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NF1-Associated Malignant Peripheral Nerve Sheath Tumor

MedGen UID:
1709893
•Concept ID:
C5236143
•
Neoplastic Process

Definition

A malignant peripheral nerve sheath tumor that is associated with NF1 gene inactivation and a history of neurofibromatosis type 1. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNF1-Associated Malignant Peripheral Nerve Sheath Tumor

Recent clinical studies

Etiology

Circulating tumor DNA for malignant peripheral nerve sheath tumors in neurofibromatosis type 1.
Jones J, Cain S, Pesic-Smith J, Choong PFM, Morokoff AP, Drummond KJ, Dabscheck G
J Neurooncol 2021 Sep;154(3):265-274. Epub 2021 Sep 16 doi: 10.1007/s11060-021-03846-z. PMID: 34529228
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: 33721151
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
Recent Advances in the Diagnosis and Pathogenesis of Neurofibromatosis Type 1 (NF1)-associated Peripheral Nervous System Neoplasms.
Longo JF, Weber SM, Turner-Ivey BP, Carroll SL
Adv Anat Pathol 2018 Sep;25(5):353-368. doi: 10.1097/PAP.0000000000000197. PMID: 29762158Free PMC Article
Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1.
Vasconcelos RAT, Coscarelli PG, Alvarenga RP, Acioly MA
Arq Neuropsiquiatr 2017 Jun;75(6):366-371. doi: 10.1590/0004-282X20170052. PMID: 28658406

Diagnosis

Circulating tumor DNA for malignant peripheral nerve sheath tumors in neurofibromatosis type 1.
Jones J, Cain S, Pesic-Smith J, Choong PFM, Morokoff AP, Drummond KJ, Dabscheck G
J Neurooncol 2021 Sep;154(3):265-274. Epub 2021 Sep 16 doi: 10.1007/s11060-021-03846-z. PMID: 34529228
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
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
Recent Advances in the Diagnosis and Pathogenesis of Neurofibromatosis Type 1 (NF1)-associated Peripheral Nervous System Neoplasms.
Longo JF, Weber SM, Turner-Ivey BP, Carroll SL
Adv Anat Pathol 2018 Sep;25(5):353-368. doi: 10.1097/PAP.0000000000000197. PMID: 29762158Free PMC Article
Neurofibromatosis type 1 (NF1) and associated tumors.
Rosenbaum T, Wimmer K
Klin Padiatr 2014 Nov;226(6-7):309-15. Epub 2014 Jul 25 doi: 10.1055/s-0034-1382021. PMID: 25062113

Therapy

MEK Inhibition Synergizes with TYK2 Inhibitors in NF1-Associated Malignant Peripheral Nerve Sheath Tumors.
Borcherding DC, Amin NV, He K, Zhang X, Lyu Y, Dehner C, Bhatia H, Gothra A, Daud L, Ruminski P, Pratilas CA, Pollard K, Sundby T, Widemann BC, Hirbe AC
Clin Cancer Res 2023 Apr 14;29(8):1592-1604. doi: 10.1158/1078-0432.CCR-22-3722. PMID: 36799629Free 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: 33721151
Combined Targeting of AKT and mTOR Inhibits Proliferation of Human NF1-Associated Malignant Peripheral Nerve Sheath Tumour Cells In Vitro but not in a Xenograft Mouse Model In Vivo.
Schulte A, Ewald F, Spyra M, Smit DJ, Jiang W, Salamon J, Jücker M, Mautner VF
Int J Mol Sci 2020 Feb 24;21(4) doi: 10.3390/ijms21041548. PMID: 32102484Free PMC Article
Overcoming BET Inhibitor Resistance in Malignant Peripheral Nerve Sheath Tumors.
Cooper JM, Patel AJ, Chen Z, Liao CP, Chen K, Mo J, Wang Y, Le LQ
Clin Cancer Res 2019 Jun 1;25(11):3404-3416. Epub 2019 Feb 22 doi: 10.1158/1078-0432.CCR-18-2437. PMID: 30796033Free PMC Article
Dual mTORC1/2 inhibition induces anti-proliferative effect in NF1-associated plexiform neurofibroma and malignant peripheral nerve sheath tumor cells.
Varin J, Poulain L, Hivelin M, Nusbaum P, Hubas A, Laurendeau I, Lantieri L, Wolkenstein P, Vidaud M, Pasmant E, Chapuis N, Parfait B
Oncotarget 2016 Jun 14;7(24):35753-35767. doi: 10.18632/oncotarget.7099. PMID: 26840085Free PMC Article

