title: Clinical presentation and long-term outcome of primary spinal peripheral primitive neuroectodermal tumors.
abstract: Primary spinal peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare entities that predominantly occur in children and young adults. Few studies have reported more than three cases. There are no current optimum treatment strategies due to the paucity of data. Here, we present 13 patients (nine females and four males) with primary intraspinal pPNETs who were surgically treated from April 2008 to February 2014. Histopathologic findings revealed the expression of CD99 in all cases. Limb weakness was the most common initial symptom (11/13, 85 %). The tumors were located mainly at the cervical level (6/13, 46 %) and in the epidural space (10/13, 77 %). The radiological diagnosis was neurinoma or meningioma in most cases (10/13, 77 %). Gross total resection was achieved in 77 % (10/13) of patients. During a mean follow-up of 25.5 months, local relapse occurred in 8 (61.5 %) patients and distant metastases occurred in 8 (61.5 %) patients. The overall 1-year survival rate was 77 % (10/13), and the overall 2-year survival rate was 54 % (7/13). The 2-year survival rate was 57.1 % in patients with adjuvant chemotherapy and 50 % in those without chemotherapy. Gross total resection and adjuvant radiotherapy with or without chemotherapy demonstrated a longer survival period (1-year survival rate: 100 %; 2-year survival rate: 86 %). Our data showed that primary spinal pPNETs are extremely rare and aggressive tumors with a poor prognosis. Radical resection is advocated. Gross total resection combined with adjuvant radiation may help to significantly improve patient survival period. Chemotherapy may also help to slightly prolong patient life.
authors.affiliation: Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100050, China.
authors.forename: Xianzeng
authors.initials: X
authors.lastname: Tong
authors.affiliation: Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100050, China.
authors.forename: Xiaofeng
authors.initials: X
authors.lastname: Deng
authors.affiliation: Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100050, China.
authors.forename: Tao
authors.initials: T
authors.lastname: Yang
authors.affiliation: Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100050, China.
authors.forename: Chenlong
authors.initials: C
authors.lastname: Yang
authors.affiliation: Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100050, China.
authors.forename: Liang
authors.initials: L
authors.lastname: Wu
authors.affiliation: Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100050, China.
authors.forename: Jun
authors.initials: J
authors.lastname: Wu
authors.affiliation: Department of Neurosurgery, Beijing Jishuitan Hospital, Fourth Medical College of Peking University, Beijing, 100035, China.
authors.forename: Yuqiang
authors.initials: Y
authors.lastname: Yao
authors.affiliation: Department of Neurosurgery, Beijing Jishuitan Hospital, Fourth Medical College of Peking University, Beijing, 100035, China.
authors.forename: Zhuang
authors.initials: Z
authors.lastname: Fu
authors.affiliation: Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100050, China.
authors.forename: Shuo
authors.initials: S
authors.lastname: Wang
authors.affiliation: Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100050, China. xuhuxi@sina.com.
authors.forename: Yulun
authors.initials: Y
authors.lastname: Xu
mesh_terms: D000071443:12E7 Antigen; D000276:Adjuvants, Immunologic / Q000627:therapeutic use; D000293:Adolescent; D000328:Adult; D015703:Antigens, CD / Q000378:metabolism*; D015815:Cell Adhesion Molecules / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000175:diagnosis / Q000628:therapy*; D013125:Spinal Neoplasms / Q000175:diagnosis / Q000628:therapy*; D015996:Survival Rate; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000071443:12E7 Antigen; D000276:Adjuvants, Immunologic; D015703:Antigens, CD; C061828:CD99 protein, human; D015815:Cell Adhesion Molecules
keywords: Primary peripheral primitive neuroectodermal tumors; Spinal surgery; Spinal tumors