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Unresectable Malignant Peripheral Nerve Sheath Tumor

MedGen UID:
1714529
•Concept ID:
C5236142
•
Neoplastic Process

Definition

A malignant peripheral nerve sheath tumor that is not amenable to surgical resection. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUnresectable 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

Phase II clinical trial of pazopanib for patients with unresectable or metastatic malignant peripheral nerve sheath tumors.
Nishida Y, Urakawa H, Nakayama R, Kobayashi E, Ozaki T, Ae K, Matsumoto Y, Tsuchiya H, Goto T, Hiraga H, Naka N, Takahashi S, Ando Y, Ando M, Kuwatsuka Y, Hamada S, Ueda T, Kawai A
Int J Cancer 2021 Jan 1;148(1):140-149. Epub 2020 Aug 3 doi: 10.1002/ijc.33201. PMID: 32638374
Outcome and prognostic factors in pediatric malignant peripheral nerve sheath tumors: An analysis of the European Pediatric Soft Tissue Sarcoma Group (EpSSG) NRSTS-2005 prospective study.
van Noesel MM, Orbach D, Brennan B, Kelsey A, Zanetti I, de Salvo GL, Gaze MN, Craigie RJ, McHugh K, Francotte N, Collini P, Bisogno G, Casanova M, Ferrari A
Pediatr Blood Cancer 2019 Oct;66(10):e27833. Epub 2019 Jun 26 doi: 10.1002/pbc.27833. PMID: 31241238
Epithelioid malignant peripheral nerve sheath tumor: clinicopathologic analysis of 63 cases.
Jo VY, Fletcher CD
Am J Surg Pathol 2015 May;39(5):673-82. doi: 10.1097/PAS.0000000000000379. PMID: 25602794
Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group.
Carli M, Ferrari A, Mattke A, Zanetti I, Casanova M, Bisogno G, Cecchetto G, Alaggio R, De Sio L, Koscielniak E, Sotti G, Treuner J
J Clin Oncol 2005 Nov 20;23(33):8422-30. doi: 10.1200/JCO.2005.01.4886. PMID: 16293873
Malignant peripheral nerve sheath tumor: a study of 21 cases.
Chang SM, Ho WL
Zhonghua Yi Xue Za Zhi (Taipei) 1994 Aug;54(2):122-30. PMID: 7954046

Diagnosis

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
Cardiac malignant peripheral nerve sheath tumor on computed tomography and magnetic resonance imaging: A case report.
Li S, Qiu Y, Yu J, Liang C, Peng L
Medicine (Baltimore) 2019 Nov;98(44):e17463. doi: 10.1097/MD.0000000000017463. PMID: 31689750Free PMC Article
Primary malignant peripheral nerve sheath tumor of the cauda equina with metastasis to the brain in a child: case report and literature review.
Xu Q, Xing B, Huang X, Wang R, Li Y, Yang Z
Spine J 2012 Apr;12(4):e7-13. Epub 2012 Apr 20 doi: 10.1016/j.spinee.2012.03.030. PMID: 22521675
Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group.
Carli M, Ferrari A, Mattke A, Zanetti I, Casanova M, Bisogno G, Cecchetto G, Alaggio R, De Sio L, Koscielniak E, Sotti G, Treuner J
J Clin Oncol 2005 Nov 20;23(33):8422-30. doi: 10.1200/JCO.2005.01.4886. PMID: 16293873
Malignant peripheral nerve sheath tumor: a study of 21 cases.
Chang SM, Ho WL
Zhonghua Yi Xue Za Zhi (Taipei) 1994 Aug;54(2):122-30. PMID: 7954046

Therapy

Outcome of Patients With Malignant Peripheral Nerve Sheath Tumors Enrolled on Sarcoma Alliance for Research Through Collaboration (SARC) Phase II Trials.
Akshintala S, Mallory NC, Lu Y, Ballman KV, Schuetze SM, Chugh R, Maki RG, Reinke DK, Widemann BC, Kim A
Oncologist 2023 May 8;28(5):453-459. doi: 10.1093/oncolo/oyac272. PMID: 36724001Free PMC Article
Phase II clinical trial of pazopanib for patients with unresectable or metastatic malignant peripheral nerve sheath tumors.
Nishida Y, Urakawa H, Nakayama R, Kobayashi E, Ozaki T, Ae K, Matsumoto Y, Tsuchiya H, Goto T, Hiraga H, Naka N, Takahashi S, Ando Y, Ando M, Kuwatsuka Y, Hamada S, Ueda T, Kawai A
Int J Cancer 2021 Jan 1;148(1):140-149. Epub 2020 Aug 3 doi: 10.1002/ijc.33201. PMID: 32638374
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
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
Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group.
Carli M, Ferrari A, Mattke A, Zanetti I, Casanova M, Bisogno G, Cecchetto G, Alaggio R, De Sio L, Koscielniak E, Sotti G, Treuner J
J Clin Oncol 2005 Nov 20;23(33):8422-30. doi: 10.1200/JCO.2005.01.4886. PMID: 16293873

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
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
Outcome and prognostic factors in pediatric malignant peripheral nerve sheath tumors: An analysis of the European Pediatric Soft Tissue Sarcoma Group (EpSSG) NRSTS-2005 prospective study.
van Noesel MM, Orbach D, Brennan B, Kelsey A, Zanetti I, de Salvo GL, Gaze MN, Craigie RJ, McHugh K, Francotte N, Collini P, Bisogno G, Casanova M, Ferrari A
Pediatr Blood Cancer 2019 Oct;66(10):e27833. Epub 2019 Jun 26 doi: 10.1002/pbc.27833. PMID: 31241238
Epithelioid malignant peripheral nerve sheath tumor: clinicopathologic analysis of 63 cases.
Jo VY, Fletcher CD
Am J Surg Pathol 2015 May;39(5):673-82. doi: 10.1097/PAS.0000000000000379. PMID: 25602794
Malignant peripheral nerve sheath tumor: a study of 21 cases.
Chang SM, Ho WL
Zhonghua Yi Xue Za Zhi (Taipei) 1994 Aug;54(2):122-30. PMID: 7954046

Clinical prediction guides

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
Epithelioid malignant peripheral nerve sheath tumor: clinicopathologic analysis of 63 cases.
Jo VY, Fletcher CD
Am J Surg Pathol 2015 May;39(5):673-82. doi: 10.1097/PAS.0000000000000379. PMID: 25602794
Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group.
Carli M, Ferrari A, Mattke A, Zanetti I, Casanova M, Bisogno G, Cecchetto G, Alaggio R, De Sio L, Koscielniak E, Sotti G, Treuner J
J Clin Oncol 2005 Nov 20;23(33):8422-30. doi: 10.1200/JCO.2005.01.4886. PMID: 16293873
Unusual primary tumors of the heart.
Vander Salm TJ
Semin Thorac Cardiovasc Surg 2000 Apr;12(2):89-100. doi: 10.1053/ct.2000.5080. PMID: 10807431
Malignant peripheral nerve sheath tumor: a study of 21 cases.
Chang SM, Ho WL
Zhonghua Yi Xue Za Zhi (Taipei) 1994 Aug;54(2):122-30. PMID: 7954046

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