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Epithelioid hemangioendothelioma is an intermediate-grade vascular tumor arising from the vascular endothelium, which usually arises in soft tissue, and skin involvement is extremely rare. We report a case that presented with primary cutaneous tumor involving the whole limb and was present since birth.
Epithelioid hemangioendothelioma (EHE) is a rare tumor of the vascular endothelium that is considered to be intermediate between hemangioma and angiosarcoma. It was first described by Weiss and Ensinger in 1982. Typically, the soft tissue is involved and, less frequently, the skin, bone, liver and lung are involved. In this report, we describe a case of cutaneous EHE that was present since birth involving the whole of the right lower limb.
A 16-year-old female presented with painful progressive swelling and erythematous skin lesions involving the whole of her right lower limb. She had lesions since birth and also had restricted movements but with no other systemic complaints. An examination revealed diffuse swelling of the right lower limb, including the vulva and the lower abdomen. There were multiple erythematous papules, nodules and plaques of varying size that were firm in consistency [Figure 1].
On subjecting the patient for investigations, her bleeding time, clotting time, prothrombin time and complete blood count were normal. Her renal and hepatic parameters were also normal. X-ray [Figure 2] and computed tomography scan revealed soft tissue swelling with diffuse osteolysis of the femur, tibia and fibula. A tentative diagnosis of Gorham's syndrome was made. The other differential diagnosis was Klippel Trenaunay syndrome, but due to the presence of osteoporotic changes in the bone, this was excluded.
Biopsy of the erythematous nodule showed nodular tumor in the dermis, which was composed of oval- to spindle-shaped epithelioid cells. Multiple vascular spaces containing red blood cells were seen [Figure 3]. The nuclei showed a coarse chromatin pattern, and occasional mitotic figures were also noted. The cells were strongly immunoreactive for CD34 [Figure 4] and factor VIII-related antigen. Finally, a diagnosis of EHE was made and the patient was referred to the vascular surgery department for further management.
Weiss and Ensinger originally described 41 cases of EHE in soft tissue. EHE has also been reported in the liver, bone, gingiva, mediastinum and lung. EHE is most commonly located on the extremities, and tumor is evenly distributed through all the adult age groups. The tumor affects both sexes equally and no predisposing factors have been identified. The neoplasm usually presents as solitary, rarely multiple, slightly painful erythematous papules, nodules, plaque and nonhealing ulcer.
Microscopic examination reveals aggregates of oval to polygonal cells with abundant eosinophilic cytoplasm and round nucleus.[6,7] The nuclear chromatin pattern is vesicular and frequent prominent nucleoli are present. Nuclear pleomorphism is minimal. Intracytoplasmic vacuolization and occasional intraluminal erythrocytes are regular features. The tumor cells are embedded in a hyalinized or myxoid stroma.
Establishing the final diagnosis may sometimes be assisted by the use of immunocytochemical techniques. The more commonly used antisera are factor VIII-related antigen, CD 31, CD34 and Ulex europeus lectin.
Our case is unusual because of its presence since birth and because of its location in the dermis and involving the underlying bone with diffuse osteolysis. A case of cutaneous EHE with underlying bone involvement was described by Malane, Sau, Benson in 1992.
The prognosis of EHE is uncertain as the mortality rate for EHE of the liver is 35% and lung is 65%. It would seem that the prognosis in primary cutaneous lesions may be good. Treatment should be limited to simple surgical excision, provided the possibility of an underlying bone lesion has been excluded.
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