Prognosis

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
Recent Advances in the Diagnosis and Pathogenesis of Neurofibromatosis Type 1 (NF1)-associated Peripheral Nervous System Neoplasms.
Longo JF, Weber SM, Turner-Ivey BP, Carroll SL
Adv Anat Pathol 2018 Sep;25(5):353-368. doi: 10.1097/PAP.0000000000000197. PMID: 29762158Free PMC Article
Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1.
Vasconcelos RAT, Coscarelli PG, Alvarenga RP, Acioly MA
Arq Neuropsiquiatr 2017 Jun;75(6):366-371. doi: 10.1590/0004-282X20170052. PMID: 28658406
Confirmation of mutation landscape of NF1-associated malignant peripheral nerve sheath tumors.
Sohier P, Luscan A, Lloyd A, Ashelford K, Laurendeau I, Briand-Suleau A, Vidaud D, Ortonne N, Pasmant E, Upadhyaya M
Genes Chromosomes Cancer 2017 May;56(5):421-426. Epub 2017 Mar 7 doi: 10.1002/gcc.22446. PMID: 28124441
Neurofibromatosis type 1 (NF1) and associated tumors.
Rosenbaum T, Wimmer K
Klin Padiatr 2014 Nov;226(6-7):309-15. Epub 2014 Jul 25 doi: 10.1055/s-0034-1382021. PMID: 25062113

Clinical prediction guides

Recent Advances in the Diagnosis and Pathogenesis of Neurofibromatosis Type 1 (NF1)-associated Peripheral Nervous System Neoplasms.
Longo JF, Weber SM, Turner-Ivey BP, Carroll SL
Adv Anat Pathol 2018 Sep;25(5):353-368. doi: 10.1097/PAP.0000000000000197. PMID: 29762158Free PMC Article
Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1.
Vasconcelos RAT, Coscarelli PG, Alvarenga RP, Acioly MA
Arq Neuropsiquiatr 2017 Jun;75(6):366-371. doi: 10.1590/0004-282X20170052. PMID: 28658406
NF1(+/-) Hematopoietic Cells Accelerate Malignant Peripheral Nerve Sheath Tumor Development without Altering Chemotherapy Response.
Dodd RD, Lee CL, Overton T, Huang W, Eward WC, Luo L, Ma Y, Ingram DR, Torres KE, Cardona DM, Lazar AJ, Kirsch DG
Cancer Res 2017 Aug 15;77(16):4486-4497. Epub 2017 Jun 23 doi: 10.1158/0008-5472.CAN-16-2643. PMID: 28646022Free PMC Article
Confirmation of mutation landscape of NF1-associated malignant peripheral nerve sheath tumors.
Sohier P, Luscan A, Lloyd A, Ashelford K, Laurendeau I, Briand-Suleau A, Vidaud D, Ortonne N, Pasmant E, Upadhyaya M
Genes Chromosomes Cancer 2017 May;56(5):421-426. Epub 2017 Mar 7 doi: 10.1002/gcc.22446. PMID: 28124441
Neurofibromatosis type 1 (NF1) and associated tumors.
Rosenbaum T, Wimmer K
Klin Padiatr 2014 Nov;226(6-7):309-15. Epub 2014 Jul 25 doi: 10.1055/s-0034-1382021. PMID: 25062113

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