病例報告：大細胞神經內分泌膀胱癌

郭炫廷、楊景偉、黃家倫、謝啟誠、王曉暹、陳光國、張心湜、蔡昇翰

振興醫療財團法人振興醫院泌尿部

Large Cell Neuroendocrine Carcinoma of Bladder - A case report

Hsuan-Ting Kuo, Chin-Wei Yang, Chia-Lun Huang, Chieh-Chen, Hsieh, Hsiao-Hsian Wang, Kuang-Kuo Chen, Luke S. Chang and Sheng-Han Tsai

Department of Urology, Cheng Hsin General Hospital, Taipei, Taiwan

Introduction:

Neuroendocrine tumors arise from neuroendocrine cells that mainly distributed in lung and gastrointestinal tract, but is a rare entity with incidence rate around 0.14 per 100,000 patient in the USA when urinary bladder is involved. We hereby presented a case of “pure” primary large cell carcinoma of urinary bladder.

Presentation of case:

A 78-year-old woman with a history of coronary artery disease status post coronary artery bypass graft, hypertension and hyperlipidemia, presented with painful gross hematuria with lower urinary tract symptoms for two months. Computed tomography scan of lower abdomen was done and disclosed a 1.5 cm mass at right ureterovesical junction with marked hydronephrosis and thin renal cortex. The tumor was grey and solid under cystoscopy and the right ureteral orifice was covered by the tumor. Transurethral resection of the tumor was performed and the right ureteral orifice was exposed. Further diagnostic ureteroscopy was done and no significant tumor was noted in the ureter. Microscopically, special stains for the tumor were negative for GATA-3 and p40, but immunoreactive for p16, CD56 and synaptophysin, which turned out to be high grade large cell neuroendocrine carcinoma with muscularis propria invasion(pT2). Radical cystectomy was suggested but the patient refused. The patient is receiving concurrent chemoradiotherapy with carboplatin now.

Discussion:

Neuroendocrine bladder cancer is a rare condition which accounts for less then 1% of all urinary bladder malignancy. Large cell neuroendocrine carcinoma of bladder is one of its extremely rare subtype with less then 40 cases so far reported in the literature. Most of the cases published had poor prognosis owing to the rapid and aggressive disease progression and metastasis. Due to its rarity, there is no consensus of treatment approaches nowadays. Multimodal treatment includes radical cystectomy, adjuvant radiotherapy or cisplatin-based chemotherapy was suggested in previous literature.