Javier Giner1, Alberto Isla, Ricardo Cubedo, Eva Tejerina. 1. *Department of Neurosurgery, University Hospital La Paz, Madrid, Spain †Department of Oncology, University Hospital Puerta de Hierro, Madrid, Spain ‡Department of Pathology, University Hospital Puerta de Hierro, Madrid, Spain.
Abstract
STUDY DESIGN: Case report. OBJECTIVE: We present a case of isolated primary epidural lumbar Ewing sarcoma and review the current literature on the standard management. We also propose laminoplasty as safe procedure in this patient population that can provide good stabilization in young people. SUMMARY OF BACKGROUND DATA: Primary epidural Ewing's sarcoma is a very rare entity. The best generally accepted treatment option in sarcomas is to achieve a gross total resection with safe margins followed by local radiotherapy and chemotherapy. A total resection with safe margins is a great challenge in neurosurgical patients. METHODS: We present a previously healthy 17-year-old girl who complained of right sciatica with an epidural lumbar mass at L3-L4. She underwent complete resection of the tumor and a laminoplasty, which, in our experience, is a good way to preserve stability. RESULTS: At surgery, an isolated and noninvasive lesion was identified. Histopathological confirmation of Ewing sarcoma was obtained by immunohistochemical study and EWSR1 gene rearrangement detection. Treatment with 6 months of chemotherapy resulted in no further identifiable lesions by PET and MRI imaging at 4 years postsurgery. The laminoplasty has remained stable. CONCLUSION: Primary epidural Ewing sarcoma is extremely rare. The detection of the EWSR1 gene rearrangement can help to diagnose these tumors. The decision on how to treat these patients is difficult and can hardly be based on data from the current literature because of the small number of patients. The laminoplasty procedure can be safely performed in the setting of sarcoma of the epidural space.
STUDY DESIGN: Case report. OBJECTIVE: We present a case of isolated primary epidural lumbar Ewing sarcoma and review the current literature on the standard management. We also propose laminoplasty as safe procedure in this patient population that can provide good stabilization in young people. SUMMARY OF BACKGROUND DATA: Primary epidural Ewing's sarcoma is a very rare entity. The best generally accepted treatment option in sarcomas is to achieve a gross total resection with safe margins followed by local radiotherapy and chemotherapy. A total resection with safe margins is a great challenge in neurosurgical patients. METHODS: We present a previously healthy 17-year-old girl who complained of right sciatica with an epidural lumbar mass at L3-L4. She underwent complete resection of the tumor and a laminoplasty, which, in our experience, is a good way to preserve stability. RESULTS: At surgery, an isolated and noninvasive lesion was identified. Histopathological confirmation of Ewing sarcoma was obtained by immunohistochemical study and EWSR1 gene rearrangement detection. Treatment with 6 months of chemotherapy resulted in no further identifiable lesions by PET and MRI imaging at 4 years postsurgery. The laminoplasty has remained stable. CONCLUSION: Primary epidural Ewing sarcoma is extremely rare. The detection of the EWSR1 gene rearrangement can help to diagnose these tumors. The decision on how to treat these patients is difficult and can hardly be based on data from the current literature because of the small number of patients. The laminoplasty procedure can be safely performed in the setting of sarcoma of the epidural space.