泌尿道小细胞性神经内分泌癌的临床病理学特征

Clinical and pathologic characteristics of small cell neuroendocrine carcinoma of urinary tract

目的探讨泌尿道小细胞性神经内分泌癌的临床及组织病理学特征。方法复习解放军总医院1999至2010年间经病理诊断为泌尿道上皮癌的病例，选取其中的小细胞性神经内分泌癌16例，结合相关文献对其临床及病理学资料进行回顾性分析。结果泌尿道小细胞性神经内分泌癌占同期泌尿道上皮癌的0．36％（16／4386），其中发生于膀胱10例，输尿管2例，肾盂3例，输尿管和肾盂多发肿瘤1例。男性8例，女性8例。年龄45～79岁，中位年龄63岁。临床症状11例有血尿，4例有尿路刺激征，4例伴腰腹部疼痛。仅2例有吸烟史。13例行根治性手术切除，1例行经尿道膀胱肿瘤电切术，1例行膀胱部分切除术，1例仅行活检。肿瘤中位直径为4．5cm，组织学15例为混合性小细胞性神经内分泌癌，其中13例混合有移行细胞癌，2例混合有腺癌，l例为单纯性小细胞癌。免疫组织化学染色显示神经内分泌标记阳性。10例见脉管内癌栓，1例伴淋巴结转移，2例伴远隔脏器转移。临床分期1例为pTl期，7例为pT2期，6例为pT3期，2例为pT4期。14例获得随访资料，整体生存时间为25个月，6例死亡，5年生存率为32．4％。结论泌尿道小细胞性神经内分泌癌恶性程度高，预后不良，确诊依赖病理学及免疫组织化学检查；早期诊断和采用手术切除仍是首选治疗方式，配合化疗和（或）放疗可提高疗效。

Objective To study the clinical and pathologic characteristics of small cell neuroendocrine carcinoma of urinary tract. Methods All cases of urinary tract carcinoma encountered in the General Hospital of People Liberation Army during the period from 1999 to 2010 were retrospectively reviewed. The elinicopathologie data of small cell neuroendocrine carcinomas were further analyzed, with literature review. Results A total of 16 cases of small cell neuroendocrine carcinoma were identified, including 10 from urinary bladder, 2 from ureter, 3 from renal pelvis, and l muhifocal tumor involving renal pelvis and ureter. There were altogether 8 males and 8 females. The median age of the patients was 63 years （range = 24 to 79 years ）. Gross hematuria （11 cases） represented the main presenting symptom. Four patients had flank pain and 4 had urinary irritation symptoms. Seven patients underwent radical cysteetomy. Six other patients underwent radical nephroureterectomy, 1 partial cystectomy, 1 TURBT and the remaining ease biopsy only. The size of the tumor ranged from 0. 8 to 8.0 em（ median = 4. 5 cm ）. Histologically, 15 eases represented mixed small cell neuroendocrine eareinoma （with 13 mixed with transitional cell carcinoma and 2 with adenoeareinoma ）. Immunohistochemical study showed positive staining for neuroendocrine markers. On presentation, 1 patient was in stage pT1, 7 in stage pT2, 6 in stage pT3, 2 in stage pT4. Six patients died of the disease after operation. The overall survival was 25 months and the 5-year survival rate was 32. 4%. Conclusions Small cell neuroendocrine carcinoma of urinary bladder is a highly malignant disease and associated with poor prognosis. The diagnosis relies on detailed histologie examination. Early diagnosis, when coupled with cystectomy or nephrouretereetomy and adjuvant chemotherapy, represents the mainstay of management.