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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 characteristics, immunophenotype and differential diagnosis of mesenchymal sarcoma (MC) in children with intracranial central nervous system. Methods The clinical manifestations, imaging, histological features and immunophenotype of MC in children with intracranial central nervous system were analyzed in two cases. The related literatures were reviewed. Results The two children with MC were all 9 years old, one case was male and one female. The lesions in the male children were located in the skull base (left sublingual nerve canal), and the lesions in the female children were located in the left cerebellopontine angle. Imaging changes are prompted “neurogenic tumors ”. Microscope showed the tumor by undifferentiated small round cells and tumorous hyaline cartilage islet two components mixed. Immunohistochemistry showed that there were (+) SOX9, CD99, Syn, Cg A, NSE, D2-40, vimentin, Bcl-2 and CD56 in undifferentiated small cells; Conclusion The MC in primary cranial central nervous system of children is extremely rare and is a highly malignant tumor characterized by the differentiation of primary mesenchyme of small cells into naive cartilage. Diagnosis depends on the pathological examination. Differential diagnosis includes dedifferentiated chondrosarcoma, metaplastic meningioma, meningeal pericytes, primitive neuroectodermal tumors and the like.