Citation Information :
Sarkar S, Sharma R, Yotham RR, Bashir I, Dumeer NK, Bhatnagar A, Sinha P, Sheedh M. A Rare Case of Malignant Peripheral Nerve Sheath Tumor in Neck and Role of VMAT Radiotherapy. Int J Head Neck Surg 2023; 14 (1):9-12.
Introduction: Malignant peripheral nerve sheath tumor (MPNST) is a rare variety of sarcoma. Its presentation is different from usual sarcomas in early presentation, aggressive behavior, high recurrence and poor prognosis. They are usually found in pelvis and extremities. Neck is an extremely rare site. About 50% cases are found in patients of nerofibromatosis type 1 (NF-1). In absence of NF-1 or any evidence of its association with nerve sheath or neurofibroma, visualization of ultrastructure features of Schwann cells in electron microscopy forms the basis of diagnosis, as S100 is weakly present in <50% cases.
Case summary: Here, we are reporting a rare case of MPNST in right side of neck in a in a 36-year-old male with NF-1.
Discussion: In literature, role of radiotherapy in MPNST has been described in general with other sarcomas. Our aim is to describe clinicopathological features and treatment with volumetric modulated arc technique (VMAT) radiotherapy in MPNST of neck.
Anghileri M,Miceli R,Fiore M, et al. Malignant peripheral nerve sheath tumors: prognostic factors and survival in a series of patients treated at a single institution. Cancer 2006;107(5):1065–1074. DOI: 10.1002/cncr.22098
Weiss SW, Goldblum JR. Enzinger and Weiss's soft tissue tumors. 5th edn St. Louis: Mosby, 2008:903–41.
Ellis GL, Abrams AM, Melrose RJ. Intraosseous benign neural sheath neoplasms of the jaws. Report of seven new cases and review of the literature. Oral Surg Oral Med Oral Pathol 1977;44(5):731–743. DOI: 10.1016/0030-4220(77)90383-8
Sham ME, Ghorpande, Shetty A, et al. Malignant peripheral nerve cell tumor. J Maxillofac Oral Surg 2010;9(1):68–71. DOI: 10.1007/s12663-010-0019-6
Che Z, Nam W, Park WS, et al. Intraosseous nerve sheath tumors in the jaws. Yonsei Med J 2006;47(2):264–270. DOI: 10.3349/ymj.2006.47.2.264
Ashraf MJ,Azarpira N,Khademi B, et al. Malignant peripheral nerve sheath tumor of the tongue. Indian J Pathol Microbiol 2010;53(4):906–907. DOI: 10.4103/0377-4929.72073
Gogate BP,Anand M,Deshmukh SD, et al. Malignant peripheral nerve sheath tumor of facial nerve: presenting as parotid mass. J Oral Maxillofac Pathol 2013;17(1): 129–131. DOI: 10.4103/0973-029X.110708
Katz D,Lazar A, Lev D. Malignant peripheral nerve sheath tumour (MPNST): the clinical implications of cellular signalling pathways. Expert Rev Mol Med 2009;11:e30. DOI: 10.1017/S1462399409001227
Common Terminology Criteria for Adverse Events (CTCAE). Common Terminology Criteria for Adverse Events (CTCAE) v.4.0 [published 2009, updated June 14, 2010]. Available from: http://evs.nci.nih.gov/ftp1/CTCAE/CTCAE_4.03_2010-06-14_QuickReference_5x7.pdf.
Ng VY, Scharschmidt TJ, Mayerson JL, et al. Incidence and survival in sarcoma in the United States: a focus on musculoskeletal lesions. Anticancer Res 2013;33(6):2597–2604. PMID: 23749914.
Widemann BC. Current status of sporadic and neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors. Curr Oncol Rep 2009;11(4):322–328. DOI: 10.1007/s11912-009-0045-z
Zou C, Smith KD, Liu J, et al. Clinical, pathological, and molecular variables predictive of malignant peripheral nerve sheath tumor outcome. Ann Surg 2009;249(6):1014–1022. DOI: 10.1097/SLA.0b013e3181a77e9a
Gregorian C, Nakashima J, Dry SM, et al. PTEN dosage is essential for neurofibroma development and malignant transformation. Proc Natl Acad Sci USA 2009;106(46):19479–19484. DOI: 10.1073/pnas.0910398106
Bradtmöller M, Hartmann C, Zietsch J, et al. Impaired Pten expression in human malignant peripheral nerve sheath tumours. PLoS One 2012;7(11):e47595. DOI: 10.1371/journal.pone.0047595
Berghmans S, Murphey RD, Wienholds E, et al. tp53 mutant zebrafish develop malignant peripheral nerve sheath tumors. Proc Natl Acad Sci USA 2005;102(2):407–412. DOI: 10.1073/pnas.0406252102
LaFemina J, Qin LX, Moraco NH, et al. Oncologic outcomes of sporadic, neurofibromatosis-associated, and radiation-induced malignant peripheral nerve sheath tumors. Ann Surg Oncol 2013;20(1):66–72. DOI: 10.1245/s10434-012-2573-2
Fletcher CDM, Bridge JA, Hogendoorn PCW. et al. WHO Classification of Tumours of Soft Tissue and Bone. Lyon, France: IARC; 2013.
Torres KE, Zhu QS, Bill K, et al. Activated MET is a molecular prognosticator and potential therapeutic target for malignant peripheral nerve sheath tumors. Clin Cancer Res 2011;17(12):3943–3955. DOI: 10.1158/1078-0432.CCR-11-0193
Stucky CC, Johnson KN, Gray RJ, et al. Malignant peripheral nerve sheath tumors (MPNST): the Mayo Clinic experience. Ann Surg Oncol 2012;19(3):878–885. DOI: 10.1245/s10434-011-1978-7
Endo M, Yamamoto H, Setsu N, et al. Prognostic significance of AKT/mTOR and MAPK pathways and antitumor effect of mTOR inhibitor in NF1-related and sporadic malignant peripheral nerve sheath tumors. Clin Cancer Res 2013;19(2):450–461. DOI: 10.1158/1078-0432.CCR-12-1067
Dunn GP, Spiliopoulos K, Plotkin SR, et al. Role of resection of malignant peripheral nerve sheath tumors in patients with neurofibromatosis type 1. J Neurosurg 2013;118(1):142–148. DOI: 10.3171/2012.9.JNS101610
Kroep JR, Ouali M, Gelderblom H, et al. First-line chemotherapy for malignant peripheral nerve sheath tumor (MPNST) versus other histological soft tissue sarcoma subtypes and as a prognostic factor for MPNST: an EORTC soft tissue and bone sarcoma group study. Ann Oncol 2011;22(1):207–214. DOI: 10.1093/annonc/mdq338
Sloan L, Terezakis SA, Blakeley JO, et al. Long-term outcomes of radiation therapy (RT) in the management of malignant peripheral nerve sheath tumors (MPNST) in patients with neurofibromatosis type 1 (NF1). Int J Radiat Oncol 2018;102(3):E474–E475. DOI: 10.1016/j.ijrobp.2018.07.1357