Intravascular Papillary Endothelial Hyperplasia Arising From the Upper Lip

Abstract

We report a rare case of intravascular papillary endothelial hyperplasia (IPEH) arising from the upper lip. Pathologically, it consisted of a few lobulated masses lined by an incomplete fibrous capsule of variable thickness which was separated from the surrounding tissue and which partially formed papillary structures bearing fibrous stalks and a single layer of endothelium. The capillary formation was poorly defined, and mitotic figures were frequently observed. Immunohistochemically, the endothelial cells were positive for factor VIII related antigen and vimentin, and many cells were positive for PCNA, not only in the solid proliferating area but also in the papillary proliferating area. This case represents IPEH with high proliferative activity.

Intravascular papillary endothelial hyperplasia of the oral cavity

“…IPEH is believed to be a reactive proliferation of blood endothelial cells caused by an abnormal process of organization in thrombosed blood vessels (5)(6)(7)(8).…”

Intravascular Papillary Endothelial Hyperplasia: A Rare Vascular Lesion Affecting Submandibular and Sublingual Region

“…However, the pathogenesis remains unknown. IPEH is now accepted as a reactive proliferation of endothelial cells arising in an organizing thrombus, possibly related to trauma, which justifies the presence of these lesions on lip cheek or tongue, as these are the most commonly affected by trauma in oral region [3] [4]. The patient was unaware of any recent trauma to the submandibular and sublingual region, but thrombosis which IPEH maybe arise from were observed under microscope.…”

“…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]. IPEH is relatively uncommon in the region of head and neck.…”

“…IPEH is relatively uncommon in the region of head and neck. The most common site is the lower lip, followed by the tongue and the upper lip [1] [3] [4]. To date, there is only one document reporting a case which affects the right submandibular region [5].…”

“…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]. In this article, we report an uncommon case affected the right submandibular and sublingual space which was misdiagnosed as venous malformations and malignant vascular tumors initially.…”