Hemangioendothelioma

Published on 11/06/2015 by admin

Filed under Pediatrics

Last modified 22/04/2025

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Sudden change of a hemangioendothelioma to deep purple in Kasabach−Merritt syndrome.

Kaposiform hemangioendothelioma of baby.

CLINICAL FEATURES

Kaposiform hemangioendotheliomas (KHE) are rare vascular tumors that may be present at birth and are usually seen before 2 years of age. Lesions are usually solitary.

Mature lesions have a nodular infiltrative growth pattern and a characteristic purple hue. They have also been described as occurring in the peritoneum.

KHE are very aggressive locally and, although not malignant, may be fatal.

Fatalities are usually associated with the Kasabach−Merritt syndrome. Spontaneous regression is not expected.

TREATMENT

Optimal treatment for small lesions is surgical excision, but unfortunately very few lesions present at a small enough size to be amenable to this therapy. Some KHE respond well to methylprednisolone 1−2 mg/kg/day.

Vincristine is a second-line therapy for steroid treatment failures.

Kasabach−Merritt syndrome should be treated aggressively.

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