Clinical manifestation and pathological characteristics of intraspinal extramedullary peripheral primitive neuroectodermal tumour
- VernacularTitle:椎管内髓外原始神经外胚层肿瘤临床病理特点分析
- Author:
Xuesong CHEN
;
Junlin YI
;
Li GAO
;
Guozhen XU
- Publication Type:Journal Article
- Keywords:
Primitive neuroectodermal neoplasms,central;
Pathological characteristics;
Treatment method
- From:
Chinese Journal of Radiation Oncology
2008;17(3):216-218
- CountryChina
- Language:Chinese
-
Abstract:
Objective To observe the clinical manifestation and pathological immunohistochemical features of intraspinal extramedullary peripheral primitive neuroeetodermal tumour (PNET). Methods From Apr. 1996 to Aug. 2007, totally 5 patients with intraspinal extramedullary peripheral PNET were treated, including 3 male and 2 female, aging from 11 to 48 years. The primary site was cervical vertebral in 2 (C5-C7 and C5-T1 ) and thoracic vertebral in 4( T4-T6, T6-T8 ,T4-T5 and C5-T1 ). The clinical manifestation was paresis of limbs in 5 and incontinence in 4. All the patients were pathologically diagnosed Results All of the three patients who had CD99 staining expressed CD99 ( + ). All the 5 patients received surgery followed by radiotherapy and 4 received postoperative chemotherapy. The most common treatment failure was local recurrence. The overall survival time of the 5 patients was 4.8year,2.5year,2. 1ycar, 1.6year and 0.8 year respectively. Conclusions Intraspinal extramedullary peripheral PNET is highly malignant tumor.Patients have very poor survival and local control New treatment protocol should be studied to improve the local disease control and increase remission rate. To distinguish central PNET from peripheral PNET has crucial significance since different entities have different treatment protocol and prognosis.
