Jiang Yue-Feng, Liu Yang, Wang Ye-Lin, Cao Hong-Yi, Wang Liang, Xu Hong-Tao, Li Qing-Chang, Qiu Xue-Shan, Wang En-Hua
Department of Pathology, the First Affiliated Hospital and College of Basic Medical Sciences, China Medical University, Shenyang, 110001, China.
Institute of Pathology and Pathophysiology, China Medical University, Shenyang, 110001, China.
Diagn Pathol. 2016 Aug 9;11(1):73. doi: 10.1186/s13000-016-0524-0.
Pleomorphic xanthoastrocytoma is rare, accounting for <1 % of all central nervous system (CNS) neoplasms. Angiomatous pleomorphic xanthoastrocytoma is an extremely rare variant of pleomorphic xanthoastrocytoma, with only six cases reported thus far.
A 24-year-old Chinese female patient who presented with seizure and loss of consciousness for 15 min underwent computed tomography and magnetic resonance imaging, which revealed a mass in the left parietal lobe. Histologically, the tumor was characterized by pleomorphic tumor cells and prominent vascularity. The angiomatous region varied, ranging from a sinusoidal pattern to a venous malformation. Focal fibrinoid necrosis, hyalinization, and a moderate infiltration by lymphocytes and plasma cells were visible in the vessel wall. The tumor cells were in close proximity with adjacent small vessels. Capillaries adjacent to or extending between tumor cells were focally observed. Most tumor cells were positive for glial fibrillary acidic protein and oligodendrocyte lineage transcription factor 2. The Ki-67 index was low. Based on the patient's history, clinical data, and pathological findings, she was diagnosed with angiomatous pleomorphic xanthoastrocytoma (WHO grade II).
This case serves as a reminder to pathologists of the need to be aware of this rare variant of pleomorphic xanthoastrocytoma to avoid a misdiagnosis of this indolent CNS tumor and therefore inappropriate treatment.
多形性黄色星形细胞瘤较为罕见,占所有中枢神经系统(CNS)肿瘤的比例不到1%。血管性多形性黄色星形细胞瘤是多形性黄色星形细胞瘤的一种极其罕见的变异型,迄今为止仅报道过6例。
一名24岁中国女性患者,出现癫痫发作和意识丧失15分钟,接受了计算机断层扫描和磁共振成像检查,结果显示左顶叶有一个肿块。组织学上,肿瘤的特征为多形性肿瘤细胞和显著的血管形成。血管性区域各不相同,从窦状模式到静脉畸形。在血管壁可见局灶性纤维蛋白样坏死、玻璃样变性以及淋巴细胞和浆细胞的中度浸润。肿瘤细胞与相邻的小血管紧密相邻。在肿瘤细胞之间或其附近局灶性观察到毛细血管。大多数肿瘤细胞胶质纤维酸性蛋白和少突胶质细胞谱系转录因子2呈阳性。Ki-67指数较低。根据患者的病史、临床资料和病理结果,她被诊断为血管性多形性黄色星形细胞瘤(世界卫生组织二级)。
该病例提醒病理学家需要认识到这种多形性黄色星形细胞瘤的罕见变异型,以避免对这种惰性中枢神经系统肿瘤误诊,从而避免不恰当的治疗。