膀胱神經內分泌腫瘤: 病例報告和文獻綜述

林毓庭、溫玉清、李良明、林克勳、林雍偉、蕭志豪、石宏仁、許軒豪、賴宗豪、許志呈

臺北市立萬芳醫院 泌尿科

Rare primary large-cell neuroendocrine carcinoma of the urinary bladder

- A case report and literature review

Allen Yu Ting Lin, Yu-Ching Wen, Liang-Ming Lee, Ke-Hsun Lin, Yung-Wei Lin, Chi-Hao Hsiao, Hung-Jen Shih, Syuan-Hao Syu, Chung-Howe Lai, Chih-Chen Hsu

Department of Urology, Wan Fan Hospital, Taipei Medical University, Taipei, Taiwan

Background:

Neuroendocrine tumors (NET) are extremely rare and aggressive. Bladder involvement is rare and two main forms have been described as small-cell and large-cell. Both considered highly aggressive since it shows poor oncologic outcomes being mainly diagnosed at advanced stage. We present a case of aggressive pathological variant bladder tumor with promising prognosis.

Case report:

A 69-year-old male presented with painless gross hematuria for 2 months. Cystoscopy revealed bladder tumor at left lateral wall. The biopsy report showed carcinoma with neuroendocrine differentiation which the IHC stain is positive for CK and Synaptophysin; negative for GATA3, PSA, chromogranin, and EBER. Staging abdominal computer tomography showed localized 5cm lesions within urinary bladder without lymph node involvement. Transurethral resection of the bladder tumor was performed and the pathology showed poorly differentiated neuroendocrine carcinoma, favoring large cell neuroendocrine carcinoma (LCNC) without urothelial component, cT2N0M0, stage II. Radical cystectomy was advised and 4 cycles of neoadjuvant chemotherapy with etoposide and cisplatin was prescribed with concurrent bladder hyperthermia. Follow up computer tomography showed complete remission. So partial cystectomy with pelvic lymph node dissection was then performed and the final pathological report showed no residual neuroendocrine carcinoma in specimen. The patient is currently free of disease 10 months after surgery.

Discussion:

No guidelines were published concerning the treatment modalities of LCNC. A multimodal therapeutic approach, such as the combination of chemotherapy, radiotherapy, and radical or partial surgeries, has been reported and higher survival rates was observed comparing with a conservative treatment. Cisplatin-based chemotherapy is often the best of the theraputic choice. A bibliographic review in showed that the survival rate for all published cases concerning LCNC was 46.2% for a maximum follow-up of 20 months.

Conclusions:

Large cell neuroendocrine carcinoma (LCNC) is one of the rarest types of bladder cancer occurring in <1%. It remains one of the most aggressive types of bladder cancer.  Neoadjuvant chemotherapy with partial cystectomy represents a good treatment option with fair prognosis. Bladder hyperthermia would be potential enhancement the theraputic effect.