Skip to main content Skip to main navigation menu Skip to site footer

Regional recurrence of malignant peripheral nerve sheath tumor in upper extremity – a rare case report

  • I Wayan Sudarsa ,
  • Putu Anda Tusta Adiputra ,


Abstract

Introduction: Malignant Peripheral Nerve Sheath Tumor (MPNST) are aggressive soft tissue sarcoma (STS), originally arising from neural cell components. MPNST had a relatively high local recurrence rate of 22% and a disease survival rate of 45 - 60%. In this study, we would like to report a rare case of recurrent MPNST affecting the upper arm region, with a history of previously resected MPNST at the patient's ipsilateral elbow.

Case Presentation: A 38-years old male patient complained of an enlarging mass in his left anterior axilla Patient had a history of left elbow mass 2 years before and had resected twice. Eight months afterward, a new tumor grew from his left axilla. From the physical examination, the mass was fixated and firm with an oval shape, size 8 x 6 cm. From chest CT scan examination, it appeared a soft tissue mass surrounded with stranding fat had infiltrated his left teres major muscle. The mass was removed successfully and the pathology result revealed a grade 2 MPNST. Post-operative radiotherapy was then scheduled. Among many STS, MPNSTs are notorious for having a high recurrence rate. NF1 mutation, tumors deeper than fascia, large tumors, high-grade tumors, and tumors arising from the trunk, head, and neck are related to poor prognosis in MPNST. Surgeons' inadequate knowledge, unplanned resections, and their dependency on radiotherapy are other iatrogenic factors for recurrences.

Conclusion: MPNST has high recurrent risk, especially in high-grade tumors, large or deep tumors, surgery with positive resection margins, and NF1 mutations. The primary option for treatment is surgery and radiation therapy.

Keywords: Malignant Peripheral Nerve Sheath Tumor Regional Recurrence Resection

References

  1. Antonescu CR, Blay J-Y, Bovee J, Bridge JA, Cunha IW, Tos APD, et al. Soft Tissue Tumours. In: WHO Classification of Tumours Editorial Board, editor. WHO Classification of Tumours Soft Tissue and Bone Tumours. 5th ed. Lyon: International Agency for Research on Cancer; 2020. p. 10.
  2. Singer S, Tap WD, Kirsch DG, Crago AM. Soft Tissue Sarcoma. In: Devita VT, Lawrence TS, Rosenberg SA, editors. Devita, Hellman, and Rosenberg’s Cancer Principles & Practice of Oncology. 11th ed. Philadelphia: Wolters Kluwer Health; 2019. p. 2490.
  3. Contreras CM, Heslin MJ. Soft Tissue Sarcoma. In: Townsend CM, Evers BM, Beauchamp RD, Mattox KL, editors. Sabiston Textbook of Surgery. 21st ed. Missouri: Elsevier; 2022. p. 734.
  4. Marickar YMF, Abraham B. Malignant peripheral nerve sheath tumour – A long story: Case report. International Journal of Surgery Case Reports. 2020;77:618–23.
  5. Brennan MF, Antonescu CR, Alektiar KM, Maki RG. Malignant Peripheral Nerve Sheath Tumor (MPNST) and Triton Tumor. In: Brennan MF, Antonescu CR, Alektiar KM, Maki RG, editors. Management of Soft Tissue Sarcoma. 2nd ed. Gewerbestrasse, Cham: Springer International Publishing; 2016. p. 166.
  6. Stucky CCH, Johnson KN, Gray RJ, Pockaj BA, Ocal IT, Rose PS, et al. Malignant Peripheral Nerve Sheath Tumors (MPNST): The Mayo Clinic experience. Annals of Surgical Oncology. 2012;19(3):878–85.
  7. Zhang R, Zheng B. Overview of Recurrence of Soft Tissue Sarcoma (STS). In: Zhang R, editor. Surgery for Recurrent Soft Tissue Sarcoma. 1st ed. Singapore: Springer Nature Singapore; 2020. p. 3–10.
  8. Sumadi WJ, Citra D, Martadiani ED, Christian NWS. Sclerosing rhabdomyosarcoma of the gingiva mimicking malignant peripheral nerve sheath tumor: A case report. Bali Medical Journal. 2021;10(3):945–9.
  9. Wibowo MD, Fathurochman AF. Aggressiveness tumor: A case report of recurrent ameloblastoma in the mandible. Bali Medical Journal. 2021;10(1):184–8.
  10. Kane JM, Gonzalez RJ, Heslin MJ, McCarter M, McGarry S V, Paz IB, et al. Soft Tissue Sarcoma. National Comprehensive Cancer Network. 2021;2(2):10–34.
  11. Bilgeri A, Klein A, Lindner LH, Nachbichler S, Knösel T, Birkenmaier C, et al. The effect of resection margin on local recurrence and survival in high grade soft tissue sarcoma of the extremities: How far is far enough? Cancers. 2020;12(9):1–13.
  12. Bishop AJ, Zagars GK, Torres KE, Bird JE, Feig BW, Guadagnolo BA. Malignant Peripheral Nerve Sheath Tumors: A Single Institution’s Experience Using Combined Surgery and Radiation Therapy. American Journal of Clinical Oncology: Cancer Clinical Trials. 2018;41(5):465–70.
  13. Albertsmeier M, Rauch A, Roeder F, Hasenhütl S, Pratschke S, Kirschneck M, et al. External Beam Radiation Therapy for Resectable Soft Tissue Sarcoma: A Systematic Review and Meta-Analysis. Annals of Surgical Oncology. 2018;25(3):754–67.
  14. Johan MP, Yurianto H, Firdaus RM, Mubarak AF, Putra LT, Kurniawan T. Evaluation of patient’s daily activities with free non-vascularized fibular head graft (Fnvfhg) as a treatment after resection of proximal humeral tumors. Bali Medical Journal. 2021;10(1):202–7.
  15. Bergamaschi L, Bisogno G, Manzitti C, D’Angelo P, Milano GM, Scagnellato A, et al. Salvage rates and prognostic factors after relapse in children and adolescents with malignant peripheral nerve sheath tumors. Pediatric Blood and Cancer. 2018;65(2):1–8.
  16. 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 Journal of Surgical Oncology. 2020;18(1):1–12.

How to Cite

Sudarsa, I. W., & Putu Anda Tusta Adiputra. (2023). Regional recurrence of malignant peripheral nerve sheath tumor in upper extremity – a rare case report. Bali Medical Journal, 12(1), 1183–1186. https://doi.org/10.15562/bmj.v12i1.4380
ACM ACS APA ABNT Chicago Harvard IEEE MLA Turabian Vancouver
Download Citation
Endnote/Zotero/Mendeley (RIS) BibTeX

HTML
5

Total
1

Share

Search Panel

I Wayan Sudarsa
Google Scholar
Pubmed
BMJ Journal

Putu Anda Tusta Adiputra
Google Scholar
Pubmed
BMJ Journal