Mentzel T, Mazzoleni G, Dei Tos A P, Fletcher C D
Department of Pathology, University of Jena, Germany.
Am J Clin Pathol. 1997 Oct;108(4):450-5. doi: 10.1093/ajcp/108.4.450.
Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm that previously has been described only in children. We report three cases occurring in adults. The patients, two men and one woman, were aged 64, 55, and 48 years, respectively. Lesions arose on the chest wall, the lateral aspect of the neck, and in the groin and buttock, and each was situated in superficial soft tissue. Tumor size ranged from 0.9 to 7 cm. Histologically, in all cases a lobular or nodular growth pattern of densely packed spindle-shaped tumor cells closely associated with small slitlike and sievelike blood vessels was seen. Within one lesion, small nodules of plumper, epithelioid tumor cells were identified. Well-formed capillaries frequently containing fibrin thrombi were observed mainly in the periphery of tumor nodules. Areas of hemorrhage and hemosiderin deposits were observed in two cases. Associated lymphangiomatosis composed of diffusely infiltrating lymphatic spaces of the dermis and subcutis was present in one case. Nuclear atypia was minimal in all cases, and mitotic figures were rare in two cases; in one case up to 6 mitoses in 10 high-power fields were noted focally. Immunohistochemically, well-formed capillaries stained positively for all the vascular markers used; however, endothelial cells lining the small slitlike and sievelike vascular spaces did not stain for von Willebrand factor. At least focally, actin-positive pericytes were seen. Neoplastic spindled cells stained only focally for CD34 and CD31. Two lesions were excised with clear margins; in one case only repeated biopsies were done. Follow-up information between 14 and 28 months revealed no sign of recurrence or metastasis. In adults, the differential diagnosis of KHE comprises especially Kaposi's sarcoma and spindle cell hemangioendothelioma; further differential diagnoses include tufted hemangioma and cellular capillary hemangioma, which occur rarely in adults.
卡波西型血管内皮瘤（KHE）是一种罕见的血管肿瘤，此前仅在儿童中被描述过。我们报告了3例发生在成人中的病例。患者为2名男性和1名女性，年龄分别为64岁、55岁和48岁。病变分别出现在胸壁、颈部外侧以及腹股沟和臀部，均位于浅表软组织。肿瘤大小在0.9至7厘米之间。组织学上，所有病例均可见密集排列的梭形肿瘤细胞呈小叶状或结节状生长模式，与小的裂隙状和筛状血管紧密相关。在一个病变中，发现了较丰满的上皮样肿瘤细胞的小结节。主要在肿瘤结节周边观察到常含有纤维蛋白血栓的成熟毛细血管。2例观察到出血和含铁血黄素沉积区域。1例存在由真皮和皮下组织弥漫性浸润的淋巴管构成的相关淋巴管瘤病。所有病例核异型性均极小，2例有丝分裂象罕见；1例在10个高倍视野中局部可见多达6个有丝分裂象。免疫组化方面，成熟毛细血管对所用的所有血管标志物均呈阳性染色；然而，小裂隙状和筛状血管腔的内皮细胞对血管性血友病因子不着色。至少在局部可见肌动蛋白阳性的周细胞。肿瘤性梭形细胞仅局部对CD34和CD31染色。2个病变切除边缘清晰；1例仅进行了重复活检。14至28个月的随访信息显示无复发或转移迹象。在成人中，KHE的鉴别诊断尤其包括卡波西肉瘤和梭形细胞血管内皮瘤；进一步的鉴别诊断包括簇状血管瘤和细胞性毛细血管瘤，这在成人中很少见。
