Intravascular papillary endothelial hyperplasia of the mouth: report of six cases and literature review.

Abstract

Oral intravascular papillary endothelial hyperplasia (IPEH) is an uncommon, reactive vascular lesion with no specific clinical features. Microscopically two subtypes have been described: a pure and a mixed form. The importance of IPEH is in its resemblance to angiosarcoma. The aim of this study was to evaluate both the prevalence of IPEH in a consecutive series of oral vascular malformations and the clinico-pathological features. Histological sections of 103 consecutive cases filed as vascular malformations were reviewed for histopathological criteria of IPEH. We found six cases of oral IPEH, of which three were diagnosed previously. Five of these cases were in males, and the mean age was 58 years. It was usually described as a blue or reddish nodule. The lower lip mucosa was the most common site followed by the tongue and the upper lip. Histologically, thrombi were always present and five out of the six lesions appeared in a mixed form, while only one was in a pure form. Three cases were diagnosed at an early stage and in the other three, the lesions were well-established. In this series, IPEH associated with vascular malformation was more common than reported in previous studies. Lesions at early stages, especially in the mixed form, may be unnoticed.