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Advanced Malignant Peripheral Nerve Sheath Tumor

MedGen UID:
1712867
•Concept ID:
C5239068
•
Neoplastic Process

Definition

A malignant peripheral nerve sheath tumor that has spread extensively to other anatomic sites or is no longer responding to treatment. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdvanced Malignant Peripheral Nerve Sheath Tumor

Professional guidelines

PubMed

Selinexor, a First in Class, Nuclear Export Inhibitor for the Treatment of Advanced Malignant Peripheral Nerve Sheath Tumor.
Al-Ezzi E, Gounder M, Watson G, Mazzocca A, D'Angelo SP, Bravetti J, Wang H, Abdul Razak A, Vincenzi B
Oncologist 2021 Apr;26(4):e710-e714. Epub 2021 Feb 17 doi: 10.1002/onco.13692. PMID: 33512749Free PMC Article

Recent clinical studies

Etiology

Management of Central and Peripheral Nervous System Tumors in Patients with Neurofibromatosis.
Brown R
Curr Oncol Rep 2023 Dec;25(12):1409-1417. Epub 2023 Oct 31 doi: 10.1007/s11912-023-01451-z. PMID: 37906356
From Genes to -Omics: The Evolving Molecular Landscape of Malignant Peripheral Nerve Sheath Tumor.
Lemberg KM, Wang J, Pratilas CA
Genes (Basel) 2020 Jun 24;11(6) doi: 10.3390/genes11060691. PMID: 32599735Free PMC Article
Peripheral tumor and tumor-like neurogenic lesions.
Abreu E, Aubert S, Wavreille G, Gheno R, Canella C, Cotten A
Eur J Radiol 2013 Jan;82(1):38-50. Epub 2011 May 10 doi: 10.1016/j.ejrad.2011.04.036. PMID: 21561733
Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems.
Rodriguez FJ, Folpe AL, Giannini C, Perry A
Acta Neuropathol 2012 Mar;123(3):295-319. Epub 2012 Feb 12 doi: 10.1007/s00401-012-0954-z. PMID: 22327363Free PMC Article
Peripheral and cranial nerve sheath tumors.
Mrugala MM, Batchelor TT, Plotkin SR
Curr Opin Neurol 2005 Oct;18(5):604-10. doi: 10.1097/01.wco.0000179507.51647.02. PMID: 16155448

Diagnosis

Management of Central and Peripheral Nervous System Tumors in Patients with Neurofibromatosis.
Brown R
Curr Oncol Rep 2023 Dec;25(12):1409-1417. Epub 2023 Oct 31 doi: 10.1007/s11912-023-01451-z. PMID: 37906356
Ultrasound and MR imaging of peripheral nerve tumors: the state of the art.
Lefebvre G, Le Corroller T
Skeletal Radiol 2023 Mar;52(3):405-419. Epub 2022 Jun 17 doi: 10.1007/s00256-022-04087-5. PMID: 35713690
Systemic Options for Malignant Peripheral Nerve Sheath Tumors.
Hassan A, Pestana RC, Parkes A
Curr Treat Options Oncol 2021 Feb 27;22(4):33. doi: 10.1007/s11864-021-00830-7. PMID: 33641042
Neurofibromatosis type 1 (NF1): diagnosis and management.
Ferner RE, Gutmann DH
Handb Clin Neurol 2013;115:939-55. doi: 10.1016/B978-0-444-52902-2.00053-9. PMID: 23931823
Plexiform neurofibromas.
Korf BR
Am J Med Genet 1999 Mar 26;89(1):31-7. doi: 10.1002/(sici)1096-8628(19990326)89:1<31::aid-ajmg7>3.0.co;2-w. PMID: 10469434

Therapy

Management of Central and Peripheral Nervous System Tumors in Patients with Neurofibromatosis.
Brown R
Curr Oncol Rep 2023 Dec;25(12):1409-1417. Epub 2023 Oct 31 doi: 10.1007/s11912-023-01451-z. PMID: 37906356
A High-Throughput Screening Platform Identifies Novel Combination Treatments for Malignant Peripheral Nerve Sheath Tumors.
Fernández-Rodríguez J, Creus-Bachiller E, Zhang X, Martínez-Iniesta M, Ortega-Bertran S, Guha R, Thomas CJ, Wallace MR, Romagosa C, Salazar-Huayna L, Reilly KM, Blakely JO, Serra-Musach J, Pujana MA, Serra E, Villanueva A, Ferrer M, Lázaro C
Mol Cancer Ther 2022 Jul 5;21(7):1246-1258. doi: 10.1158/1535-7163.MCT-21-0947. PMID: 35511749Free PMC Article
Neurofibromatosis in the Era of Precision Medicine: Development of MEK Inhibitors and Recent Successes with Selumetinib.
Galvin R, Watson AL, Largaespada DA, Ratner N, Osum S, Moertel CL
Curr Oncol Rep 2021 Mar 15;23(4):45. doi: 10.1007/s11912-021-01032-y. PMID: 33721151Free PMC Article
Functional imaging of RAS pathway targeting in malignant peripheral nerve sheath tumor cells and xenografts.
Butler E, Schwettmann B, Geboers S, Hao G, Kim J, Nham K, Sun X, Laetsch TW, Xu L, Williams NS, Skapek SX
Pediatr Blood Cancer 2020 Dec;67(12):e28639. Epub 2020 Sep 25 doi: 10.1002/pbc.28639. PMID: 32975370
Diffusion tensor tractography for the surgical management of peripheral nerve sheath tumors.
Schmidt M, Kasprian G, Amann G, Duscher D, Aszmann OC
Neurosurg Focus 2015 Sep;39(3):E17. doi: 10.3171/2015.6.FOCUS15228. PMID: 26323819

