Intravascular Papillary Endothelial Hyperplasia

INTRODUCTION Intravascular papillary endothelial hyperplasia (IPEH) is an unusual benign vascular lesion of proliferating endothelial cells in response to thrombus formation. It is most frequently seen in the extremities, but it can occur in other parts of the body as well. It can appear as a primary lesion developing within the lumen of a distended vessel, or it can be associated with other vascular lesions such as hemangioma, pyogenic granuloma, or lymphangioma. Lesions are typically associated with a thrombus in varying stages of development. IPEH clinically and histologically resembles soft-tissue angiosarcomas with which they are most often confused.

CLINICAL PRESENTATION IPEH is usually seen in middle-aged adults and presents as a bluish or purple mass within the skin or subcutaneous tissues. It is confined to the intraluminal space of either an artery or vein, and the walls of the vessel form the "capsule" of the mass. This lesion is not associated with systemic or local disease. As the lesion enlarges it can compress adjacent soft tissues resulting in ptosis, ectropion or other eyelid malpositions. It can occur deep in the orbit where it can involve the ophthalmic artery and other orbital vessels.

(Courtesy of Tamara Fountain, M.D.) (Courtesy of Tamara Fountain, M.D.)

Intravascular Papillary Endothelial Hyperplasia (Contd.)

HISTOPATHOLOGY This lesion lies within a blood vessel of the dermis or subcutis. It is composed of numerous, small, eosinophilic papillae covered by a layer of endothelial cells that are often flattened. The endothelial cells lack cytological atypia and mitotic activity. Thrombus is often adjacent to the lesion and is commonly in varying stages of organization.

DIFFERENTIAL DIAGNOSIS The most important lesion in the differential diagnosis is angiosarcoma from which it must be distinguished. Other similar lesions include pyogenic granuloma, hemangioma, intravascular fasciitis, and organized thrombus.

TREATMENT Biopsy is often required for diagnosis. Management is surgical by opening the vessels and removing the mass. For lesions in areas that are not readily accessible surgically, like the deep orbit, radiosurgery has proven effective.


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