巨大婴儿促纤维增生性星形细胞瘤一例报道并文献复习

Giant desmoplastic infantile astrocytoma：l case report and review of the literatures

目的探讨婴幼儿促纤维增生性星形细胞瘤（desmoplastic infantile astrocytoma，DIA）的临床表现、病理特征、鉴别诊断及治疗方法。方法报道1例巨大DIA并进行组织形态学和免疫组织化学研究，结合文献对其临床表现、病理形态特点、鉴别诊断、治疗方法及预后进行探讨。结果手术全切除肿瘤，术后病理检查发现瘤组织由梭形细胞和星形细胞样细胞组成，免疫组织化学显示瘤细胞神经胶质纤维酸性蛋白（GFAP）阳性。患儿恢复良好。结论DIA是一种罕见的发生在婴幼儿的脑肿瘤，根据病理学特征，可以作出明确的诊断。DIA属于低度恶性肿瘤，手术全切除后预后较好。

Objective To study on clinical, pathological and surgical management of desmoplastic infantile astrocytoma （DIA） . Method One case of DIA was reported with emphases on its clinic pathological features, and differential diagnosis combined with clinic histology and immunohistochemistry studies. Results In our case, we punctured and drainaged of cystic lesion to decrease the high ICP, and then total tumor recection was performed. On the immunohistoehemistry, the tumor showed Glial Fibrillary Acidic Protein （GFAP） positive. Follow-up showed a good outcome. Conclusions DIA is a rare tumor that presents as a largehemispheric mass in infants. Definite diagnosis depends on pathological examination. Decompression by puncture the cystic lesion before total tumor recection is a good choice for huge tumor. The tumor has an overall good prognosis.