Hemangioendotheliomas are a family of vascular neoplasms of intermediate malignancy. Signs and symptoms
They have been described as masses that fall between a hemangioma and angiosarcoma. 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. Cause
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Diagnosis
Classification
Hemangioendotheliomas may be classified as:

Epithelioid hemangioendothelioma is an uncommon vascular tumor of intermediate malignancy that was first described by Steven Billings, Andrew Folpe, and Sharon Weiss in 2003. These tumors are so named because their histologic appearance resembles a proliferation of epithelioid cells, with polygonal shape and eosinophilic cytoplasm. Composite hemangioendothelioma is a low-grade angiosarcoma typically occurring in adults, although it has been described in infancy. : 601 
Spindle-cell hemangioendothelioma) is a vascular tumor that was first described in 1986 by Sharon Weiss, M.D., and commonly presents in a child or young adult who develops blue nodules of firm consistency on a distal extremity. : 599  These tumors were reclassified by Dr. Weiss in 1996 as "spindle cell hemangioma", rather than hemangioendothelioma, due to the excellent prognosis observed in a group of 78 patients. Retiform hemangioendothelioma (also known as a "Hobnail hemangioendothelioma") is a low-grade angiosarcoma, first described in 1994, presenting as a slow-growing exophytic mass, dermal plaque, or subcutaneous nodule. : 601 
Kaposiform hemangioendothelioma (also known as "Infantile kaposiform hemangioendothelioma") is an uncommon vascular tumor, first described by Niedt, Greco, et al.
Infants who have severe anemia and/or thrombocytopenia can be given blood products; for those who have cardiac failure, diuretics and digoxin are often given. To stop further growth and to speed regression of lesions in infants with more significant clinical sequelae, treatment with corticosteroids or interferon-α-2a is administered. To slow the growth of tumors that are rapidly enlarging, chemotherapy and radiation therapy have been used. Surgical resection, partial hepatectomy, and embolization of afferent vessels should be considered for severe cases. Treatment
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. References


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