Ewing sarcoma family of tumors: Causes, diagnosis and treatment

Abstract

The Ewing's sarcoma Family of Tumors (EFT) includes classic Ewing's Sarcoma (ES) of bone, Extraskeletal Ewing's Sarcoma (EES) and malignant peripheral primitive Neuroectodermal Tumor (pNET) of bone and soft tissue. ES is an aggressive tumor with a high incidence of local recurrence and distant metastasis. The skeletal form is more common and typically occurs in the long bones of the extremities. The extra skeletal form occurs in the soft tissues of the lower extremities, paravertebral tissues, chest wall, retroperitoneum and rarely in the head and neck region in about 1-4% of cases. Involvement of the head and neck is usually identified in the nasal or oral cavities, sinuses or soft tissues of the neck.