title: Long-Term, Disease-Free Survival of a Patient With a Primitive Neuroectodermal/Ewing Sarcoma in the Mobile Spine With Extracompartmental Extension.
abstract: METHODS
  Case report.
  
  
  BACKGROUND
  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.
  
  
  METHODS
  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.
authors.affiliation: Department of Pediatric Orthopaedic Surgery, Children's Hospital of New Orleans and Louisiana State University School of Medicine, 200 Henry Clay Avenue, New Orleans, LA 70118, USA. Electronic address: edwardsouthern@doctorsouthern.com.
authors.forename: Edward P
authors.initials: EP
authors.lastname: Southern
authors.affiliation: 1414 West Fair Avenue, Suite 190, Advanced Orthopaedics, Marquette General Hospital, Marquette, MI 49855, USA; Physician Assistant Program, Central Michigan University, 1280 E. Campus DriveHealth Professions Building 1222, Mount Pleasant, MI 48859, USA.
authors.forename: Sebastian
authors.initials: S
authors.lastname: Hohl
authors.affiliation: Pediatric Hematology and Oncology Service, Long Beach Memorial Miller Children's Hospital, 2801 Atlantic Avenue, Long Beach, CA 90806, USA; 9173 Tower Pines Cove, Oltewah, TN 37363-9347, USA.
authors.forename: Iqbal
authors.initials: I
authors.lastname: Singh
authors.affiliation: Pediatric Hematology and Oncology Service, Long Beach Memorial Miller Children's Hospital, 2801 Atlantic Avenue, Long Beach, CA 90806, USA.
authors.forename: Jerry Z
authors.initials: JZ
authors.lastname: Finklestein
keywords: En bloc resection; Ewing sarcoma family of tumors; Primitive neuroectodermal tumors; Spine; Survival