儿童颅内间叶性软骨肉瘤2例临床病理观察  

Intracranial mesenchymal chondrosarcoma in children: a clinicopathological observations of two cases

作　　者：熊振芳[1] 熊秋迎[1] 胡秀华[2] 万红萍[1] 熊磊[1] 梅金红[1] 

机构地区：[1]南昌大学第一附属医院病理科,南昌330006 [2]南昌市洪都中医院急诊科,南昌330038

出　　处：《诊断病理学杂志》2015年第11期697-700,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨儿童颅内中枢神经系统间叶性软骨肉瘤(MC)的临床病理特征、免疫表型及鉴别诊断。方法对2例罕见的儿童颅内中枢神经系统MC的临床表现、影像学、组织学形态和免疫表型进行分析,并复习相关文献。结果 2名MC患儿均为9岁,男、女各1例,男性患儿病变位于颅底(左舌下神经管区),女性患儿病变位于左侧桥小脑角。影像学改变均提示"神经源性肿瘤"。镜下示肿瘤由未分化的小圆细胞和肿瘤性透明软骨小岛两种成分混杂组成。免疫组化:未分化小细胞SOX9、CD99、Syn、Cg A、NSE、D2-40、vimentin、Bcl-2及CD56呈(+);软骨区S-100及SOX9灶状(+)。结论原发于儿童颅内中枢神经系统的MC极为罕见,是一种高度恶性肿瘤,其组织学特点是小细胞原始间叶组织向幼稚软骨组织的分化。确诊需依靠病理检查。鉴别诊断包括去分化软骨肉瘤、化生型脑膜瘤、脑膜血管周细胞肿瘤、原始神经外胚叶肿瘤等。Objective To investigate the clinicopathological features,immunohistochemistry and differential diagnosis of mesenchymal chondrosarcoma（ MC） of the central nervous system in children. Methods Two cases of MC were reported with review of the literature. The clinical,imaging,pathological and immunohistochemical features of this neoplasm were described in detail. Results One case was 9-year-old male with a mass in the basis cranii（ the left hypoglossal canal area）. Another case was 9-year-old female with a mass in the left side of the cerebellopontine angle.Image analysis suggested ＂neurogenic tumors＂. The histological characteristic features showed undifferentiated small cells together with islands of chondrosarcoma. The tumors cells were positive for SOX9,CD99,Syn,Cg A,NSE,D2-40,vimentin,Bcl-2 and CD56. The cells in chondroid matrix were focal positive for S-100 and SOX9. Conclusion Intracranial mesenchymal chondrosarcoma（ MC） of the central nervous system in children is a very rare and highly malignant cartilaginous tumor. It has the characteristics of primary mesenchyme which differentiates to congenital cartilage.The diagnosis should be made based on pathological examination. It must be differentiated from other lesions,including atypical teratoid / rhabdoid tumor,metaplastic meningioma,gliosarcoma,PENT and so on.

关 键 词：软骨肉瘤 免疫组化 诊断 

分 类 号：R739.41[医药卫生—肿瘤]