Clinical Report of Intravascular Papillary Endothelial Hyperplasia

Purpose: Intravascular papillary endothelial hyperplasia (IPEH), also known as Masson's pseudoangiosarcoma, is a rare disease which is now considered as a reactive process of the endothelium rather than a benign neoplasm. It can occur in any blood vessels in the body but more common in the head and neck region as a solitary, often tender, bluish or reddish nodule. IPEH is characterized by the development of endothelial-lined papillary projections in a vascular lumen, usually associated with thrombotic material, the endothelial cells in the papillary structures showing only slight atypia and occasional mitotic Figures, the absence of tissue necrosis. Methods: 8 patients with IPEH were enrolled in the study from 2002 to 2007. All 8 lesions were surgically excised for histopathologic diagnosis. Results: 4 patients were female. The duration of the lesions ranged from 3 months to 15 years. The tumors were first noted between the ages of 20 and 72 years. 4 patients had lesions on the head; 2 on the toe; 1 on the back; and 1 on the finger, respectively. All lesions were solitary, ranged in size from 2 mm to 27 mm. There were no recurrences. Conclusion: The clinical appearance of IPEH is not specific, presented as a primary neoplasm, and the diagnosis can be established by microscopic examination. Complete surgical excision is the best choice of therapy for patients with IPEH, and is both diagnostic and curative. Awareness of this lesion will prevent incorrect diagnosis and overly aggressive treatment.