Oral intravascular papillary endothelial hyperplasia

Abstract

Intravascular papillary endothelial hyperplasia (IPEH) is usually a cutaneous lesion thought to represent a peculiar manifestation of an organizing thrombus. IPEH can be subdivided into a pure form that occurs within a dilated vascular space and a mixed form that appears as a focal change within a preexisting vascular lesion, such as hemangioma. The principle significance of the lesion lies in the fact that it may be mistaken for angiosarcoma. Oral lesions are uncommon and the present paper reports on 16 new cases of oral IPEH, analyzing their clinical and histomorphologic features. Histologically, 11 lesions appeared in a pure form and 5 lesions in a mixed form. Thrombi were detected in all but one case. The most common location was the lip, followed in decreasing frequency by the tongue and buccal mucosa. Local excision was the treatment of choice.

Extravascular type of intravascular papillary endothelial hyperplasia mimicking parotid gland neoplasia and the possible role of ferritin in the pathogenesis: A case report

“…Levere et al reported elevated level of basic fibroblast growth factor (bFGF) in cases of IPEH when compared with non-IPEH organizing thrombi; in addition, they suggested that bFGF was released by macrophages in response to trauma (23). A number of studies pointed to IPEH as an unusual form of thrombus that undergoes fragmentation, followed by active endothelization of its fragments (24). Accordingly, Albrecht et al described the positivity of thrombus-lining cells for ferritin, vimentin and factor VIII-related antigen, indicating cells progressing from a histiocytic to an endothelial phenotype (25).…”

Intravascular Papillary Endothelial Hyperplasia Arising From the Upper Lip

“…It is characterized histologically by papillary fronds lined by proliferating endothelium 17) . IPEH is in fact the proliferation of endothelial cells or vascular malformation, the main significance of this intravascular endothelial hyperplasia is its microscopic resemblance to angiosarcoma and its possible misdiagnosis as such 3,7,10,17) . It is important to recognize such lesions histologically because they may mimic the appearance of angiosarcoma in their proliferation of endothelial cells, mitotic activity, and papillary projections.…”

“…Furthermore, the principal significance of the lesion lies in the fact that it may be mistaken for angiosarcoma 3) . Table 1 shows the locations of IPEH in the literature, showing that it is slightly more frequent in the lower lip around the oral area.…”

“…Thus the incidence of IPEH is only 0.5% of vascular lesions from the oral area in our hospital. IPEH and angiosarcoma may be exceedingly difficult to distinguish, but there are several important hallmark characteristics of IPEH: (1) the proliferative process is entirely confined to an intravascular space, (2) most of the papillary structures are associated with thrombi, (3) the papillae consist of fibrohyalinized tissue that is covered by no more than two endothelial cell layers, (4) the endothelial cells may be hyperchromatic, but extreme nuclear atypia and frequent mitotic figures are absent, (5) tissue necrosis is absent 3) . This case adequately exhibits the above-mentioned characteristics of IPEH, except for the mitotic figures.…”

“…It is characterized histologically by papillary fronds lined by proliferating endothelium 17) . IPEH in the oral area is relatively uncommon; it usually presents as a cutaneous lesion 2,3,9) . We report a relatively rare case of an IPEH in the upper lip.…”