Treatment of uncomplicated kaposiform hemangioendothelioma and tufted angioma

There is no evidence-based standard of care for kaposiform hemangioendotheliomas and tufted angiomas. Treatment varies according to size, location, presence of symptoms, and severity of coagulopathy.

Treatment options for uncomplicated kaposiform hemangioendotheliomas and tufted angiomas include the following:

Observation is an option for patients with low-risk tumors (i.e., no Kasabach-Merritt phenomenon, small tumor size, asymptomatic). Spontaneous regression and/or stability has been noted.[16]

Kaposiform hemangioendotheliomas and tufted angiomas that are uncomplicated and localized can be treated with surgical excision, pulse-dye laser, or topical agents (steroids, sirolimus, or tacrolimus).[16-18]

Propranolol therapy has been reported as a treatment option for patients with kaposiform hemangioendotheliomas based on positive results of propranolol use for other more benign vascular tumors. Results have been mixed, with a report of improved effectiveness using higher doses of propranolol.[19,20] Preliminary results indicate that propranolol should be reserved for patients with kaposiform hemangioendotheliomas without Kasabach-Merritt phenomenon and with smaller, less complicated lesions.