Kaposiform hemangioendothelioma with distant lymphangiomatosis without an association to Kasabach-Merritt-Syndrome in a female adult

Kaposiform hemangioendothelioma with distant lymphangiomatosis without an association to Kasabach-Merritt-Syndrome in a female adult

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 comp...

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Bibliographic Details
Published in:Vascular health and risk management Vol. 4; no. 1; pp. 263 - 266
Main Authors: Vetter-Kauczok, Claudia S, Ströbel, Philipp, Bröcker, Eva B, Becker, Jürgen C
Format: Journal Article
Language:English
Published: New Zealand Dove Press 2008
Dove Medical Press
Subjects:
Adult
Case Report
Diagnosis, Differential
Female
Hemangioendothelioma - complications
Hemangioendothelioma - pathology
Hemangioendothelioma - surgery
Humans
Immunohistochemistry
Kaposiform hemangioendothelioma
Kasabach-Merritt-Syndrome
Lymphangioma - complications
Lymphangioma - pathology
Lymphangioma - surgery
lymphangiomatosis
Sarcoma, Kaposi - complications
Sarcoma, Kaposi - pathology
Sarcoma, Kaposi - surgery
Skin Neoplasms - complications
Skin Neoplasms - pathology
Skin Neoplasms - surgery
Thrombocytopenia
lymphangiomatosis
Kaposiform hemangioendothelioma
Kasabach-Merritt-Syndrom
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Description
Summary: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.
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ISSN:1176-6344
1178-2048
1178-2048
DOI:10.2147/vhrm.2008.04.01.263