Wang Jian, Lin Xu-Yong, Qiu Xue-Shan, Wang En-Hua, Yang Lian-He, Yu Juan-Han
Department of Pathology, the First Affiliated Hospital and College of Basic Medical Sciences, China Medical University, Shenyang, China.
Neuropathology. 2017 Apr;37(2):105-109. doi: 10.1111/neup.12329. Epub 2016 Aug 24.
Hemangioblastoma is a well-circumscribed, highly vascular, lipid-rich and low-grade tumor of uncertain histogenesis. Its histopathological features have been well established. Herein, we present a case of cerebellar hemangioblastoma in a 43-year-old woman. Histologically, the tumor was predominantly composed of cellular areas showing eosinophilic or vacuolated stromal cells arranged in nests and sheets. Focally, conventional reticular areas could be seen. Additionally, in some areas, the stromal cells were arranged radially around blood vessels, exhibiting perivascular pseudorosette structures, which were similar mostly to those of ependymomas. Immunohistochemically, the stromal cells markedly showed diffused staining for Vimentin, S-100, CD56, NSE, inhibin-a, podoplanin, alpha-Thalassemia/mental retardation syndrome X and carbonic anhydrase IX, and were negative for cytokeratin, epithelial membrane antigen, oligodendrocyte transcription factor 2, neuronal nuclear antigen, synaptophysin, isocitrate dehydrogenase 1 (R132H), P53 and CD34. Interestingly, the reticular and cellular areas either showed no or individual scattering of the GFAP-positive cells, respectively, while, the perivascular pseudorosette areas strongly and diffusely expressed GFAP. Nuclear mitosis and necrosis were not observed. The MIB-1 antibody labeling index was especially low (about 3%). Based on these findings, the patient was diagnosed with cerebellar hemangioblastoma. In the present case, we documented a distinctive histological appearance of perivascular pseudorosettes in hemangioblastoma and provided the evidence for stromal cells with glial differentiation.
血管母细胞瘤是一种边界清晰、血管丰富、富含脂质且组织发生不明的低级别肿瘤。其组织病理学特征已明确。在此，我们报告一例43岁女性的小脑血管母细胞瘤病例。组织学上，肿瘤主要由细胞区域组成，这些区域可见嗜酸性或空泡状基质细胞呈巢状和片状排列。局部可见传统的网状区域。此外，在一些区域，基质细胞围绕血管呈放射状排列，呈现血管周围假菊形团结构，这大多与室管膜瘤的结构相似。免疫组织化学显示，基质细胞对波形蛋白、S-100、CD56、神经元特异性烯醇化酶、抑制素-α、足板蛋白、α-地中海贫血/智力发育迟缓综合征X和碳酸酐酶IX呈明显弥漫性染色，而细胞角蛋白、上皮膜抗原、少突胶质细胞转录因子2、神经元核抗原、突触素、异柠檬酸脱氢酶1（R132H）、P53和CD34均为阴性。有趣的是，网状和细胞区域分别未见或仅有个别散在的GFAP阳性细胞，而血管周围假菊形团区域则强烈且弥漫性表达GFAP。未观察到核分裂和坏死。MIB-1抗体标记指数特别低（约3%）。基于这些发现，该患者被诊断为小脑血管母细胞瘤。在本病例中，我们记录了血管母细胞瘤中血管周围假菊形团独特的组织学表现，并为具有神经胶质分化的基质细胞提供了证据。
