摘要： 目的   探讨促纤维增生性非婴儿星形细胞瘤的临床病理学表现，以及诊断与鉴别诊断特点。方法与结果   2 例患者均为女性，年龄分别为5 岁和10 岁。例1 临床表现为反复神志不清伴四肢抽搐，MRI 显示左侧颞角内侧占位性病变，术中可见肿瘤位于颞叶底面皮质，大小约3.00 cm × 3.50 cm ×3.50 cm，剖面呈黄色、血供中等，肿瘤边界尚清楚，于显微镜下手术全切除；例2 以反复头痛伴呕吐发病，MRI显示右侧额颞叶侧脑室内巨大囊实性占位性病变，术中可见肿瘤位于皮质下1 cm，瘤体内抽吸出黄色囊液约80 ml，肿瘤大小约为11.00 cm × 10.00 cm × 10.00 cm，剖面黄色，质地较脆，血供丰富，与脑室壁界面脑组织粘连紧密，手术全切除。组织病理学观察肿瘤组织主要由间质纤维增生伴肿瘤性星形细胞成分所组成，可见小灶性原始神经上皮细胞；表达波形蛋白、胶质纤维酸性蛋白、S-100 蛋白和巢蛋白，不表达广谱细胞角蛋白、上皮膜抗原、神经元核抗原，但原始细胞仅表达突触素；Ki-67 抗原标记指数分别为6%和10%；可见网织纤维围绕肿瘤细胞。术后分别随访18 和52 个月，无复发和转移。结论   促纤维增生性非婴儿星形细胞瘤为临床罕见的幕上神经上皮肿瘤，患者预后良好，但需注意与其他肿瘤，尤其是恶性胶质瘤相鉴别。

关键词: 神经胶质瘤, 诊断, 鉴别； 纤维化, 增生, 预后

Abstract: Objective To investigate clinical pathological characteristics, diagnosis and differentiation of desmoplastic non-infantile astrocytoma (DNIA). Methods & Results Two female patients with DNIA aged 5 and 10 years old, respectively. The patient of case 1 had repeated unconsciousness with limbs twitch, MR image showed a mass in the inner part of the left temporal horn, the tumor was located in the underside of cortex of temporal lobe, about 3.00 cm × 3.50 cm × 3.50 cm in size with yellow in color and medium blood supply, the boundaries of the tumor were fairly clear and the mass was completely dissected. The patient of case 2 had recurrent headache with vomiting, MR image showed a large cystic solid mass in the right frontal temporal lobe-lateral ventricle. The tumor was located at 1 cm under the subcortex and drew off yellow cyst fluid about 80 ml. The tumor was about 11.00 cm × 10.00 cm × 10.00 cm in size with yellow in color and brittle texture and abundant blood supply, adhesion to the brain tissue and was completely dissected. Histology showed obvious interstitial fibrosis associated with neoplastic astrocytes with primary neuroepithelium. Immunohistochemical staining indicated that the expression of both tumor cells was positive for vimentin, GFAP, S-100, Nestin, and negative for CK, EMA, NeuN, the expression of primary cells was positive for Syn; Ki-67 labeling index was 6% and 10% , respectively. Histochemical staining showed that the tumor cells were surrounded by reticular fibers. Patients were followed up for 18 and 52 months, respectively, both had no recurrence and metastasis. Conclusion DNIA is a rare supratentorial neuroepithelial tumor. The prognosis is generally good. It needs to be differentiated from other tumors, especially malignant glioma.

Key words: Glioma, Diagnosis, differential, Fibrosis, Hyperplasia, Prognosis