Retiform hemangioendothelioma over forehead

Abstract

Retiform hemangioendothelioma (RH) is low grade tumor of skin and subcutaneous tissue. It needs to be differentiated from angiosarcoma as RH has excellent prognosis. It is usually seen in young adults on extremities. Sometimes it may mimic benign conditions and can delay treatment. Surgery has been mainstay of its treatment with or without adjuvant radiation. We present first case of RH on face. This is only second case being treated with definitive chemoradiation. So it's important to distinguish RH from angiosarcoma due to treatment implications as well.

Cutaneous Vascular Neoplasms of Uncertain Biological Behavior

“…RHE is a rare tumor that may be associated with underlying lymphedema or preceding radiation therapy. It occurs most often in middle-aged adults, but some cases have been reported in children [ 27 ]. The preponderance of tumors occurs in females [ 27 , 28 , 29 , 30 , 31 ].This tumor typically occurs on the trunk or extremities and is locally aggressive and rarely metastasizes; however, several cases reported lymph node metastases [ 32 , 33 , 34 , 35 , 36 , 37 , 38 ].…”

“…Tumor cells are reactive for vascular endothelial markers, including CD31, CD34, ERG, and FLI-1. Most reported cases are negative for lymphatic markers like D2-40 and VEGFR-3; however, a few rare cases have been identified expressing lymphatic markers [ 27 , 30 , 31 , 34 ].…”

“…Another critical distinction is to distinguish an RHE from cutaneous angiosarcoma due to treatment implications and the malignant potential of cutaneous angiosarcomas. Clinically, cutaneous angiosarcomas tend to occur in older populations and demonstrate higher levels of cytologic atypia and high mitotic rate [ 27 , 31 ].…”

“…3.4.1. Historical Aspects RHE was first described in 1994 by Calonje et al when it was distinguished as a separate entity from cutaneous angiosarcoma in a series of fifteen cases [27,28]. In 2010, Hirsh et al presented a case series of another 25 cases which fit the morphology of the RHE, and since then, there have been a few more cases reported, making the RHE overall exceedingly rare tumor [28][29][30][31].…”