罕見原發性上泌尿道小細胞神經內分泌癌: 病例報告及文獻回顧

陳兆宏 吳翊豪 林政鋒 劉冠麟 陳文祥 林承家 陳鴻毅

基隆長庚醫院 外科部 泌尿科

A rare case of small cell neuroendocrine carcinoma of the upper urinary tract: A case report of and Literature Review

Chao-Hung Chen, Yi-Hao Wu, Cheng-Feng Lin, Kuan-Lin Liu , Wen-Hsiang Chen, Cheng-Chia Lin, Hong-Yi Chen

Divisions of Urology, Department of Surgery, Chang Gung Memorial Hospital, Keelung, Taiwan

Introduction:

Small cell neuroendocrine carcinoma of the upper urinary tract is extremely rare and is associated with a poor prognosis due to aggressive clinical course. We present the case of a ureteral small cell neuroendocrine carcinoma in a 80-year-old woman.

Case presentation:

   The 80-year-old woman had past history of hypertension and diabetes mellitus under medication control. She was admitted due to painless gross hematuria for several months. During the admission, she received the abdomen CT scan, which showed soft tissue in the distal third of the left ureter with hydronephrosis. Ureteroscopy showed a polypoid mass and biopsy was done, which pathology report showed neuroendocrine carcinoma. Besides, we also arranged the chest CT scan for further survey, which revealed no lung or lymph node metastasis and bone scan showed no definite evidence of bone metastasis. Left robotic-assisted laparoscopic nephroureterectomy with bladder cuff excision was performed.

Pathologically, the tumor, which measured 3.2 x 2.4 cm, was seen in the lower portion of the left ureter with margin negative finding. Microscopically, the tumor was composed of uroepithelium infiltrated with solid aggregates of small tumor cells with invasion beyond muscularis into periureteral fat. Immunohistochemically, the tumor cells are immunoreactive for synaptophysin and Ki-67.

Conclusion:

The neuroendocrine carcinoma is extremely rare arising from ureter.The diagnosis mainly relies on pathological characteristics and the prognosis is poorer compared with that of urothelial cancer. From the literature, there are no standard treatment for neuroendocrine carcinoma of upper urinary tract and it supports chemotherapy and radiotherapy followed by nephroureterectomy.