原发性肾小细胞神经内分泌癌1例报道并文献复习

Primary renal neuroendocrine carcinoma:a case report and review of the literature

目的探讨肾脏小细胞神经内分泌癌的起源、临床病理特征、诊断、治疗方法,以提高对该病的认识。方法分析我科收治的1例肾脏小细胞神经内分泌癌患者的临床资料,结合国内外文献对该病进行复习总结。结果患者无明显症状及阳性体征,因体检发现左肾占位入院。术后标本大体观:左肾上中极见一肿物,大小9cm×8cm×8cm,切面灰白,质软呈实性,肾盂、肾盏结构破坏。镜下观:肿瘤细胞为小圆形或梭形,小梁状、巢状或片状排列,核分裂易见。免疫组化:CD10(-)、CD117(+)、CD56(+)、CD99(+)、CgA(-)、CK(部分+)、CK7(-)、CK8(+)、Ki-67(≥25%)、Syn(+)、Vim(+)。结论肾脏小细胞神经内分泌癌临床罕见,临床表现缺乏特异性,确诊主要依靠组织学特点和免疫组织化学检查,治疗以根治性手术为主,预后较差。

Objective To discuss the origin, clinicopathological features, dignosis and treat- ment of neuroendocrine carcinoma in the kindey, so as to improve the understanding of this disease. Methods We report on the clinical and pathological information of one case of a patient with a pri- mary renal neuroendocrine carcinoma as well as review relative literature. Results The patient was diagnosed with a renal tumor when he received physical examination, and exhibited no positive symp- toms. Macroscopically, the diameter of tumor was 9 cm in the middle part of the left kindey, the cross surface of the tumor was grey-white, soft and solid. The tumor had invaded the renal pelvis of the left kidney. Microscopically, the tumor was small round and spindle. It was composed mainly of trabeculae and nests or sheets of tumor cells. Immunohistoehemical analysis： CD10 （-）, CDl17 （＋）, CD56（＋）, CD99（＋）, CgA（-）, CK（partial positive）, CK7（-）, CKS（＋）, Ki-67（≥ 25%）, Syn（＋）, Vim（＋）. Conclusions Primary renal neuroendocrine carcinoma is a rare and aggressive tumor which is lack of specific features in clinical performances with a poor prognosis. The dignosis of it mainly depends on the results of pathological examination and immunohistochemistry. The primary treatment of localized primary renal neuroendocrine carcinoma is radical nephrectomy.