恶性外周神经鞘瘤临床与病理特征

陈再励, 何育城, 黄理金, 张冠华, 夏海军, 刘铁坚

doi:10.13418/j.issn.1001-165x.2019.05.018

中国临床解剖学杂志 >

2019 , Vol. 37 >Issue 5: 573 - 577

DOI: https://doi.org/10.13418/j.issn.1001-165x.2019.05.018

临床研究

恶性外周神经鞘瘤临床与病理特征

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1.南方医科大学第三附属医院康复医学科，广州 510630； 2.深圳市人民医院龙华分院药剂科，广东深圳 518110；
3.南方医科大学第三附属医院神经外科，广州 510630

陈再励（1987-），男，主管治疗师，主要从事神经系统疾病和骨关节疾病康复治疗的临床研究，E-mail：552081468@qq.com

收稿日期: 2019-06-04

网络出版日期: 2019-09-26

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Clinical and pathological features of malignant peripheral nerve sheath tumors

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1.Department of Rehabilitation Medicine, The Third Affiliated Hospital of Southern Medical University, Guangzhou 510630; 2. Department of Pharmacy, Shenzhen People's Hospital Longhua Branch, Shenzhen 518100, Guangdong Province; 3. Department of Neurosurgery, The Third Affiliated Hospital of Southern Medical University, Guangzhou 510630, China

Received date: 2019-06-04

Online published: 2019-09-26

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摘要

目的　探讨恶性外周神经鞘瘤（MPNST）的临床与病理形态特征。方法　收集本院收治的22例MPNST临床资料，应用光学显微镜观察病理形态特点，免疫组化分析其表型，并进行相关文献复习。结果　22例MPNST中，男10例，女12例，年龄15~82岁，中位年龄43岁。头颈部3例，躯干及四肢近端13例，四肢远端5例，全身多发1例。临床表现主要是局部逐渐增大的无痛性肿块，症状与体征与肿块的部位及进展速度有关。19例患者行肿瘤切除手术，术后其中6例患者被嘱须行后续放疗，13例患者行化疗（以多柔比星和异环磷酰胺为主）。免疫组化检测，Vimentin（15/15）、CD99（8/8）、IMP3（10/11）、S-100蛋白（16/19）阳性，Ki-67增殖指数5%~80%。1年病死率45%，中位生存时间25个月。局部复发率55%（12例），远处转移率32%（7例）。末次随访3例无瘤生存。结论　MPNST的临床及病理有其特点，但某些医生对其认识不足。本文总结辨析要点，以期提高该病确诊率，指导治疗和康复。

关键词： 恶性外周神经鞘瘤; 　免疫组化; 　多学科协作诊疗

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陈再励, 何育城, 黄理金, 张冠华, 夏海军, 刘铁坚 . 恶性外周神经鞘瘤临床与病理特征[J]. 中国临床解剖学杂志, 2019 , 37(5) : 573 -577 . DOI: 10.13418/j.issn.1001-165x.2019.05.018

Abstract

Objective　To investigate the clinical and pathological features of malignant peripheral nerve sheath tumors (MPNST)，and increase the understanding of the diseases.　Methods　The clinical data of 22 cases of MPNST hospitalized in our hospital were collected. The pathological features and immunohistochemical phenotypes were observed by light microscopy and immunohistochemistry, and related literature review was carried out.　Results　Of the 22 MPNST patients, 10 were male and 12 female, whose age ranged between 15~82 years old, with a median age of 43 years. There were 3 cases located in the head and neck, 13 cases in trunk and proximal extremities, 5 cases in distal extremities, and 1 case with multiple locations over the body. The clinical manifestations were mainly local painless mass lumps. And the symptoms and signs were related to the location of the mass and the speed of progression. In the treatment, 19 patients underwent tumor resection, among which 6 were recommended for follow-up radiotherapy and 13 underwent chemotherapy (mainly with doxorubicin and ifosfamide). For immunohistochemistry, Vimentin (15/15), CD99 (8/8), IMP3 (10/11), S-100 protein (16/19) positive, and Ki-67 proliferation index ranged between 5%~80%. The 1-year case fatality rate was 45%, and the median survival time was 25 months. Local recurrence rate was 55% (12 cases), and distance metastasis rate was 32% (7 cases). Three cases got tumor free survival to date.　Conclusion　MPNSTs have special clinical and pathological characteristics. However, the clinical understanding of MPNST is insufficient. The study provides the key points of discrimination for this group of cases that can hopefully increase the diagnostic rate, and guide therapy and recovery.

Key words： Malignant peripheral nerve sheath tumors; 　Immunohistochemistry; 　Multiple disciplinary team (MDT)

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