Malignant peripheral nerve sheath tumors (MPNST) are malignant soft tissue neoplasms which account for 2% of all soft tissue sarcomas. These tumors are associated with poor prognosis, propensity to metastasize and high recurrence rates. About half of the MPNST arise from pre-existing neurofibroma and are associated with Neurofibromatosis type I (NF1), 10% are radiotherapy induced others are sporadic. Pathogenesis of MPNST is not fully understood yet. MPNST are common in extremities followed by trunk but are less common in head and neck area. MPNST show limited sensitivity to radiotherapy and chemotherapy and wide surgical resection is mainstay of treatment. We present a series of 3 cases of MPNST of head and neck region originating in patients having NF1.
Thway K, Fisher C. Malignant peripheral nerve sheath tumour: Pathology and genetics. Ann Diagn Pathol [Internet]. 2014;18(2):109–16. Available from: http://dx.doi.org/10.1016/j.anndiagpath.2013.10.007
Valentin T, Le Cesne A, Ray-Coquard I, Italiano A, Decanter G, Bompas E, et al. Management and prognosis of malignant peripheral nerve sheath tumours: The experience of the French Sarcoma Group (GSF-GETO). Eur J Cancer [Internet]. 2016;56:77–84. Available from: http://dx.doi.org/10.1016/j.ejca.2015.12.015
Report S. Neurofibromatosis type 1 and malignancy in childhood. 2016;341–5.
Chen A, Wang T, Xu X. Giant Malignant Peripheral Nerve Sheath Tumor of the Head and Neck: A Case Report and Literature Review. Ear, Nose Throat J. 2020;1–5.
James AW, Shurell E, Singh A, Dry SM, Eilber FC. Malignant Peripheral Nerve Sheath Tumor. Surg Oncol Clin N Am [Internet]. 2016;25(4):789–802. Available from: http://dx.doi.org/10.1016/j.soc.2016.05.009
André R, Vasconcelos T De, Coscarelli PG, Alvarenga RP, Acioly MA. Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1. 2017;(February):366–71.
Stucky CCH, Johnson KN, Gray RJ, Pockaj BA, Ocal IT, Rose PS, et al. Malignant Peripheral Nerve Sheath Tumors (MPNST): The Mayo Clinic experience. Ann Surg Oncol. 2012;19(3):878–85.
Lafemina J, Qin LX, Moraco NH, Antonescu CR, Fields RC, Crago AM, et al. Oncologic outcomes of sporadic, neurofibromatosis-associated, and radiation-induced malignant peripheral nerve sheath tumours. Ann Surg Oncol. 2013;20(1):66–72.
Mullins BT, Hackman T. Malignant Peripheral Nerve Sheath Tumors of the Head and Neck: A Case Series and Literature Review. Case Rep Otolaryngol. 2014;2014:1–6.
Martin E, Coert JH, Flucke UE, Slooff WBM, van de Sande MAJ, van Noesel MM, et al. Neurofibromatosis-associated malignant peripheral nerve sheath tumors in children have a worse prognosis: A nationwide cohort study. Pediatr Blood Cancer. 2020;67(4):1–9.
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