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Malignant peripheral nerve sheath tumor

MedGen UID:
155614
•Concept ID:
C0751690
•
Neoplastic Process
Synonyms: Malignant Neurilemmoma; Malignant Neurilemmomas; Malignant Neurilemoma; Malignant Neurilemomas; Malignant Peripheral Nerve Sheath Tumor; Malignant Peripheral Nerve Sheath Tumors; Malignant Schwannoma; Malignant Schwannomas; MPNST; Neurilemmoma, Malignant; Neurilemmosarcoma; Neurilemmosarcomas; Neurilemoma, Malignant; Peripheral Nerve Sheath Tumors, Malignant; Schwannoma, Malignant
SNOMED CT: MPNST - Malignant peripheral nerve sheath tumor (19897006); Malignant peripheral nerve sheath tumor (404037002); Malignant schwannoma (404037002); Malignant peripheral nerve sheath tumor (19897006); Malignant neurilemoma (404037002)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0017827
Orphanet: ORPHA3148

Definition

Malignant peripheral nerve sheath tumor (MPNST) is a rare and often aggressive soft tissue sarcoma occurring in a wide range of anatomical sites. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
Follow this link to review classifications for Malignant peripheral nerve sheath tumor in Orphanet.

Professional guidelines

PubMed

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
Care of adults with neurofibromatosis type 1: a clinical practice resource of the American College of Medical Genetics and Genomics (ACMG).
Stewart DR, Korf BR, Nathanson KL, Stevenson DA, Yohay K
Genet Med 2018 Jul;20(7):671-682. Epub 2018 Apr 26 doi: 10.1038/gim.2018.28. PMID: 30006586
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

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

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
Comparison of Cancer Prevalence in Patients With Neurofibromatosis Type 1 at an Academic Cancer Center vs in the General Population From 1985 to 2020.
Landry JP, Schertz KL, Chiang YJ, Bhalla AD, Yi M, Keung EZ, Scally CP, Feig BW, Hunt KK, Roland CL, Guadagnolo A, Bishop AJ, Lazar AJ, Slopis JM, McCutcheon IE, Torres KE
JAMA Netw Open 2021 Mar 1;4(3):e210945. doi: 10.1001/jamanetworkopen.2021.0945. PMID: 33734413Free PMC Article
Neoadjuvant Chemotherapy in High-Risk Soft Tissue Sarcomas: Final Results of a Randomized Trial From Italian (ISG), Spanish (GEIS), French (FSG), and Polish (PSG) Sarcoma Groups.
Gronchi A, Palmerini E, Quagliuolo V, Martin Broto J, Lopez Pousa A, Grignani G, Brunello A, Blay JY, Tendero O, Diaz Beveridge R, Ferraresi V, Lugowska I, Merlo DF, Fontana V, Marchesi E, Braglia L, Donati DM, Palassini E, Bianchi G, Marrari A, Morosi C, Stacchiotti S, Bagué S, Coindre JM, Dei Tos AP, Picci P, Bruzzi P, Casali PG
J Clin Oncol 2020 Jul 1;38(19):2178-2186. Epub 2020 May 18 doi: 10.1200/JCO.19.03289. PMID: 32421444
Care of adults with neurofibromatosis type 1: a clinical practice resource of the American College of Medical Genetics and Genomics (ACMG).
Stewart DR, Korf BR, Nathanson KL, Stevenson DA, Yohay K
Genet Med 2018 Jul;20(7):671-682. Epub 2018 Apr 26 doi: 10.1038/gim.2018.28. PMID: 30006586
Neurofibromatosis type 1.
Cimino PJ, Gutmann DH
Handb Clin Neurol 2018;148:799-811. doi: 10.1016/B978-0-444-64076-5.00051-X. PMID: 29478615

