Kimura Tokuhiro, Mukai Makio, Kaneko Yuko, Hirakata Michito, Okamoto Shinichiro, Sakamoto Michiie, Okada Yasunori, Ikeda Yasuo
Department of Pathology, Keio University School of Medicine, Tokyo, Japan.
Pathol Int. 2006 Nov;56(11):694-701. doi: 10.1111/j.1440-1827.2006.02032.x.
Vascular neoplasms characterized by epithelioid endothelial cells consist of several different entities from benignity to high-grade malignancy. Because of histological overlap between them, there is substantial difficulty in classifying them correctly. The present patient, a 33-year-old man, presented with hepatomegaly, striking eosinophilia and elevated serum interleukin-5 level. Biopsy and autopsy revealed an unusual epithelioid vascular tumor in the liver, which is histologically distinct from epithelioid hemangioma, epithelioid hemangioendothelioma, or epithelioid angiosarcoma. The tumor cells had vasoformative and partly solid growth with no severe nuclear atypia and very low mitotic activity, and the histological features were similar to those of the entity recognized as hemangioendothelioma of bone. Organs other than the liver, for example the testes and bone, were also involved. This tumor should be considered in the differential diagnosis of severe eosinophilia.
以上皮样内皮细胞为特征的血管肿瘤包括从良性到高级别恶性的几种不同实体。由于它们之间存在组织学重叠，正确分类存在很大困难。本例患者为一名33岁男性，表现为肝肿大、显著嗜酸性粒细胞增多和血清白细胞介素-5水平升高。活检和尸检显示肝脏中有一种不寻常的上皮样血管肿瘤，其组织学特征与上皮样血管瘤、上皮样血管内皮瘤或上皮样血管肉瘤不同。肿瘤细胞呈血管形成性和部分实性生长，无严重核异型性，有丝分裂活性极低，组织学特征与被认为是骨血管内皮瘤的实体相似。肝脏以外的器官，如睾丸和骨骼，也有累及。在鉴别诊断严重嗜酸性粒细胞增多症时应考虑这种肿瘤。
