Lumbar malignant peripheral nerve sheath tumor: a rare case in a young patient
Keywords:
malignant peripheral nerve sheath tumors (MPNSTs), soft tissue sarcomas (STS), neurofibromatosis type I (NF-1), prognosisAbstract
Malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue sarcoma that originate from peripheral nerves or from cells associated with the nerve sheath. We report the case of a 30‑year‑old male patient with a history of neurofibromatosis type I (NF-1) and a MPNST located in the lumbar region. The mass was resecated but surgical margins weren’t clear. Recurrence of disease was observed after few months. A close monitoring of subjects with NF-1 is crucial to diagnose MPNST at an earlier stage and allow a complete surgical resection.
References
Grobmyer SR, Reith JD, Shahlaee A, Bush CH, Hochwald SN. Malignant Peripheral Nerve Sheath Tumor: molecular pathogenesis and current management considerations. J Surg Oncol. 2008 Mar 15;97(4):340-9.
Gupta G, Mammis A, Maniker A. Malignant peripheral nerve sheath tumors. Neurosurg Clin N Am. 2008 Oct;19(4):533-43.
Widemann BC. Current status of sporadic and neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors. Curr Oncol Rep. 2009 Jul;11(4):322-8.
Staedtke V, Bai RY, Blakeley JO. Cancer of the Peripheral Nerve in Neurofibromatosis Type 1. Neurotherapeutics. 2017 Apr;14(2):298-306.
American Cancer Society. (2014, December 29). Soft Tissue Sarcoma Early Detection, Diagnosis and Staging. https://www.cancer.org/cancer/soft-tissue-sarcoma/detection-diagnosis-staging.html.
Widemann BC, Italiano A. Biology and Management of Undifferentiated Pleomorphic Sarcoma, Myxofibrosarcoma, and Malignant Peripheral Nerve Sheath Tumors: State of the Art and Perspectives. J Clin Oncol. 2018 Jan 10;36(2):160-167.
McPherson JR, Ong CK, Ng CC, et al. Whole-exome sequencing of breast cancer, malignant peripheral nerve sheath tumor and neurofibroma from a patient with neurofibromatosis type 1. Cancer Med. 2015 Dec;4(12):1871-8.
Farid M, Demicco EG, Garcia R, et al. Malignant peripheral nerve sheath tumors. Oncologist. 2014 Feb;19(2):193-201.
Evans DG, Baser ME, McGaughran J, et al. Malignant peripheral nerve sheath tumours in neurofibromatosis 1. J Med Genet 2002, 39:311–314.
Stucky CC, Johnson KN, Gray RJ, et al. Malignant peripheral nerve sheath tumors (MPNST): the Mayo Clinic experience. Ann Surg Oncol. 2012 Mar;19(3):878-85.
Levy AD, Manning MA, Al-Refaie WB, Miettinen MM. Soft-Tissue Sarcomas of the Abdomen and Pelvis: Radiologic-Pathologic Features, Part 1-Common Sarcomas: From the Radiologic Pathology Archives. Radiographics. 2017 Mar-Apr;37(2):462-483.
Lisson CS, Lisson CG, Beer M, Schmidt SA. Radiological Diagnosis of Soft Tissue Tumors in Adults: MRI Imaging of Selected Entities Delineating Benign and Malignant Tumors. Rofo. 2019 Apr;191(4):323-332.
Singh HP, Grover S, Garg B, Sood N. Histopathological Spectrum of Soft-Tissue Tumors with Immunohistochemistry Correlation and FNCLCC grading: A North Indian Experience. Niger Med J. 2017 Sep-Oct;58(5):149-155.
Bourcier K, Le Cesne A, Tselikas L, et al. Basic Knowledge in Soft Tissue Sarcoma. Cardiovasc Intervent Radiol. 2019 Sep;42(9):1255-1261.
Yang JC, Chang AE, Baker AR et al. Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity. J Clin Oncol, 16((1)): 197-203, 1998.
Baehring JM, Betensky RA, Batchelor TT. Malignant peripheral nerve sheath tumor: the clinical spectrum and outcome of treatment. Neurology. 2003 Sep 9;61(5):696-8.
Baharvahdat H, Ganjeifar B, Roshan NM, Baradaran A. Spinal Intradural Primary Malignant Peripheral Nerve Sheath Tumor with Leptomeningeal Seeding: Case Report and Literature Review. Turk Neurosurg. 2018;28(2):317-322.
Wong WW, Hirose T, Scheithauer BW, Schild SE and Gunderson LL. Malignant peripheral nerve sheath tumor: Analysis of treatment outcome. Int J Radiat Oncol Biol Phys. 42:351–360. 1998.
Kobayashi D, Hirayama M, Komohara Y, et al. Translationally controlled tumor protein is a novel biological target for neurofibromatosis type 1-associated tumors. J Biol Chem. 2014 Sep 19;289(38):26314-26.
Downloads
Published
Issue
Section
License
Copyright (c) 2022 Maria Teresa Paparella, Laura Eusebi, Roberta Mazzucchelli, Giuseppe Guglielmi
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Transfer of Copyright and Permission to Reproduce Parts of Published Papers.
Authors retain the copyright for their published work. No formal permission will be required to reproduce parts (tables or illustrations) of published papers, provided the source is quoted appropriately and reproduction has no commercial intent. Reproductions with commercial intent will require written permission and payment of royalties.
How to Cite
