Clinical and pathological features of malignant peripheral nerve sheath tumors

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

Chinese Journal of Clinical Anatomy ›› 2019, Vol. 37 ›› Issue (5) : 573-577. DOI: 10.13418/j.issn.1001-165x.2019.05.018

CHEN Zai-li¹, HE Yu-cheng², HUANG Li-jin³, ZHANG Guan-hua³, XIA Hai-jun³, LIU Tie-jian³

Author information +

History +

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)

Cite this article

EndNote

Ris (Procite)

Bibtex

Download Citations

CHEN Zai-li, HE Yu-cheng, HUANG Li-jin, ZHANG Guan-hua, XIA Hai-jun, LIU Tie-jian. Clinical and pathological features of malignant peripheral nerve sheath tumors[J]. Chinese Journal of Clinical Anatomy. 2019, 37(5): 573-577 https://doi.org/10.13418/j.issn.1001-165x.2019.05.018

References

[1] Doyle LA. Sarcoma classification: an update based on the 2013 World Health Organization Classification of Tumors of Soft Tissue and Bone [J]. Cancer, 2014, 120(12): 1763-1774.

[2] Kim DH, Murovic JA, Tiel RL, et al. A series of 397 peripheral neural sheath tumors: 30-year experience at Louisiana State University Health Sciences Center [J]. J Neurosurg, 2005, 102(2): 246-255.

[3] Perrin RG , Guha A . Malignant peripheral nerve sheath tumors [J]. Neurosurg Clin N AM, 2004, 15(2): 203-216.
[4] Fletcher CD. The evolving classification of soft tissue tumors: an update based on the new WHO classification [J]. Histopathology, 2014, 64(1): 2-11.
[5] 杨学军, 江涛. 解读《世界卫生组织中枢神经系统肿瘤分类（2016年）》[J]. 中国神经精神疾病杂志, 2016, 42(6): 321-329.
[6] LaFemina J, Qin LX, Moraco NH, et al. Oncologic outcomes of sporadic, neurofibromatosis-associated, and radiation-induced malignant peripheral nerve sheath tumors[J]. Ann Surg Oncol, 2013, 20(1): 66-72.
[7] L'Heureux-Lebeau B, Saliba I. Updates on the diagnosis and treatment of intracranial nerve malignant peripheral nerve sheath tumors [J]. OncoTargets Ther, 2013, 6: 459-470.
[8] Bin Samsuddin MF, Bin Omar MA. Malignant peripheral nerve sheath tumor: treat or not treat[J]. Asian J Neurosurg, 2019, 14(1): 283-285.
[9] Stucky CC, Johnson KN, Gray RJ, et al. Malignant peripheral nerve sheath tumors (MPNST): the Mayo clinic experience [J]. Ann Surg Oncol, 2012, 19 (3): 878-885.
[10]Fan Q, Yang J, Wang G. Clinical and molecular prognostic predictors of malignant peripheral nerve sheath tumor [J]. Clin Transl Oncol, 2014, 16(2): 191-199.
[11] Valentina T, Le Cesne A, Ray-Coquard I, et al. Management and prognosis of malignant peripheral nerve sheath tumors: the experience of the French Sarcoma Group (GSF-GETO)[J]. Eur J Cancer, 2016, 56(1): 77-84.
[12] Von Mehren M, Randall RL, Benjamin RS, et al. Soft tissue sarcoma, version 2.2018, NCCN clinical practice guidelines in oncology[J]. J Natl Compr Canc Netw, 2018, 16(5): 536-563.
[13] 袁振南. 140例恶性外周神经鞘瘤的临床病理特征与预后影响因素分析[D]. 北京：北京协和医学院, 2017: 1-39.
[14] Reilly KM, Kim A, Blakely J, et al. Neurofibromatosis type 1-associated MPNST state of science: outlining a research agenda for the future [J]. J Natl Cancer Inst, 2017, 109(8):1-6.
[15] Schaefer IM, Hornick JL. Diagnostic immunohistochemistry for soft tissue and bone tumors: an update [J]. Adv Anat Pathol, 2018, 25(6): 400-412.
[16] Genadry KC, Pietrobono S, Rota R, et al. Soft tissue sarcoma cancer stem cells: an overview [J]. Front Oncol, 2018, 8(1): 475-481.
[17] Ray-Coquard I, Serre D, Reichardt P, et al. Options for treating different soft tissue sarcoma subtypes [J]. Future Oncol, 2018, 14(10s): 25-49.
[18] Bishop AJ, Zagars GK, Torres KE, et al. Malignant peripheral nerve sheath tumors: a single institution's experience using combined surgery and radiation therapy[J]. Am J Clin Oncol, 2018, 41(5): 465-470.