婴幼儿促纤维增生性星形细胞瘤的临床病理特点

目的；分析婴幼儿促纤维增生性星形细胞瘤（desmoplastic infantile astrocytoma,DIA)的病理形态特征及其与其他肿瘤的鉴别诊断。方法：对2例DIA进行组织形态学、组织化学和免疫组织化学分析，结合文献对其临床表现、病理形态特点及鉴别诊断进行探讨。结果：本组2例DIA的CT检查表现均为左颞叶巨大的囊性病变；组织学表现在丰富的纤维性间质中，含有灶状或巢状的向星形细胞分化的神经上皮成分；免疫表型显示瘤细胞呈GFAP阳性，Vim阳性，NSE阴性；网织纤维染色示在致密的嗜银纤维区之间可见岛屿状的空染区。结论：DIA是一种罕见的发生在婴幼儿的中枢神经肿瘤。根据其组织学特点，结合组织化学和免疫组化染色结果，可以做出明确的病理诊断。

A clinic pathological analysis for desmoplastic infantile astrocytoma: 2 cases report and review of the literature

Purpose To summarize the clinic pathological characteristics and to analyze differential diagnosis of desmoplastic infantile astrocytoma (DIA) with other tumors located within CNS. Methods Two case of DIA was reported with emphases on its clinic pathological features and differential diagnosis combined with histology and histochemistry and immunohistochemistry studies. Results In these case, CT scan presented a massive cystic within tumor of the left temporal lobe. Histologically, DIA was characterized that the dense fibrous desmoplasia and the neoplastic astrocytes arranged in streams, nests or cabinet. The neoplastic astrocytes were located within a marked desmoplastic component pattern. Immunohistochemical staining showed that the neoplastic astrocytes cells were positive for GFAP and vimentin, but negative for synaptophysin. Reticulin staining showed clear space appeared in the area of abundant argentaffine fibers. Conclusion DIA is a rare CNS neoplasm arising in the cerebral hemispheres of infant. Combined with clinical, histopathological and immunohistochemical data, it can be diagnosed correctly.