恶性外周神经鞘瘤伴多向分化临床病理观察  

Malignant peripheral nerve sheath tumor with divergent differentiation

作　　者：郭爱桃[1] 刘爱军[1] 韦立新[1] 

机构地区：[1]解放军总医院病理科,北京100853

出　　处：《诊断病理学杂志》2010年第6期435-438,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨伴有多向分化的恶性外周神经鞘瘤(MPNST)的临床病理特征及诊断依据。方法对1例79岁男性患者的左大腿软组织肿瘤进行组织形态学观察及免疫组化检测,结合文献对其病理组织学特征进行分析。结果本例MPNST肿瘤的组织形态复杂多样,在梭形细胞背景上见明确的腺样结构、神经内分泌分化伴有菊形团形成、纤维组织细胞样、软骨、横纹肌母细胞和脂肪肉瘤样分化及色素细胞和神经节细胞分化。结论单纯用其他肿瘤来命名不能囊括如此复杂多样的组织形态,因此最后诊断为MPNST伴多向分化。此外,在同一MPNST中同时见到如此多的形态文献尚未见报道。Objective To describe 1 case of subcutaneous malignant peripheral nerve sheath tumor（MPNST） with divergent differentiation,and to discuss its pathologic features and diagnostic evidence.Methods Histological observation and immunohistochemical examinations were performed on the tumor that arose on the left thigh in a 79-year-old man.Morphologic features were described in combination with review of the literature.Results Morphologic features of this tumor were very pleomorphic and complicated： glandular structure,neuroendocrine differentiation with rosettes formation,fibrohistiocytoid,cartilaginous,rhabdmyoblast and liposarcomatous differentiation,pigment and gangliocyte differentiation were found in the background of spindle tumor cells.Conclusion So complicated and divergent morphologic features could not be included in any other tumor besides MPNST,and then the final diagnosis is malignant peripheral nerve sheath tumor（MPNST） with divergent differentiation.In addition,the occurrence of all these differentiation in the same PMNST case has not been described in literature.

关 键 词：恶性外周神经鞘瘤 上皮样分化 腺样分化 神经内分泌分化 蝾螈瘤 软骨分化 脂肪肉瘤样分化 色素细胞分化 神经节细胞分化 

分 类 号：R739.4[医药卫生—肿瘤]