Long-term, disease-free survival of a patient with a primitive neuroectodermal/ewing sarcoma in the mobile spine with extracompartmental extension

Study Design Case report. Introduction Long-term survival of a patient with a Ewing sarcoma family of tumors/primitive neuroectodermal tumors of the central spine with pathologic fracture and extradural extension is presented. Literature-based evidence for a survival benefit with modern neoadjuvant chemotherapy and en bloc resection with and without radiotherapy is reviewed. Case Report More than 10-year clinical and radiographic follow-up is given for a 14-cm-diameter tumor originating from the L4 body with pathologic fracture, unilateral pedicle involvement, and extradural canal extension. Neoadjuvant chemotherapy led to 90% tumor regression and the authors' subsequent en bloc resection attempt. The postoperative chemotherapy was resumed early and postoperative radiotherapy was administered owing to positive microscopic margins. The patient is alive more than 10 years after completing treatment and is without evidence of recurrent disease or secondary malignancy. Late effects of chemotherapy are limited to mild cardiomyopathy controlled with medication. Conclusions The Ewing sarcoma family of tumors of the spinal column accounts for approximately 2% of Ewing sarcoma lesions of the skeletal system; local and systemic relapses are higher than for the extremity sites. Survival is enhanced by en bloc surgical resection in cases where clear margins are obtained, but the prognosis of patients with central (spine and pelvis) sites is typically poor. This case adds to the literature with documentation of high quality of life with long-term, disease-free survival after modern chemotherapy surgical intervention and combined radiotherapy, a finding suggested in recent large Surveillance, Epidemiology, and End Results database studies and smaller case series of this uncommon, high-grade spinal tumor. Neoadjuvant chemotherapy with attempted en bloc resection, and postoperative radiotherapy to the resected tumor bed if there are microscopic positive margins, can still lead to long-term, disease-free survival.