The treatment and prognosis of primary spinal Ewing′s sarcoma family tumor

English Keywords:Spine  Ewing′s sarcoma  PNET  Treatment strategy

English Abstract:

  【Abstract】 Objectives: To investigate the clinical features, treatment and prognosis of patient with primary spinal Ewing′s sarcoma family tumor(ESFT). Methods: 28 patients were retrospectively investigated between May 1997 and June 2010, 18 patients(17 males and 11 females) with Ewing′s sarcoma and 10 patients with peripheral malignant neuroectodermal tumor(PNET). The age of patients ranged from 5 to 45 years with an average of 19 years. The tumor involved cervical vertebrae(4 cases), thoracic vertebrae(5 cases), lumbar vertebrae(6 cases)， thoracic-lumbar segments(1 case) and sacral vertebrae(12 cases). The treatment included surgery, chemotherapy and/or radiotherapy. A five-drug regimen of chemotherapy(vincristine, doxorubicin, cyclophosphamide, ifosfamide and etoposide) was administered to all patients. Surgery was performed on 27 patients, surgery followed by radiotherapy on 22. 7 cases underwent en bloc surgery.　　Results: The follow-up period was from 3 to 12 years with an average of 6.7 years. One patient died of tumor progression during chemotherapy. Twenty patients(20/23, 86.9%) obtained satisfied outcomes with nerurofunction improved for 1-2 Frankel grade. Nine(33.3%) patients developed isolated recurrence and 10(37.1%) patients developed lung metastasis. Only 3 patients developed multiple bone metastasis. The treatment after relapse included administration of second line chemotherapy(13 cases) and combined surgery(6 cases). 11 patients died at final follow-up. The 2-year and 5-year survival rate was 74.3% and 53.3%, respectively. Conclusions: The spinal Ewing′s sarcoma family tumor is a highly malignant tumor with poor prognosis. The local control and chemotherapy is necessary for improving the prognosis of the ESFT. Surgery plays an important role especially for the cases in which the tumor can be resected. En bloc resection is useful to decrease the recurrence. Radiotherapy is applicable for the patient whose tumor can not be resected.