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[尿路小细胞神经内分泌癌的临床及病理特征]

[Clinical and pathologic characteristics of small cell neuroendocrine carcinoma of urinary tract].

作者信息

Guo Ai-tao, Chen Wei, Wei Li-xin

机构信息

Department of Pathology, the General Hospital of People Liberation Army, Beijing, China.

出版信息

Zhonghua Bing Li Xue Za Zhi. 2012 Nov;41(11):747-51. doi: 10.3760/cma.j.issn.0529-5807.2012.11.008.

Abstract

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 clinicopathologic 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 1 multifocal 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 cystectomy. Six other patients underwent radical nephroureterectomy, 1 partial cystectomy, 1 TURBT and the remaining case biopsy only. The size of the tumor ranged from 0.8 to 8.0 cm (median = 4.5 cm). Histologically, 15 cases represented mixed small cell neuroendocrine carcinoma (with 13 mixed with transitional cell carcinoma and 2 with adenocarcinoma). 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 histologic examination. Early diagnosis, when coupled with cystectomy or nephroureterectomy and adjuvant chemotherapy, represents the mainstay of management.

摘要

目的

研究泌尿道小细胞神经内分泌癌的临床及病理特征。

方法

回顾性分析1999年至2010年解放军总医院收治的所有泌尿道癌病例。对小细胞神经内分泌癌的临床病理资料进行进一步分析,并复习相关文献。

结果

共确诊16例小细胞神经内分泌癌,其中膀胱10例,输尿管2例,肾盂3例,1例为累及肾盂和输尿管的多灶性肿瘤。男女各8例。患者中位年龄63岁(范围24至79岁)。主要临床表现为肉眼血尿(11例)。4例患者有侧腹痛,4例有尿路刺激症状。7例行根治性膀胱切除术。另外6例行根治性肾输尿管切除术,1例行部分膀胱切除术,1例行经尿道膀胱肿瘤切除术,其余1例仅行活检。肿瘤大小为0.8至8.0 cm(中位值 = 4.5 cm)。组织学上,15例为混合性小细胞神经内分泌癌(其中13例与移行细胞癌混合,2例与腺癌混合)。免疫组化研究显示神经内分泌标志物呈阳性染色。初诊时,1例为pT1期,7例为pT2期,6例为pT3期,2例为pT4期。6例患者术后死于该疾病。总生存期为25个月,5年生存率为32.4%。

结论

膀胱小细胞神经内分泌癌是一种高度恶性疾病,预后较差。诊断依赖于详细的组织学检查。早期诊断并结合膀胱切除术或肾输尿管切除术及辅助化疗是主要的治疗方法。

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