Clinical characteristics and prognosis of primary intraspinal peripheral primitive neuroectodermal tumors

Abstract

Objective To investigate the clinical features, treatment methods and prognosis of primary intraspinal peripheral primitive neuroectodermal tumors (pPNETs). Methods A retrospective study was performed on 26 patients with primary spinal pPNETs who were surgically treated at Department of Neurosurgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology from January 2005 to February 2018. The dorsal standard midline approach was performed in 25 patients and the remaining 1 case underwent biopsy. Two weeks after the first operation, patients were recommended to undergo chemotherapy and/or radiotherapy depending on the age of the patient and the Karnofsky Performance Status (KPS) scores. Clinical follow-up was performed in all patients. The contents of follow-up included tumor recurrence and metastasis, survival time and cause of death. Results Of the 25 patients who underwent surgical treatment, gross total resection was achieved in 14 cases, subtotal resection in 8 and partial resection in 3. Postoperative pathological examination showed that 26 cases were primary intraspinal pPNETs. The follow-up period ranged from 2 months to 86 months. The average follow-up period was 17.0±5.1 months. During the follow-up, 5 patients survived and 16 died. The cause of death was tumor recurrence and/or distant metastasis. The average survival time of 21 patients was 27.3±12.9 months. The overall 1-year, 2-year and 5-year survival rates were 55.3%(11/21), 30.6%(6/21) and 15.3%(3/21), respectively. Of the 13 patients who underwent adjuvant radiotherapy and chemotherapy, the 1-year, 2-year and 5-year survival ratios were 9/13, 7/13 and 3/13, respectively. For 2 patients with adjuvant chemotherapy alone, the 1-year survival was one. The 1-year survival ratio was 1/6 in patients treated without adjuvant therapy. Conclusions Primary intraspinal pPNETs have an acute onset, short duration, rapid progression and poor prognosis. Gross total resection of the tumor is the preferred method of treatment. Gross total resection combined with adjuvant radiotherapy and chemotherapy could improve the quality of life and help prolong survival time. Key words: Primitive neuroectodermal tumors; Treatment outcome; Clinical features; Prognosis