DOI: https://doi.org/10.14710/dmj.v15i3.53468

RECURRENT MALIGNANT PERIPHERAL NERVE SHEATH TUMOR OF THE LEFT FEMUR POST MULTIMODAL THERAPY IN 61 YEARS OLD MALE: A CASE REPORT

*Diaz Alifarizki Zuvarcan orcid  -  Faculty of Medicine, Sebelas Maret University, Indonesia
Christopher Bryan  -  Clinical Clerkship, Faculty of Medicine, Sebelas Maret University, Surakarta, Indonesia, Indonesia
Sri Marwanta  -  Division of Hematology and Oncology, Department of Internal Medicine, Dr. Moewardi Regional Hospital, Central Java, Indonesia, Indonesia
Open Access Copyright 2026 Diaz Alifarizki Zuvarcan
Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

Citation Format:
Abstract

Background: Malignant peripheral nerve sheath tumors (MPNST) are rare, aggressive soft tissue sarcomas with a high rate of recurrence and metastasis. Sporadic MPNSTs, especially in older adults without neurofibromatosis type 1, present additional diagnostic and therapeutic challenges. Case Presentation: We report a 61-year-old male with a recurrent sporadic MPNST of the left femur. Over six years, he underwent three surgical excisions, radiotherapy, and two lines of chemotherapy. Despite initial partial response, the disease progressed with pulmonary metastases. Histopathology revealed spindle cell sarcoma with lymphovascular and skeletal muscle invasion. Conclusion: This case highlights the difficulty of achieving durable control in recurrent MPNST despite multimodal therapy. It underscores the importance of vigilant follow-up, individualized treatment plans, and supportive care in advanced disease settings.

Fulltext View|Download Email colleagues
Keywords: case report; malignant peripheral nerve sheath tumor; multimodal therapy; recurrent tumor; soft tissue sarcoma

Article Metrics:

