U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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
Analysis of Survival Differences Between Cutaneous and Subcutaneous Malignant Peripheral Nerve Sheath Tumors.
Kromer CM, Yacoub N, Xiong D, Knackstedt T
Dermatol Surg 2023 Apr 1;49(4):322-329. Epub 2023 Feb 9 doi: 10.1097/DSS.0000000000003717. PMID: 36763849
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
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
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
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
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
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
Tumor expression of survivin, p53, cyclin D1, osteopontin and fibronectin in predicting the response to neo-adjuvant chemotherapy in children with advanced malignant peripheral nerve sheath tumor.
Karpinsky G, Krawczyk MA, Izycka-Swieszewska E, Fatyga A, Budka A, Balwierz W, Sobol G, Zalewska-Szewczyk B, Rychlowska-Pruszynska M, Klepacka T, Dembowska-Baginska B, Kazanowska B, Gabrych A, Bien E
J Cancer Res Clin Oncol 2018 Mar;144(3):519-529. Epub 2018 Jan 13 doi: 10.1007/s00432-018-2580-1. PMID: 29332262Free PMC Article

Prognosis

Shaping Our Understanding of Malignant Peripheral Nerve Sheath Tumor: A Bibliometric Analysis of the 100 Most-Cited Articles.
Rechberger JS, Millesi E, Power EA, Wang H, Mardini S, Spinner RJ, Daniels DJ
World Neurosurg 2024 Apr;184:293-302.e11. Epub 2024 Jan 14 doi: 10.1016/j.wneu.2024.01.054. PMID: 38219800
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
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

Supplemental Content

Table of contents

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...