Diagnosis

Malignant Spinal Tumors.
Noureldine MHA, Shimony N, Jallo GI
Adv Exp Med Biol 2023;1405:565-581. doi: 10.1007/978-3-031-23705-8_22. PMID: 37452954
Neurofibromatosis type 1.
Cimino PJ, Gutmann DH
Handb Clin Neurol 2018;148:799-811. doi: 10.1016/B978-0-444-64076-5.00051-X. PMID: 29478615
Histopathologic evaluation of atypical neurofibromatous tumors and their transformation into malignant peripheral nerve sheath tumor in patients with neurofibromatosis 1-a consensus overview.
Miettinen MM, Antonescu CR, Fletcher CDM, Kim A, Lazar AJ, Quezado MM, Reilly KM, Stemmer-Rachamimov A, Stewart DR, Viskochil D, Widemann B, Perry A
Hum Pathol 2017 Sep;67:1-10. Epub 2017 May 24 doi: 10.1016/j.humpath.2017.05.010. PMID: 28551330Free 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
Tumors and tumor-like lesions of peripheral nerves.
Woertler K
Semin Musculoskelet Radiol 2010 Nov;14(5):547-58. Epub 2010 Nov 11 doi: 10.1055/s-0030-1268073. PMID: 21072731

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
Single-Center Experience with Trabectedin for the Treatment of Non-L-sarcomas.
Cerdà Serdà P, Terés R, Sebio A, Bagué S, Orellana R, Moreno ME, Riba M, López-Pousa A
Adv Ther 2022 Apr;39(4):1596-1610. Epub 2022 Feb 7 doi: 10.1007/s12325-022-02051-2. PMID: 35129790
NF1, Neurofibromin and Gene Therapy: Prospects of Next-Generation Therapy.
Cui XW, Ren JY, Gu YH, Li QF, Wang ZC
Curr Gene Ther 2020;20(2):100-108. doi: 10.2174/1566523220666200806111451. PMID: 32767931
Neoadjuvant Chemotherapy in High-Risk Soft Tissue Sarcomas: Final Results of a Randomized Trial From Italian (ISG), Spanish (GEIS), French (FSG), and Polish (PSG) Sarcoma Groups.
Gronchi A, Palmerini E, Quagliuolo V, Martin Broto J, Lopez Pousa A, Grignani G, Brunello A, Blay JY, Tendero O, Diaz Beveridge R, Ferraresi V, Lugowska I, Merlo DF, Fontana V, Marchesi E, Braglia L, Donati DM, Palassini E, Bianchi G, Marrari A, Morosi C, Stacchiotti S, Bagué S, Coindre JM, Dei Tos AP, Picci P, Bruzzi P, Casali PG
J Clin Oncol 2020 Jul 1;38(19):2178-2186. Epub 2020 May 18 doi: 10.1200/JCO.19.03289. PMID: 32421444
Neurofibromatosis Type 1-Associated MPNST State of the Science: Outlining a Research Agenda for the Future.
Reilly KM, Kim A, Blakely J, Ferner RE, Gutmann DH, Legius E, Miettinen MM, Randall RL, Ratner N, Jumbé NL, Bakker A, Viskochil D, Widemann BC, Stewart DR
J Natl Cancer Inst 2017 Aug 1;109(8) doi: 10.1093/jnci/djx124. PMID: 29117388Free PMC Article

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
Malignant peripheral nerve sheath tumor: models, biology, and translation.
Somatilaka BN, Sadek A, McKay RM, Le LQ
Oncogene 2022 Apr;41(17):2405-2421. Epub 2022 Apr 7 doi: 10.1038/s41388-022-02290-1. PMID: 35393544Free PMC Article
The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior.
Tariq MU, Din NU, Abdul-Ghafar J, Park YK
Diagn Pathol 2021 Apr 20;16(1):32. doi: 10.1186/s13000-021-01095-2. PMID: 33879215Free PMC Article
The 2020 WHO Classification: What's New in Soft Tissue Tumor Pathology?
Kallen ME, Hornick JL
Am J Surg Pathol 2021 Jan;45(1):e1-e23. doi: 10.1097/PAS.0000000000001552. PMID: 32796172
Histopathologic evaluation of atypical neurofibromatous tumors and their transformation into malignant peripheral nerve sheath tumor in patients with neurofibromatosis 1-a consensus overview.
Miettinen MM, Antonescu CR, Fletcher CDM, Kim A, Lazar AJ, Quezado MM, Reilly KM, Stemmer-Rachamimov A, Stewart DR, Viskochil D, Widemann B, Perry A
Hum Pathol 2017 Sep;67:1-10. Epub 2017 May 24 doi: 10.1016/j.humpath.2017.05.010. PMID: 28551330Free PMC Article

