Abstract

Intravascular papillary endothelial hyperplasia is an unusual benign vascular lesion of the skin and subcutaneous tissue consisting of papillary formations lined with proliferative endothelial cells. The clinical characteristic is not specific and the diagnosis is based on histological examination. The lesion is often mistaken for angiosarcoma and a group of other benign and malignant vascular lesions. IPEH has been relatively rarely described in the head and neck region. An uncommon case located in the submandibular and sublingual region is presented which was misinterpreted as venous malformations and malignant tumors of vascular origin initially. A surgical excision was performed and no recurrences have been found after 5 years of follow-up.

Highlights

  • In 1923, Masson described a case of a 68-year-old man with an ulcerated hemorrhoid that could not be reduced

  • We report an uncommon case affected the right submandibular and sublingual space which was misdiagnosed as venous malformations and malignant vascular tumors initially

  • intravascular papillary endothelial hyperplasia (IPEH) can occur in any blood vessel in the body but most commonly it affects the skin and subcutaneous tissues of the fingers and trunk [4] [5]

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Summary

Introduction

In 1923, Masson described a case of a 68-year-old man with an ulcerated hemorrhoid that could not be reduced. In 1976, Clearkin and Enzinger coined the term intravascular papillary endothelial hyperplasia (IPEH) which seems to be more descriptive and less confusing and is the most frequently used in the English literatures obtained [1]. IPEH is an unusual benign non-neoplasm especially rarely found in head and neck region comprising approximately 2% of the vascular tumors of the skin and subcutaneous tissue. IPEH is characterized by papillary fronds lined by proliferating endothelium within a vascular lumen [1] [2]. The main significance of IPEH is its clinical and microscopic resemblance to angiosarcoma and its possible misinterpretation as vascular malformations or mucocele [1] [3] [4]. We report an uncommon case affected the right submandibular and sublingual space which was misdiagnosed as venous malformations and malignant vascular tumors initially

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