Clinical outcomes of 36 children with peripheral primitive neuroectodermal tumor receiving comprehensive therapy

Abstract

Objective: To investigate the clinical features of children with peripheral primitive neuroectodermal tumor (pPNET) and the efficacy of comprehensive therapy in these patients. Methods: The medical records and the follow-up information of 36 children with pPNET were collected and reviewed. All these patients received surgery, chemotherapy and radiotherapy between January 2002 and January 2013 in Tianjin Medical University Cancer Institute and Hospital. The therapeutic effect, recurrence rate and 1-, 3- and 5-year overall survival rates were analyzed. Results: At the end of the comprehensive therapy, 30 cases achieved complete remission, 2 cases achieved partial remission, 2 cases were in stable disease, one case had progressive disease, and one died of pPNET; the response rate reached 94.4%. By the end of the follow up, 14 (46.7%) cases had tumor recurrence; sixteen cases had a disease-free survival, 4 cases survived with tumor, while 12 cases died of recurrence and 4 cases died of tumor progression. The median survival time was 29 months. The 1-, 3- and 5-year overall survival rate of all 36 cases were 94.4%, 50.0% and 30.6%, respectively. The median survival time of cases who were initialy diagnosed of metastatic pPNET was poorer than that of the cases who were initialy diagnosed of pPNET without metastasis (10 months vs 33 months, P = 0.001). Conclusion: Children with pPNET have very poor prognosis. Those who are initialy diagnosed of pPNET without metastasis have a better clinical outcome, while those who develop the recurrence or metastasis have a poor prognosis. DOI:10.3781/j.issn.1000-7431.2014.33.536