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标题：Kaposiform hemangioendothelioma with distant lymphangiomatosis without an association to Kasabach-Merritt-Syndrome in a female adult!
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作者：Claudia S Vetter-Kauczok ; Philipp Ströbel ; Eva B Bröcker 等
期刊名称：Vascular Health and Risk Management
印刷版ISSN：1176-6344
电子版ISSN：1178-2048
出版年度：2008
卷号：4
期号：1
页码：263-266
DOI：10.2147/VHRM.S2348
出版社：Dove Medical Press Ltd
摘要：Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor which usually occurs in infants. Clinically it appears as ill-defined red to purple indurated plaque. KHE is commonly associated with Kasabach-Merritt syndrome (KMS) and lymphangiomatosis. Microscopically, the tumor is composed of infiltrating lobulated nodules with slitlike or crescentic vessels which are poorly canalized and lined by spindle shaped endothelial cells. We report a 36-year old female who developed a reddish tumor on the chest. Histological examination revealed a KHE, which was clinically not associated with thrombocytopenia or bleeding complications, but lymphangiomatosis at the right submandibular region. The association of KHE in a female adult with lymphangioma rather than KMS in this case supports the hypothesis that such an association may represent a benign subform of this disease in an adult and excision seems to be curative.
关键词：Kasabach-Merritt-Syndrome; Kaposiform hemangioendothelioma; lymphangiomatosis