Invited Review : Three New Vascular Tumors—Tufted Angioma, Kaposiform Infantile Hemangioendothelioma, and Proliferative Cutaneous Angiomatosis

Abstract

The features of tufted angioma (Nakagawa), Kaposiform infantile hemangioendotheli oma, and proliferative cutaneous angiomatosis are reviewed. Tufted angioma, which usually appears before the age of ten years, is a large, poorly demarcated, macular, cutaneous and subcutaneous lesion most commonly located on the neck or trunk. Histologically, there is a "cannon ball" pattern of concentrically whorled lobules and tufts of endothelial and perithelial cells. Tufted angioma is difficult to excise and usually persists throughout life but causes no serious symptoms. Kaposiform infantile heman gioendothelioma is a rare, aggressive vascular tumor of the deep soft tissues, bone, and skin that presents during the first year of life and is often complicated by Kasabach- Merritt syndrome. Histologically, there is a Kaposiform pattern, but some cells may be arranged in nodules and tufts indistinguishable from tufted angioma. Kaposiform infantile hemangioendothelioma responds to interferon therapy. On the basis of the few cases so far recognized, proliferative cutaneous angiomatosis is a large, diffuse, mainly superficial angioma that grows insidiously and is biopsied in the second or third decade of life. Histologically, there are irregular, interconnecting cords and sheets of endothelial cells that form anastomosing spaces. Extravasated red cells and endothe lial hemosiderosis are prominent. The superficial location, older age, and the distinctive histology suggest that proliferative cutaneous angiomatosis differs from Kaposiform infantile hemangioendothelioma. Int J Surg Pathol 2(1):63-72, 1994