Epithelioid Sarcoma Presenting as Non-Healing Traumatic Ulcer: A Case Report and Review of the Literature

Abstract

Epithelioid sarcoma is a rare soft tissue sarcoma. It is a slow-growing neoplasm, which usually presents as a painless mass in the extremities and typically grows along deep dermal and subcutaneous planes. In contrast to other types of sarcoma, it has a strong tendency for nodal metastasis and local metastasis adjacent to the primary lesion within the affected limb. In this article, we present a case of chronic traumatic ulcer in the upper extremity in an adolescent male that was subsequently diagnosed as epithelioid sarcoma, which is a very unusual mode of presentation of this particular tumour. The patient was treated with wide local excision and reconstruction with a free flap. Histopathological examination and immunochemistry studies confirmed the diagnosis and the patient underwent radiotherapy post-operatively as a part of the treatment regime. His post-treatment period was unremarkable, and he was put on regular surveillance to monitor the development of any signs of disease recurrence. Patients with epithelioid sarcomas often present late due to the slow-growing nature of the tumour. Unusual presentations like this will further delay the diagnosis and treatment, which will eventually worsen the prognosis. Awareness of such presentations can encourage primary care physicians to make early referrals to experts, which, in turn, may help the patients get early treatment and have a better prognosis.

Sarcoma epitelioide de tipo proximal de vulva

“…Existen dos tipos histológicos. El distal (o clásico) es el más frecuente y aparece en extremidades (especialmente manos y muñecas) (5) . El tipo proximal (o axial) surge de los tejidos blandos en zonas proximales (pelvis, periné, genitales), con características histológicas muy distintas.…”

“…El pronóstico de los pacientes con sarcoma epitelioide de tipo proximal en general es desfavorable, debido a la alta frecuencia de recurrencia local y metástasis tempranas. La tasa de supervivencia a los 5 años de los pacientes con tumores proximales es peor que la de los tipos distales, que se encuentra entre 50% y 80% (5) . Aquellos pacientes con tumores mayores de 3 centímetros de diámetro, invasión vascular, índice mitótico elevado superior a 2 por 10 campos de alta potencia, localización profunda y/o necrosis focal tienen peor pronóstico (2,13) .…”