Quek Marcus L, Nichols Peter W, Yamzon Jonathan, Daneshmand Siamak, Miranda Gus, Cai Jie, Groshen Susan, Stein John P, Skinner Donald G
Departments of Urology, Keck School of Medicine, University of Southern California, University of Southern California/Norris Comprehensive Cancer Center, Los Angeles, California, USA.
J Urol. 2005 Jul;174(1):93-6. doi: 10.1097/01.ju.0000162085.20043.1f.
Primary neuroendocrine tumors of the bladder are rare and they include small and large cell variants. We reviewed our experience with treating these tumors with radical cystectomy to evaluate their histopathological characteristics and clinical outcomes.
From August 1971 to June 2004, 2,005 patients underwent radical cystectomy for primary bladder cancer at our institution, of whom 25 (1.2%) had neuroendocrine tumors of the bladder, including small cell carcinoma in 20 and large cell carcinoma in 5. Pure neuroendocrine-type histology was identified in 16 cases, including 1 with small and large cell features, while the remaining 9 had mixed histology, that is transitional cell carcinoma in 8 and adenocarcinoma in 1. Multi-agent chemotherapy was administered to 14 patients.
Median patient age was 68 years (range 40 to 82) and 19 patients were male (76%). A total of 19 patients (76%) had lymph node involvement, of whom 2 had small liver metastases found intraoperatively, while only 4 (16%) had organ confined tumors and 2 (8%) had extravesical, node negative disease. These tumors tended to have a flat, ulcerative gross appearance with lymphovascular invasion, carcinoma in situ and necrosis present microscopically. Median followup was 11.8 years (range 18 days to 15.1 years). Five-year overall and recurrence-free survival was 10% and 13%, respectively. There was no significant survival difference between small and large cell carcinoma. Mixed histologies tended to do better than pure neuroendocrine tumors, although this did not attain statistical significance (p = 0.064). Patients receiving multimodality therapy had significantly better overall (p = 0.051) and recurrence-free (p = 0.003) survival than those treated with cystectomy alone.
Neuroendocrine tumors of the bladder usually present with advanced pathological stage and portend a poor prognosis. Adjuvant chemotherapy protocols may provide improved survival compared with cystectomy alone.
膀胱原发性神经内分泌肿瘤较为罕见，包括小细胞和大细胞亚型。我们回顾了采用根治性膀胱切除术治疗这些肿瘤的经验，以评估其组织病理学特征和临床结局。
1971年8月至2004年6月，我院共有2005例患者因原发性膀胱癌接受了根治性膀胱切除术，其中25例（1.2%）患有膀胱神经内分泌肿瘤，包括20例小细胞癌和5例大细胞癌。16例为纯神经内分泌型组织学，其中1例具有小细胞和大细胞特征，其余9例为混合组织学，即8例移行细胞癌和1例腺癌。14例患者接受了多药化疗。
患者中位年龄为68岁（范围40至82岁），19例为男性（76%）。共有19例患者（76%）出现淋巴结转移，其中2例术中发现有小的肝转移，仅4例（16%）肿瘤局限于器官内，2例（8%）肿瘤侵犯膀胱外但淋巴结阴性。这些肿瘤大体外观往往扁平、溃疡，伴有淋巴管浸润，显微镜下可见原位癌和坏死。中位随访时间为11.8年（范围18天至15.1年）。5年总生存率和无复发生存率分别为10%和13%。小细胞癌和大细胞癌之间的生存率无显著差异。混合组织学类型的预后往往优于纯神经内分泌肿瘤，尽管未达到统计学显著性（p = 0.064）。接受多模式治疗的患者总体生存率（p = 0.051）和无复发生存率（p = 0.003）显著优于单纯接受膀胱切除术的患者。
膀胱神经内分泌肿瘤通常呈现晚期病理分期，预后较差。与单纯膀胱切除术相比，辅助化疗方案可能提高生存率。
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