Amanda N Fletcher1, Joanne Abby M Marasigan1, Stephen V Hiatt1, John T Anderson1, Eugenio M Taboada1, Richard M Schwend1. 1. Department of Orthopaedic Surgery, Duke University Medical Center, Durham, North Carolina (Dr. Fletcher); Department of Orthopaedic Surgery, University of Missouri Kansas City School of Medicine, Kansas City, Missouri (Dr. Marasigan); the Department of Orthopaedic Surgery, Kansas City Bone and Joint Clinic, Kansas City, Missouri (Dr. Hiatt); the Department of Orthopaedic Surgery, Kansas City, Missouri (Dr. Anderson and Dr. Schwend), Children's Mercy Hospital, Kansas City; and the Department of Pathology and Laboratory Medicine, Children's Mercy Hospital, Kansas City, Missouri (Dr. Taboada).
Abstract
Primary spinal epidural/extramedullary ewing sarcoma (ES) is a rare extraosseous lesion. Extraosseous ES has a similar demographic as osseous ES, primarily affecting adolescents and young adults and male propensity. Reported 5-year survival is 0% to 37.5% for spinal extraosseous ES. METHODS: Two girls, 19 and 14 months old, presented with progressive lower extremity paraplegia and incontinence. Both had a compressive epidural/extramedullary mass without metastases and underwent decompression with multilevel laminectomy and tumor excision. Primary spinal epidural/extramedullary ES was diagnosed. RESULTS: Case 1 received 34 weeks of chemotherapy and radiation therapy, and case 2 received 14 cycles of chemotherapy and autologous stem cell rescue without radiation therapy. After more than 5- and 8-year follow-up, case 1 and case 2 are walking and disease-free, respectively. CONCLUSION: These cases are the youngest presentation reported for primary spinal epidural/extramedullary ES and suggest that toddlers have a better prognosis for survival than older children and adolescents.
Primary spinal epidural/extramedullary ewing sarcoma (ES) is a rare extraosseous lesion. Extraosseous ES has a similar demographic as osseous ES, primarily affecting adolescents and young adults and male propensity. Reported 5-year survival is 0% to 37.5% for spinal extraosseous ES. METHODS: Two girls, 19 and 14 months old, presented with progressive lower extremity paraplegia and incontinence. Both had a compressive epidural/extramedullary mass without metastases and underwent decompression with multilevel laminectomy and tumor excision. Primary spinal epidural/extramedullary ES was diagnosed. RESULTS: Case 1 received 34 weeks of chemotherapy and radiation therapy, and case 2 received 14 cycles of chemotherapy and autologous stem cell rescue without radiation therapy. After more than 5- and 8-year follow-up, case 1 and case 2 are walking and disease-free, respectively. CONCLUSION: These cases are the youngest presentation reported for primary spinal epidural/extramedullary ES and suggest that toddlers have a better prognosis for survival than older children and adolescents.