Primary cutaneous extraskeletal Ewing sarcoma of the plantar region: clinicopathological features and review of the literature

Background∕Objectives: Primary cutaneous extraskeletal Ewing sarcoma (pcEES) is an exceptionally rare malignant small round cell tumor, with limited cases reported and challenging diagnosis due to overlapping clinicopathological features. We aimed to review its diagnostic and therapeutic aspects and present a rare plantar case. Study selection for review: A PubMed search was performed to evaluate current data regarding the epidemiology, diagnosis, differential diagnosis, and management of pcEES. Case presentation: A 62-year-old woman with recurrent pcEES of the left plantar region presented with severe pain, ulceration, superinfection, inguinal lymph node involvement, and suspected osseous invasion. Histopathology and immunohistochemistry supported the diagnosis. Due to advanced locoregional disease, left transfemoral amputation was performed. Conclusions: pcEES is a rare and diagnostically demanding malignancy that may show aggressive behavior despite superficial location. Early diagnosis and multidisciplinary management are essential.