU) (Figure 1A) revealed hydronephrotic left kidney suggestive of pelvi-ureteric junction obstruction (PUJO) and diuretic renogram showed obstructive drainage from the left kidney. However, on careful retrospective evaluation of IVU plates there was evidence of upper ureteric compression with mild lateral deviation of left ureter which we missed preoperatively. With preoperative diagnosis of left PUJO, the patient was taken up for pyeloplas- ty and on table retrograde pyelogram (pyelogram) exhibited patent ureter with jet sign at pelvis which collabo- 1Department of Urology, Calcutta National Medical College, Kolkata - 700014 ,India. *Correspondence: Department of Urology, Calcutta National Medical College, Kolkata - 700014 Phone: +91-9007205371. Email: rajan_rims@yahoo.co.in. Received March 2016& Accepted August 2016 Figure 1. (A) Intravenous urography revealing hydronephrotic left kidney suggestive of pelvi-ureteric junction obstruction. (B) Intraop- erative image showing a large retroperitoneal mass with lateral deviation of ureter. (C) Hematoxylin & Eosin stained picture of incisional biopsy of retroperitoneal mass showing transitional cell carcinoma under 400X magnification. (D) Computed Tomography scan of abdo- men showing mass encasing the great vessels. Case Report 2869 Vol 13 No 05 September-October 2016 2870 rated with our diagnosis. On exploration, a large mass was found in the retroperitoneum which was encasing the great vessels (Figure 1B). The ureter was arching over the mass with no evidence of primary PUJO. In- cisional biopsy from the mass was taken and double-J stent was inserted into left ureter via ureterotomy. His- topathological examination showed multiple pieces of greyish tissue on gross examination, and microscopical- ly it was suggestive of high grade urothelial carcinoma with area of necrosis and perineural invasion (Figure 1C). Considering that the mass could be a secondary from an unknown primary site within the urinary tract, Computed Tomography (CT) urogram, cystoscopy, urine for malignant cell cytology, bilateral ureteroscopy and retrograde pyelography were performed, but no ev- idence of urothelial carcinoma was found in the urinary tract. CT of abdomen showed a large mass encircling the aorta and compressing the inferior vena cava with areas of necrosis (Figure 1D). With the final diagno- sis of primary retroperitoneal TCC, chemotherapy with Gemcitabine and Cisplatin was started after consulta- tion with medical oncologists. Patient did not respond to the chemotherapy and his general condition deteri- orated. Moreover, a swelling was noticed in left side of neck after first cycle of chemotherapy. Fine needle aspiration cytology of that mass was suggestive of met- astatic deposits of malignant epithelial neoplasm. Fi- nally, 5 months after diagnosis he succumbed to death. DISCUSSION Adult retroperitoneal tumours are rare and constitute only 0.2-0.5% of all malignant tumours (2). They may arise from fat, areolar connective tissue, fascia, mus- cle, vascular tissue, nervous tissue, lymphatic tissue and urogenital ridge remnants. The two most common malignant primary tumours of the retroperitoneum are lymphosarcoma and liposarcoma (3). Amongst the ret- roperitoneal tumours, primary TCCs are extremely rare and carry a worst prognosis. Till date, only 4 cases have been reported and all of which were cystic pelvic mass occurring in female patients (1). Like ours, three out of those four patients had unresectable mass. This is the first case reported in male as per our best knowledge. Retroperitoneal tumours grow unrestricted be- cause of loose surrounding tissue. They are gen- erally asymptomatic until they grow to very large sizes. The symptoms are often due to obstruction or compression of the adjacent structures. As they grow to a very large size before being detected, only 25% of these tumours can be completely excised (4). Primary retroperitoneal TCC is an extremely un- common entity. They are thought to originate from the remnants of urogenital ridge(5). Braasch et al.(6) reviewed 101 cases of retroperitoneal tumour of which only 3 cases were found to arise from urogen- ital ridge. However, Handfied-Jones et al.(7) were of the opinion that majority of retroperitoneal tu- mours arise from urogenital apparatus. Hansmann et al.(8) also supported their view of similar origin. Koyanagi et al.(9) were first to report primary TCC of re- troperitoneum arising from cloacal cyst. Later Gupta et al.(4) and Basu et al.(2) also reported primary retroperito- neal TCC. All these above 3 cases died within 2 years of diagnosis. Radiotherapy alone was used in 2 cases, while surgery was the primary modality of treatment in one of them followed by adjuvant chemotherapy. Recently, Ichinobe et al. (1) reported a case of primary retrop- eritoneal TCC with complete remission of the disease achieved with radiotherapy and concurrent oral chemo- therapy (tegafur-uracil). The explanation they gave for their success was that the chemotherapeutic agent acted as a radiosensitisor. In our case, the patient died after 5 months with development of a metastatic neck deposit. To conclude, primary retroperitoneal TCC is an exceptionally infrequent neoplasm. Due to retro- peritoneal location they grow to very large size before being detected. Modality of therapy has not been yet defined due to their rarity. Progno- sis is poor as most of the patient die within months. REFERENCES 1. K Ichinohe, M Ijima, T Usami, S Baba. Complete remission of primary retroperitoneal transitional cell carcinoma after radiotherapy and oral chemotherapy: a case report. Ann R Coll Surg Engl. 2013; 95:e52–e54. 2. Basu S, Ansari M, Gupta S, Kumar A. Primary retroperitoneal transitional cell carcinoma presenting as a dumb-bell tumour. Singapore Med J 2009; 50: e384–e87. 3. Kransdorf MJ. Malignant soft-tissue tumors in a large referral population: distribution of diagnoses by age, sex and location. Am J Radiol 1995; 164:129-34. 4. Gupta S, Gupta S, Sahni K. Retroperitoneal urogenital ridge transitional cell carcinoma. J Surg Oncol1983; 22: 41–44. 5. Donnelly BA. Primary retroperitoneal tumors-A report of 95 cases and a review of literature. Surg Gynecol Obstet 1946;83:705- 17. 6. Braasch JW, Mon AB. Primary retroperitoneal tumors. Surg Clin North Am 1967; 47:663-78. 7. Handfield-Jones RM. Retroperitoneal cysts: their pathology, diagnosis and treatment. Br J Surg 1924; 12:119-34. 8. Hansmann GM, Budd JW. 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