Oliveira Rui Caetano, Velasco Ricardo, Santos Gonçalo Roque, Rebelo Olinda
Department of Pathology, Hospitais da Universidade de Coimbra, Coimbra, Portugal.
Department of Neurosurgery, Centro Hospitalar Tondela Viseu EPE, Viseu, Portugal.
BMJ Case Rep. 2018 Jan 23;2018:bcr-2017-223111. doi: 10.1136/bcr-2017-223111.
A 49-year-old female with history of headache, nausea and vomiting with some weeks of evolution, without neurological symptoms. Radiology revealed an expansive lesion near the inferior vermix and cerebellar tonsils, with heterogeneous gadolinium uptake and mass effect on the fourth ventricle, representing a probable extraventricular origin for the lesion. Pathological examination showed a proliferation of oval/spindle cell proliferation with eosinophil cytoplasm and small and monotonous nuclei, without mitoses. The cells had a concentric growth, surrounding thin-walled blood vessels with foci of stromal myxoid degeneration and whorled pattern. The vessels had a haemangiopericytoma pattern and were lined by non-atypical endothelial cells. The tumorous cells expressed vimentin, alpha-smooth actin and heavy-chain caldesmon and were negative for epithelial membrane antigen, protein S100, HMB45, CD34, calponin and desmin, thus providing the final diagnosis of intracranial myopericytoma.
一名49岁女性，有头痛、恶心和呕吐病史，病程数周，无神经症状。影像学检查显示在小脑蚓部下份和小脑扁桃体附近有一占位性病变，钆摄取不均匀，对第四脑室有占位效应，提示该病变可能起源于脑室外。病理检查显示椭圆形/梭形细胞增生，胞质嗜酸性，核小且单一，无核分裂象。细胞呈同心性生长，围绕薄壁血管，伴有间质黏液样变性灶和漩涡状结构。血管呈血管外皮细胞瘤样，内衬无异型性的内皮细胞。肿瘤细胞表达波形蛋白、α-平滑肌肌动蛋白和钙调蛋白重链，上皮膜抗原、蛋白S100、HMB45、CD34、钙蛋白和结蛋白均为阴性，最终诊断为颅内肌周细胞瘤。
