Yoshizato K, Yoshioka S, Tamai T, Tsuji K, Nishio S
Department of Neurosurgery, Oita Prefectural Hospital, Japan.
No Shinkei Geka. 1999 Mar;27(3):243-8.
A 2-month-old girl with a supratentorial primitive neuroectodermal tumor (PNET), which extended into the skull, is herein presented. The patient underwent total removal of the tumor and also received a course of postoperative chemotherapy. After a follow-up period of 12 months, the infant is alive without recurrence. Histologically, the tumor was composed of poorly differentiated neuroectodermal cells, and these neoplastic cells showed a mild immunohistochemical reaction for GFAP and synaptophysin, and a moderate reactivity for neuron specific enolase and vimentin. In addition, a moderate level of immunoreactivity for HBA71 antigen (p30/32M1C2), which is the product of the M1C2 gene and is found in peripheral PNETs but not in central PNETs, was noted in many neoplastic cells. Although this tumor was located intracranially, it may be classified as a peripheral PNET.
本文报告了一名2个月大的女孩,患有幕上原始神经外胚层肿瘤(PNET),肿瘤已侵犯颅骨。该患者接受了肿瘤全切术,并进行了术后化疗。随访12个月后,患儿存活且无复发。组织学检查显示,肿瘤由低分化神经外胚层细胞组成,这些肿瘤细胞对GFAP和突触素呈轻度免疫组化反应,对神经元特异性烯醇化酶和波形蛋白呈中度反应。此外,在许多肿瘤细胞中发现,对HBA71抗原(p30/32M1C2)呈中度免疫反应,该抗原是M1C2基因的产物,见于外周PNET,而不见于中枢PNET。尽管该肿瘤位于颅内,但可能归类为外周PNET。