Aristeidis Alevizopoulos, Huay Shan Yuen, Soumadri Sen and Rowan Casey
Primary extra-pulmonary small cell carcinomas (SCC) are rare, with <40 cases reported in the current literature. Only 1% of all primary upper urinary tract tumors are SCCs. We present a new case of a primary SCC of the right upper urinary tract. A 78-year-old male patient presented with frank hematuria and a lump in the left posterior triangle of his neck. CT imaging revealed a right renal pelvic mass associated with suspected liver metastases, cervical and retroperitoneal lymphadenopathy. Rigid ureteroscopy demonstrated a tumor involving the right ureter. Immunohistochemical analysis of neck and ureteric biopsies revealed high proliferative activity and positive CD56 and TTF1 markers, in keeping with a diagnosis of small cell carcinoma. The patient was offered systemic chemotherapy, which he declined. He developed further skeletal metastases and underwent treatment with radiotherapy and dexamethasone. He succumbed to his illness 13 months after initial presentation. >40% of SCCs of the urinary tract are metastatic on presentation. In these cases, surgical options are limited and prognosis is poor. The literature suggests that neoadjuvant chemotherapy in organ-confined disease improves clinical outcome. However due to rarity of cases, there is limited evidence to guide management of metastatic SCC.
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