Orbital intravascular papillary endothelial hyperplasia in a Nigerian child: a case report and review of the literature

Abstract

IntroductionIntravascular papillary endothelial hyperplasia is a reactive proliferative lesion of endothelial cells in blood vessels. It typically presents as a painless, reddish purple lesion in the sites affected. The orbit remains an uncommon site of affectation of this relatively common disease. It is noteworthy that this is the first reported case, to the best of our knowledge, of orbital intravascular papillary endothelial hyperplasia in a Nigerian child.Case presentationThe case reported here is an orbital intravascular papillary endothelial hyperplasia causing non-axial proptosis and loss of vision in a 14-year-old Nigerian boy. We describe the clinical and histological findings of intravascular papillary endothelial hyperplasia in the orbit of this 14-year-old boy. The key distinguishing features are discussed and relevant literature is reviewed.ConclusionAlthough unusual in presentation, intravascular papillary endothelial proliferation should be considered in the list of differentials of proptosis due to mass lesion in young Nigerians and, possibly, Africans.

Intravascular papillary endothelial hyperplasia: An unusual histopathological entity

“…The first theory originally proposed by Masson, states that endothelial proliferation is the primary phenomenon whereas the thrombus arises secondary to endothelial proliferation. [ 2 ] In contrast, Clearkin and Enzinger in 1976 suggested that this papillary structure appears after a preexisting thrombus organizes. Nowadays, it is considered to be a reactive vascular proliferation following traumatic vascular stasis.…”

“…In 1923, Pierre Masson first described an intravascular papillary proliferation formed within the lumen of inflamed hemorrhoidal plexus in a man and named it “Hemangioendotheliome vegetant intravasculaire.”[ 1 ] From the time of its initial description it has been referred to by various eponyms, including Masson's tumor, Masson's hemangioma, Masson's intravascular hemangioendothelioma, intravascular papillary endothelial hyperplasia (IPEH) or reactive papillary endothelial hyperplasia. [ 2 ] Masson described it to be a form of neoplasm and explained the pathogenesis as proliferation of endothelial cells into the vessel lumen, followed by obstruction and secondary degeneration and necrosis. On the other hand, Henschen[ 3 ] depicted the lesion as a reactive process rather than a neoplasm.…”

“…Kauffman and Stout[ 3 ] remarked that, although endothelial proliferation that can be easily mistaken for a characteristic of sarcoma is present, the endothelial layer of the lesion is composed of normal endothelial cells, the endothelial proliferation is of benign papillae pattern and the cells show no atypia. However, it is now believed to be a reactive vascular proliferation following traumatic vascular stasis, and the current terminology, IPEH, was proposed by Clearkin and Enzinger[ 2 ] in 1976. It is a rare benign condition mimicking angiosarcoma and hence it is being reported here.…”

Intravascular Papillary Endothelial Hyperplasia – Case Report

“…Moreover, angiosarcoma is rarely located intravascular, usually invading tissues outside the vessel. Solid areas and necrosis are frequent fi ndings in angiosarcomas 17 . Benign conditions such as hemangiomas, lymphangiomas, hematomas, vascular malformations, pyogenic granulomas, angiolymphoid hyperplasias with eosinophilia, fi bromas, traumatic neuromas, neurofi bromas, schwannomas should also be considered in the diff erential diagnosis of IPEH.…”

Masson’s Tumor of the Hand: An Uncommon Histopathological Entity

“…Intravascular papillary endothelial hyperplasia (IPEH) was first described by Pierre Masson as an intravascular papillary proliferation that forms within the lumen of an inflamed hemorrhoidal plexus, which was named vegetated intravascular hemangioendothelioma. Since then, various terms were considered for this pathology, such as hemangioendothelioma and reactive papillary endothelial hyperplasia or better known as Masson's hemangioma [1][2][3]. It can arise from normal blood vessels or in vascular malformations.…”