They are vascular tumors that commonly present with an enlarging mass and most commonly involve the lungs, liver, and musculoskeletal system, although many other body sites have been reported, including the head and neck, intestines, lymph nodes, pleura, retroperitoneum, heel, stomach.

[citation needed] Possibly Bartonella spp bacteraemia[citation needed] Hemangioendotheliomas may be classified as: Treatment is varied and depends on the site and extent of tumor involvement, sites of metastasis, and specific individual factors.

Surgical resection, radiotherapy, and chemotherapy have all been used to treat these masses, although studies on survival have yet to be conducted to delineate various treatment regimens.

Kaposiform hemangioendothelioma might respond to chemotherapy or antiangiogenic therapies.

Recently propanolol and steroids have been shown to be very effective in kaposiform hemangioendothelioma.