Clinical prediction guides

Genomic Patterns of Malignant Peripheral Nerve Sheath Tumor (MPNST) Evolution Correlate with Clinical Outcome and Are Detectable in Cell-Free DNA.
Cortes-Ciriano I, Steele CD, Piculell K, Al-Ibraheemi A, Eulo V, Bui MM, Chatzipli A, Dickson BC, Borcherding DC, Feber A, Galor A, Hart J, Jones KB, Jordan JT, Kim RH, Lindsay D, Miller C, Nishida Y, Proszek PZ, Serrano J, Sundby RT, Szymanski JJ, Ullrich NJ, Viskochil D, Wang X, Snuderl M, Park PJ, Flanagan AM, Hirbe AC, Pillay N, Miller DT; Genomics of MPNST (GeM) Consortium
Cancer Discov 2023 Mar 1;13(3):654-671. doi: 10.1158/2159-8290.CD-22-0786. PMID: 36598417Free PMC Article
The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior.
Tariq MU, Din NU, Abdul-Ghafar J, Park YK
Diagn Pathol 2021 Apr 20;16(1):32. doi: 10.1186/s13000-021-01095-2. PMID: 33879215Free 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
Neoadjuvant Chemotherapy in High-Risk Soft Tissue Sarcomas: Final Results of a Randomized Trial From Italian (ISG), Spanish (GEIS), French (FSG), and Polish (PSG) Sarcoma Groups.
Gronchi A, Palmerini E, Quagliuolo V, Martin Broto J, Lopez Pousa A, Grignani G, Brunello A, Blay JY, Tendero O, Diaz Beveridge R, Ferraresi V, Lugowska I, Merlo DF, Fontana V, Marchesi E, Braglia L, Donati DM, Palassini E, Bianchi G, Marrari A, Morosi C, Stacchiotti S, Bagué S, Coindre JM, Dei Tos AP, Picci P, Bruzzi P, Casali PG
J Clin Oncol 2020 Jul 1;38(19):2178-2186. Epub 2020 May 18 doi: 10.1200/JCO.19.03289. PMID: 32421444
Histopathologic evaluation of atypical neurofibromatous tumors and their transformation into malignant peripheral nerve sheath tumor in patients with neurofibromatosis 1-a consensus overview.
Miettinen MM, Antonescu CR, Fletcher CDM, Kim A, Lazar AJ, Quezado MM, Reilly KM, Stemmer-Rachamimov A, Stewart DR, Viskochil D, Widemann B, Perry A
Hum Pathol 2017 Sep;67:1-10. Epub 2017 May 24 doi: 10.1016/j.humpath.2017.05.010. PMID: 28551330Free PMC Article

Recent systematic reviews

Mismatch repair deficiency is rare in bone and soft tissue tumors.
Lam SW, Kostine M, de Miranda NFCC, Schöffski P, Lee CJ, Morreau H, Bovée JVMG
Histopathology 2021 Oct;79(4):509-520. Epub 2021 Jun 8 doi: 10.1111/his.14377. PMID: 33825202Free PMC Article
Imaging of non-neurogenic peripheral nerve malignancy-a case series and systematic review.
Luna R, Fayad LM, Rodriguez FJ, Ahlawat S
Skeletal Radiol 2021 Jan;50(1):201-215. Epub 2020 Jul 23 doi: 10.1007/s00256-020-03556-z. PMID: 32699955
Treatment of neurofibromatosis 1-associated malignant peripheral nerve sheath tumors: a systematic review.
Tora MS, Xenos D, Texakalidis P, Boulis NM
Neurosurg Rev 2020 Aug;43(4):1039-1046. Epub 2019 Jun 17 doi: 10.1007/s10143-019-01135-y. PMID: 31209658
Radiation-Induced Malignant Peripheral Nerve Sheath Tumors: A Systematic Review.
Yamanaka R, Hayano A
World Neurosurg 2017 Sep;105:961-970.e8. Epub 2017 Jun 8 doi: 10.1016/j.wneu.2017.06.010. PMID: 28602926
Recurring intracranial malignant peripheral nerve sheath tumor: case report and systematic review of the literature.
van den Munckhof P, Germans MR, Schouten-van Meeteren AY, Oldenburger F, Troost D, Vandertop WP
Neurosurgery 2011 Apr;68(4):E1152-8; discussion E1159. doi: 10.1227/NEU.0b013e31820a1599. PMID: 21242836

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    Clinical resources

    Practice guidelines

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      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.

    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

    Reviews

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