腹膜后囊性黑色素性神经鞘瘤一例报道并文献复习  

Retroperitoneal Cystic Melanotic Schwannoma: A Case Report and Literature Review

作　　者：何珏[1] 肖秋香[1] 

机构地区：[1]赣南医学院病理学教研室,江西省赣州市341000

出　　处：《中国全科医学》2015年第30期3741-3744,共4页Chinese General Practice

摘　　要：目的探讨腹膜后囊性黑色素性神经鞘瘤的临床病理特征,以提高对该肿瘤的诊治水平。方法赣南医学院第一附属医院肿瘤外科2014-03-17收治1例腹膜后囊性黑色素性神经鞘瘤患者,总结其临床资料、影像学表现、病理特征,并结合国内外文献进行综合分析。结果患者,男,47岁,因左侧后腰部疼痛就诊,增强CT扫描发现左侧腰大肌旁肿瘤,与左侧肾脏关系密切,外科手术切除;巨检:肿瘤为囊性,大小15.0 cm×10.0 cm×10.0 cm,其内见大量咖啡样黏稠液体,囊壁厚1.0 cm,部分外壁与左侧肾脏周围组织粘连;镜下:囊壁内肿瘤细胞较少,被纤维组织分隔成结节状,肿瘤细胞呈梭形及上皮样,胞质内见大量黑色素沉积。免疫组织化学标记:HMB45、Melan-A、S100、CD57、波形蛋白(Vimentin)均为(+),细胞外胶原基膜Ⅳ型胶原(CollagenⅣ)(+),Ki-67约4%(+)。病理诊断:(腹膜后)囊性黑色素性神经鞘瘤(经典型)。结论囊性黑色素性神经鞘瘤是少见的具有神经鞘细胞特征及黑色素细胞分化的软组织肿瘤,囊性者罕见,有潜在恶性生物学行为,需长期随访。Objective To investigate the clinicopathologic features of retroperitoneal cystic melanotic schwannoma in order to improve the level of diagnosis and treatment of such tumor. Methods Selected one patient with retroperitoneal cystic melanotic schwannoma who were admitted into the Department of Oncological Surgery of the First Affiliated Hospital of Gannan Medical University on March 17,2014. We made a review about clinical data, imaging findings, pathological features and treatment methods,and we conducted a general analysis combining with domestic and overseas literatures. Results Due to the pain in the left rear waist,this 47- year- old male went to see a doctor. A tumor was found beside the psoas muscle of left side by enhanced CT scan,which was closely related to the left kidney. And it was removed by surgical operation. Macroexamination：this tumor was cystic with the size 15. 0 cm × 10. 0 cm × 10. 0 cm,within which much sticky liquid like coffee can be seen. And the capsule wall was 1. 0 cm thick, part of which was adhered to peripheral tissues of the left kidney. Examination by optical microscope： cyst wall included few tumor cells,and they were divided into nodular shape by fiber tissues; the tumor cells were fusiform and epithelioid,and much melanin deposition can be seen in the cytoplast. Immunohistochemistry： HMB45,Melan-A,S100,CD57,Vimmentin and CollagenⅣ were（ ＋）. Ki- 67 was 4%（ ＋）. Pathological diagnosis： it was a typical Retroperitonea cystic melanotic schwannoma. Conclusion Melanotic schwannoma is a rare soft tissue tumor with the features of neurilemma cells and melanocytes differentiation. Cystic melanotic schwannoma is rare and contains potential malignant biological behavior. And such patients need long- term follow- up.

关 键 词：神经鞘瘤 黑色素 腹膜后肿瘤 诊断 免疫组织化学 预后 

分 类 号：R730.264.3[医药卫生—肿瘤]