Article Info
Section: Case Report
Language : EN
  1. Knight SWE, Knight TE, Santiago T, Murphy AJ, Abdelhafeez AH. Malignant Peripheral Nerve Sheath Tumors—A Comprehensive Review of Pathophysiology, Diagnosis, and Multidisciplinary Management. Children 2022;9. https://doi.org/10.3390/children9010038
  2. Huang J, Shi H, Ge L, Zhu B, Liu J, Wang X, et al. Epidemiologic and survival analysis of malignant peripheral nerve sheath tumors: a retrospective cohort study. Int J Surg 2024;110:8210–4. https://doi.org/10.1097/JS9.0000000000001756
  3. Cai Z, Tang X, Liang H, Yang R, Yan T, Guo W. Prognosis and risk factors for malignant peripheral nerve sheath tumor: A systematic review and meta-analysis. World J Surg Oncol 2020;18. https://doi.org/10.1186/s12957-020-02036-x
  4. Yuan Z, Xu L, Zhao Z, Xu S, Zhang X, Liu T, et al. Clinicopathological features and prognosis of malignant peripheral nerve sheath tumor: a retrospective study of 159 cases from 1999 to 2016. Oncotarget 2017;8:104785–95
  5. Gutmann DH, Ferner RE, Listernick RH, Korf BR, Wolters PL, Johnson KJ. Neurofibromatosis type 1. Nat Rev Dis Primers 2017;3. https://doi.org/10.1038/nrdp.2017.4
  6. Watson KL, Al Sannaa GA, Kivlin CM, Ingram DR, Landers SM, Roland CL, et al. Patterns of recurrence and survival in sporadic, neurofibromatosis Type 1-associated, and radiation-associated malignant peripheral nerve sheath tumors. J Neurosurg 2017;126:319–29. https://doi.org/10.3171/2015.12.JNS152443
  7. Al-Mistarehi A-H, Zaitoun KJ, Khalifeh J, Saint-Germain MA, Horowitz MA, Ghaith AK, et al. An Assessment of Surgical Outcomes in Malignant Peripheral Nerve Sheath Tumors: A Systematic Review and Meta-Analysis of Surgical Interventions. Cancers (Basel) 2025;17:1997. https://doi.org/10.3390/cancers17121997
  8. Pellerino A, Verdijk RM, Nichelli L, Andratschke NH, Idbaih A, Goldbrunner R. Diagnosis and Treatment of Peripheral and Cranial Nerve Tumors with Expert Recommendations: An EUropean Network for RAre CANcers (EURACAN) Initiative. Cancers (Basel) 2023;15. https://doi.org/10.3390/cancers15071930
  9. Sbaraglia M, Bellan E, Dei Tos AP. The 2020 WHO Classification of Soft Tissue Tumours: News and perspectives. Pathologica 2021;113:70–84. https://doi.org/10.32074/1591-951X-213
  10. Shurell-Linehan E, Dipardo BJ, Elliott IA, Graham DS, Eckardt MA, Dry SM, et al. Pathologic response to neoadjuvant therapy is associated with improved long-Term survival in high-risk primary localized malignant peripheral nerve sheath tumors. American Journal of Clinical Oncology: Cancer Clinical Trials 2019;42:426–31. https://doi.org/10.1097/COC.0000000000000536
  11. Zhang L, Lemberg KM, Calizo A, Varadhan R, Siegel AH, Meyer CF, et al. Analysis of treatment sequence and outcomes in patients with relapsed malignant peripheral nerve sheath tumors. Neurooncol Adv 2023;5. https://doi.org/10.1093/noajnl/vdad156
  12. Reilly KM, Kim AR, Blakely J, Ferner RE, Gutmann DH, Legius E, et al. Neurofibromatosis Type 1–Associated MPNST State of the Science: Outlining a Research Agenda for the Future. J Natl Cancer Inst 2017;109. https://doi.org/10.1093/jnci/djx124
  13. Hirbe AC, Dehner CA, Dombi E, Eulo V, Gross AM, Sundby T, et al. Contemporary Approach to Neurofibromatosis Type 1–Associated Malignant Peripheral Nerve Sheath Tumors. American Society of Clinical Oncology Educational Book 2024;44. https://doi.org/10.1200/edbk_432242
  14. Miao R, Wang H, Jacobson A, Lietz AP, Choy E, Raskin KA, et al. Radiation-induced and neurofibromatosis-associated malignant peripheral nerve sheath tumors (MPNST)have worse outcomes than sporadic MPNST. Radiotherapy and Oncology 2019;137:61–70. https://doi.org/10.1016/j.radonc.2019.03.015
  15. Yao C, Zhou H, Dong Y, Alhaskawi A, Hasan Abdullah Ezzi S, Wang Z, et al. Malignant Peripheral Nerve Sheath Tumors: Latest Concepts in Disease Pathogenesis and Clinical Management. Cancers (Basel) 2023;15. https://doi.org/10.3390/cancers15041077
  16. Zhang X, Murray B, Mo G, Shern JF. The role of polycomb repressive complex in malignant peripheral nerve sheath tumor. Genes (Basel) 2020;11. https://doi.org/10.3390/genes11030287
  17. Cortes-Ciriano I, Steele CD, Piculell K, Al-Ibraheemi A, Eulo V, Bui MM, et al. Genomic Patterns of Malignant Peripheral Nerve Sheath Tumor (MPNST) Evolution Correlate with Clinical Outcome and Are Detectable in Cell-Free DNA. Cancer Discov 2023;13:654–71. https://doi.org/10.1158/2159-8290.CD-22-0786
  18. Hornick JL, Nielsen GP. Beyond “Triton” Malignant Peripheral Nerve Sheath Tumors With Complete Heterologous Rhabdomyoblastic Differentiation Mimicking Spindle Cell Rhabdomyosarcoma. Am J Surg Pathol 2019
  19. Ito Y, Kohashi K, Endo M, Yoshimoto M, Ishihara S, Toda Y, et al. Clinicopathological and prognostic significance of H3K27 methylation status in malignant peripheral nerve sheath tumor: correlation with skeletal muscle differentiation. Virchows Archiv 2021;479:1233–44. https://doi.org/10.1007/s00428-021-03189-0
  20. Seres R, Hameed H, McCabe MG, Russell D, Lee ATJ. The Multimodality Management of Malignant Peripheral Nerve Sheath Tumours. Cancers (Basel) 2024;16. https://doi.org/10.3390/cancers16193266
  21. Magallón-Lorenz M, Fernández-Rodríguez J, Terribas E, Creus-Batchiller E, Romagosa C, Estival A, et al. Chromosomal translocations inactivating CDKN2A support a single path for malignant peripheral nerve sheath tumor initiation. Hum Genet 2021;140:1241–52. https://doi.org/10.1007/s00439-021-02296-x
  22. Du P, Zhu J, Zhang ZD, He C, Ye MY, Liu YX, et al. Recurrent epithelioid malignant peripheral nerve sheath tumor with neurofibromatosis type 1: A case report and literature review. Oncol Lett 2019;18:3072–80. https://doi.org/10.3892/ol.2019.10676
  23. Khan N, Hashmi I, Atallah L, Shaaban H, Guron G, Fedida A. A rare case report of malignant peripheral nerve sheath tumor involving both the small bowel and large bowel. Ann Afr Med 2021;20:228–31. https://doi.org/10.4103/aam.aam_10_20
  24. Farzin M, Jafari F, Yousefi A. Two-Year Local and Distant Control of Malignant Peripheral Nerve Sheath Tumor (MPNST) in NF1 Patients after Multiple Recurrences: A Case Report. Case Reports in Clinical Practice 2024;9:57–9. https://doi.org/10.1002/cncr.22098
  25. Moldovan H, Ciomaga I, Nechifor E, Tigănașu R, Badea A, Dobra I, et al. A Rare Case of Left Ventricular Malignant Peripheral Nerve Sheath Tumour—Case Report and Review of the Literature. Medicina (Lithuania) 2022;58. https://doi.org/10.3390/medicina58101404
  26. Mencia MM, Moonsie R. Successful Outcome Following Limb Conservation Surgery for a Recurrent Malignant Peripheral Nerve Sheath Tumour: A Case Report. J Orthop Case Rep 2023;13:53–7. https://doi.org/10.13107/jocr.2023.v13.i12.4076
  27. Rodon J, Rodriguez E, Maitland ML, Tsai FYC, Socinski MA, Berlin JD, et al. A phase I study to evaluate the safety, pharmacokinetics, and pharmacodynamics of PF-06939999 (PRMT5 inhibitor) in patients with selected advanced or metastatic tumors with high incidence of splicing factor gene mutations. ESMO Open 2024;9. https://doi.org/10.1016/j.esmoop.2024.102961
  28. Chen Q, Cui H, Zheng K, Xu M, Yu X. Denosumab combined with chemotherapy followed by anlotinib in the treatment of multiple metastases of malignant peripheral nerve sheath tumor: a case report and literature review. Front Oncol 2024;14. https://doi.org/10.3389/fonc.2024.1399021
  29. Widemann BC, Italiano A. Biology and Management of Undifferentiated Pleomorphic Sarcoma, Myxofibrosarcoma, and Malignant Peripheral Nerve Sheath Tumors: State of the Art and Perspectives. Journal of Clinical Oncology 2017;36:160–7. https://doi.org/10.1200/JCO.2017

Last update:

No citation recorded.

Last update:

No citation recorded.