Kaposiform hemangioendothelioma with distant lymphangiomatosis without an association to Kasabach-Merritt-Syndrome in a female adult!
نویسندگان
چکیده
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.
منابع مشابه
Kaposiform Hemangioendothelioma of Abdominal Wall Associated with Kasabach-Merritt in A Newborn with Dramatic Response to Sirolimus and Vincristine
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Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor and has a high mortality in newborns when associated with Kasabach-Merritt syndrome (KMS). In two newborns with KHE and severe KMS refractory to medical treatment, emergency embolization led to clinical improvement in the acute neonatal setting by reducing tumor volume, increasing the platelet count, and improving other clotting par...
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عنوان ژورنال:
- Vascular Health and Risk Management
دوره 4 شماره
صفحات -
تاریخ انتشار 2008