Aditya G S, Santosh Vani, Yasha T C, Shankar S K
Department of Neuropathology, National Institute of Mental Health & Neurosciences, NIMHANS, Hosur Road, Bangalore.
Indian J Pathol Microbiol. 2003 Oct;46(4):645-9.
Hemangioendothelioma (HE) is a borderline or intermediate type of vascular neoplasm. We report clinical and histopathological characteristics of four cases of HE arising from the skull bones because of its extreme rarity in this location. The age of the patients ranged from 6-45 years. Three patients presented with a painless swelling over the head and one case had sphenoid wing mass with dimness of vision and proptosis. Radiographic images showed a well-demarcated, osteolytic lesion in the skull bone in all the four, one case in addition had sclerotic edges and another had specks of calcification. Grossly, the tumour was very vascular with hemorrhagic areas. Histologically, three cases showed features of an epithelioid variant of HE, with short strands and solid nests of rounded to slightly spindled, eosinophilic endothelial cells, some of them having small intracellular vacuoles. The stroma was myxoid--hyalinised with focal mixed inflammatory infiltrate. One case had features of a 'retiform' histological variant composed of numerous elongated vessels lined by a single layer of hobnail endothelial cells, focal lymphocytic infiltrate and papillae with hyaline collagenous cores. The tumour cells in all the four were immuno-labelled by antibody to factor VIII-associated protein. The tumour cells lacked cytological atypia and mitosis was sparse. These features were important in prognostication as low-grade tumours can be cured by complete wide-resection.
血管内皮瘤（HE）是一种介于良恶性之间的血管肿瘤。由于其在颅骨部位极为罕见，我们报告了4例起源于颅骨的HE的临床和组织病理学特征。患者年龄在6至45岁之间。3例患者表现为头部无痛性肿胀，1例有蝶骨翼肿物并伴有视力减退和眼球突出。影像学图像显示所有4例患者颅骨均有边界清晰的溶骨性病变，1例病变边缘有硬化，另1例有钙化斑点。大体上，肿瘤血管丰富，有出血区域。组织学上，3例表现为HE上皮样变异型特征，有短束状和实性巢状排列的圆形至轻度梭形嗜酸性内皮细胞，部分细胞有小的细胞内空泡。间质呈黏液样-玻璃样变，有局灶性混合性炎症浸润。1例具有“网状”组织学变异型特征，由众多被单层鞋钉样内皮细胞衬覆的细长血管、局灶性淋巴细胞浸润以及带有透明胶原核心的乳头构成。所有4例肿瘤细胞均被抗VIII因子相关蛋白抗体免疫标记。肿瘤细胞缺乏细胞学异型性，有丝分裂少见。这些特征对预后很重要，因为低级别肿瘤通过完整的广泛切除可以治愈。
