Diário da Espinha

Sclerosing Epithelioid Fibrosarcoma – A Rare Sacral Lesion and Literature Review

Abstract

Luís Rocha, Filipa Moreno, Joao Silva, Sérgio Moreira, Ricardo Taipa, Joaquim Reis, Mario Gomes and Ernesto Carvalho

Sclerosing epithelioid fibrosarcoma (SEF) is a singular entity, recognized as malignant fibroblastic sarcoma variant, which deviates from classic type due to the indolent progression and late metastasis. Typically occurring in middle aged adults, it presents a predilection for deep soft tissues, particularly skeletal muscle. It often involves the fascia or periosteum, and less frequently it may invade or arise from bone. Diagnosis and recognition are critical, as the generally bland appearance or when intraosseous similarity with an osteoid lesion, can lead to misdiagnosis. We herein present a case showing an unusual location and challenging radiological morphological and surgical features.