Ewing’s Sarcoma of the Pelvis

Ewing’s sarcoma is the most common malignant bone tumor of the pelvis in children and adolescents. It often presents late, with an insidious onset of vague symptoms. Histology is notable for sheets of small, round blue cells, and the EWSR1 gene rearrangement is present in 95% of cases. It continues to have the worst prognosis of Ewing’s tumors due to large size at diagnosis, frequent metastases, and difficult surgical approach and reconstruction. The presence of metastatic disease at presentation is the most important prognostic factor. Treatment approach generally consists of neoadjuvant chemotherapy, surgical resection with or without pelvic reconstruction, and adjuvant chemotherapy. The addition of radiation may be indicated in some patients.