Prognosis

Management of Central and Peripheral Nervous System Tumors in Patients with Neurofibromatosis.
Brown R
Curr Oncol Rep 2023 Dec;25(12):1409-1417. Epub 2023 Oct 31 doi: 10.1007/s11912-023-01451-z. PMID: 37906356
Systemic Options for Malignant Peripheral Nerve Sheath Tumors.
Hassan A, Pestana RC, Parkes A
Curr Treat Options Oncol 2021 Feb 27;22(4):33. doi: 10.1007/s11864-021-00830-7. PMID: 33641042
Peripheral Nerve Sheath Tumors of Head and Neck: Imaging-Based Review of World Health Organization Classification.
Abdel Razek AAK, Gamaleldin OA, Elsebaie NA
J Comput Assist Tomogr 2020 Nov/Dec;44(6):928-940. doi: 10.1097/RCT.0000000000001109. PMID: 33196600
PET Imaging of Peripheral Nerve Tumors.
Assadi M, Velez E, Najafi MH, Matcuk G, Gholamrezanezhad A
PET Clin 2019 Jan;14(1):81-89. Epub 2018 Oct 24 doi: 10.1016/j.cpet.2018.08.013. PMID: 30420224
Malignant peripheral nerve sheath tumors.
Farid M, Demicco EG, Garcia R, Ahn L, Merola PR, Cioffi A, Maki RG
Oncologist 2014 Feb;19(2):193-201. Epub 2014 Jan 27 doi: 10.1634/theoncologist.2013-0328. PMID: 24470531Free PMC Article

Clinical prediction guides

CDK4/6 inhibition enhances SHP2 inhibitor efficacy and is dependent upon RB function in malignant peripheral nerve sheath tumors.
Wang J, Calizo A, Zhang L, Pino JC, Lyu Y, Pollard K, Zhang X, Larsson AT, Conniff E, Llosa NJ, Wood DK, Largaespada DA, Moody SE, Gosline SJ, Hirbe AC, Pratilas CA
Sci Adv 2023 Nov 24;9(47):eadg8876. doi: 10.1126/sciadv.adg8876. PMID: 38000020Free PMC Article
SWI/SNF complex-deficient soft tissue neoplasms: An update.
Schaefer IM, Hornick JL
Semin Diagn Pathol 2021 May;38(3):222-231. Epub 2020 Jun 5 doi: 10.1053/j.semdp.2020.05.005. PMID: 32646614Free PMC Article
Peripheral Nerve Sheath Tumors of Head and Neck: Imaging-Based Review of World Health Organization Classification.
Abdel Razek AAK, Gamaleldin OA, Elsebaie NA
J Comput Assist Tomogr 2020 Nov/Dec;44(6):928-940. doi: 10.1097/RCT.0000000000001109. PMID: 33196600
Peripheral tumor and tumor-like neurogenic lesions.
Abreu E, Aubert S, Wavreille G, Gheno R, Canella C, Cotten A
Eur J Radiol 2013 Jan;82(1):38-50. Epub 2011 May 10 doi: 10.1016/j.ejrad.2011.04.036. PMID: 21561733
Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems.
Rodriguez FJ, Folpe AL, Giannini C, Perry A
Acta Neuropathol 2012 Mar;123(3):295-319. Epub 2012 Feb 12 doi: 10.1007/s00401-012-0954-z. PMID: 22327363Free PMC Article

Recent systematic reviews

Impact of CT and MRI in the diagnostic workup of malignant triton tumour-a monocentric analysis and review of the literature.
Drews MA, Milosevic A, Hamacher R, Grüneisen JS, Haubold J, Opitz MK, Bauer S, Umutlu L, Forsting M, Schaarschmidt BM
Br J Radiol 2024 Feb 2;97(1154):430-438. doi: 10.1093/bjr/tqad035. PMID: 38308031

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