Five cases report and literature review of malignant peripheral nerve sheath tumor

Sep 28, 2026

Rationale Malignant Peripheral Nerve Sheath Tumor (MPNST) is an aggressive soft tissue sarcoma with limited treatment options and poor prognosis. Its occurrence is strongly linked to genetic factors, including Neurofibromatosis type I (NF1) in about 50% of cases, with incidence reported between 3% and 10%. Methods Retrospective analysis evaluated age at onset, tumor site and size, metastasis, treatment plan, and prognosis of MPNST patients from January 2018 to January 2022. Results Five cases (20–64 years) were identified, with varied surgical and survival outcomes, and ongoing follow-up. Conclusion Clinical features, management, and outcomes from five cases, including an NF1-associated mutation case, are summarized to support experience in treating this rare tumor.

LanguageEnglish

CategoryHealthcare

FormatPDF

Pages7

Reading Time18 min

AccessFree to download

Abstract

Introduction

Case Study

Pathology of 5 patients

Imaging and pathological findings

Treatment and prognosis review

Conclusion

Wu et al. Discover Oncology (2025) 16:2335 [https://doi.org/10.1007/s12672-025-03949-3](https://doi.org/10.1007/s12672-025-03949-3) Discover Oncology CASE STUDY Open Access Five cases report and literature review of malignant peripheral nerve sheath tumor Xinmeng Wu2†, Yan Zhang 1†, Xiaomei Liu2, Qingqin Zhang2, Qinghui Yang2, Min Zhang 1* and Ping Lu 1* †Xinmeng Wu andYan Zhang are co-first authors. *Correspondence: Min Zhang [zhangmin1982@xxmu.edu.cn](zhangmin1982@xxmu.edu.cn)[ ](zhangmin1982@xxmu.edu.cn)Ping Lu [lupingdoctor@126.com](lupingdoctor@126.com)[ ](lupingdoctor@126.com)1Department of Oncology, The First Affiliated Hospital of Henan Medical University, Xinxiang, Henan, China 2Department of Radiation Oncology, The First Affiliated Hospital of Henan Medical University, Xinxiang, Henan, China Abstract Rationale Malignant Peripheral Nerve Sheath Tumor (MPNST) is an aggressive soft tissue sarcoma with limited therapeutic options and a poor prognosis. Its occurrence is closely related to genetic factors, and it is associated with Neurofibromatosis type I (NF1) in about 50% of cases. The overall incidence ranges from 3% to 10%, and it has been on the rise in recent years. Methods Retrospectively analyze the age of onset, tumor site, tumor size, metastasis, treatment plan, and prognosis of MPNST patients admitted to The First Affiliated Hospital of Henan Medical University from January 2018 to January 2022. Results In the past 5 years, 5 patients with MPNST aged 20–64 years were admitted to our center, including 2 males and 3 females. As of our writing of this manuscript: Of the 4 patients who underwent surgery, Case 1 patient died due to severe complications with overall survival of 11 months, Cases 2 and 4 patients are still alive with overall survival of 68 and 42 months, respectively, Case 5 patient with combined NF1 with overall survival of 18 months, and the patient in Case 3 patient was found to be inoperable due to the evaluation of condition at the time of discovery, but survived after systemic treatment with an overall survival of 45 months. All surviving patients continue to be followed up. Conclusion We described the clinical features, treatment, and clinical outcomes of 5 cases of MPNST, including one case of MPNST with NF1 gene mutation. This provides valuable clinical experience for the treatment of this rare tumor. Keywords Malignant peripheral nerve sheath tumor (MPNST), Retrospective analysis, NF1, Survival, Pathology 1 Introduction Malignant peripheral nerve sheath tumor (MPNST) is a rare malignant tumor that originates from the peripheral nerve or shows different differentiation of the nerve sheath, accounting for about 2–10% of all soft tissue sarcomas and less than 1% of all malignancies [1]. The overall prognosis of MPNST is poor, with 5-year and 10-year survival rates of 34%-52% and 23%-34%, local recurrence rates of 40%-65%, and distant metastasis rates of 40%-68%[2]. NF1 is a negative regulatory factor that regulates cell proliferation and signal transduction, mainly negatively regulating the Ras/MAPK signaling pathway. © The Author(s) 2025. Open Access This article is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License, which permits any non-commercial use, sharing, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if you modified the licensed material. You do not have permission under this licence to share adapted material derived from this article or parts of it. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or ex

FAQs

What clinical and prognostic factors were analyzed in this study of MPNST cases?

The study retrospectively assessed patients’ age at onset, tumor site, tumor size, metastasis, treatment plan, and overall prognosis.

How many MPNST patients were included, and what were the key demographic details?

Five patients aged 20–64 years were included, consisting of 2 males and 3 females.

What did the study conclude about NF1-associated MPNST cases?

One case included an NF1 gene mutation, and the paper notes that NF1-associated groups tend to have the worst prognosis with significantly lower 5-year survival.

Sep 28, 2026

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