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目的探讨毛细胞黏液样星形细胞瘤(pilomyxoid astrocytoma,PMA)的临床病理特点及鉴别诊断。方法报道3例PMA患者的临床资料,并对其进行组织学观察及免疫组化(EnVision-plus法)标记,其中1例行电镜观察。结果本组PMA均为女性,年龄分别为13岁4、岁和17岁。肿瘤分别位于右侧丘脑、右侧颞叶基底节区及下丘脑/视交叉区。镜下见双极性的梭形瘤细胞呈星网状散在分布于黏液背景中,部分区域呈血管中心性生长,瘤细胞异型性小,核分裂象偶见,间质血管增生明显。1例见大片出血,1例见少许嗜酸性颗粒小体及小灶坏死,3例PMA均未见双相性结构及Rosenthal纤维。免疫组化标记示瘤细胞表达GFAPv、imentin、S-100及Olig-2,而CKpan、EMA、NF、CgA、Neu-N、p53均呈阴性表达,Syn有2例局灶阳性,Ki-67阳性率为1%～2%。仅有的1例电镜表现除具有伸长细胞特征外,胞质内还可见神经内分泌样颗粒。结论 PMA为毛细胞星形细胞瘤(pilocytic astrocytoma,PA)的亚型,与经典PA相比,具有独特的组织学特征,并具有更强的侵袭性,故应加强对其的认识。
Objective To investigate the clinicopathological features and differential diagnosis of pilomaxoid astrocytoma (PMA). Methods The clinical data of 3 patients with PMA were reported. Histological observation and immunohistochemistry (EnVision-plus) were performed on the 3 patients. One patient underwent electron microscopy. Results The PMA in this group were all female and were 13 years old, 4 years old and 17 years old respectively. Tumors were located in the right thalamus, right temporal lobe basal ganglia and hypothalamic / optic chiasm area. Microscopically, the bipolar Fusiform tumor cells were scattered in the mucus-like background in the form of a star-shaped network. Some of the tumors showed centriole growth, small atypia of the tumor cells, occasional mitosis, and obvious interstitial vascular hyperplasia. 1 case of large hemorrhage, a case of a small number of eosinophilic granules and small focal necrosis, three cases of PMA did not see the biphasic structure and Rosenthal fibers. Immunohistochemical staining showed that GFAPv, imentin, S-100 and Olig-2 were expressed in tumor cells, but negative in CKpan, EMA, NF, CgA, Neu- The positive rate of 1% to 2%. Only one case of electron microscopy in addition to the characteristics of elongated cells, the cytoplasm also visible neuroendocrine-like particles. Conclusion PMA is a subtype of pilocytic astrocytoma (PA). Compared with classical PA, PMA has unique histological features and is more aggressive. Therefore, PMA should be strengthened.