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.

REFERENCES

Barras C, Olver JM, Cole C, Seet JE. Intravascular papillary endothelial hyperplasia (IPEH) mimicking a lacrimal sac mass. Eye 2001; 15:685-687.

Cagli S, Oktar N, Dalbasti T, et al. Intravascular papillary endothelial hyperplasia of the central nervous system—four case reports. Neurol Med Chir 2004; 44:302-310.

Font RL, Wheeler TM, Boniuk M. Intravascular papillary endothelial hyperplasia of the orbit and ocular adnexa. A report of five cases. Arch Ophthalmol 1983; 101:1731-1736.

Ohshima T, Ogura K, Nakayashiki N, Tachibana E. Intravascular papillary endothelial hyperplasia at the superior orbital f issure: report of a case successfully treated with gamma knife radiosurgery. Surg Neurol 2005; 64:266-269.

Patt S, Kaden B, Stoltenburg-Didinger G. Intravascular papillary endothelial hyperplasia at the fissure orbitalis superior: a case report. Clin Neuropathol 1992; 11:128-130.

Shields JA, Shields CL, Eagle RC Jr, Diniz W. Intravascular papillary endothelial hyperplasia with presumed bilateral orbital varices. Arch Ophthalmol 1999; 117:1247-1249.

Sorenson RL, Spencer WH, Stewart WB, Miller WW, Kleinhenz RJ. Intravascular papillary endothelial hyperplasia of the eyelid. Arch Ophthalmol 1983; 101:1728-1730.

Truong L, Font RL. Intravascular papillary endothelial hyperplasia of the ocular adnexa. Report of two cases and review of the literature. Arch Ophthalmol 1985; 103:1364-1367.

Weber FL, Babel J. Intravascular papillary endothelial hyperplasia of the orbit. Br J Ophthalmol 1981; 65:18-22.

Werner MS, Hornblass A, Reifler DM, Dresner SC, Harrison W. Intravascular papillary endothelial hyperplasia: collection of four cases and a review of the literature. Ophthal Plast Reconstr Surg 1997; 13:48-56.

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