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High Grade Malignant Peripheral Nerve Sheath Tumor

MedGen UID:
232285
•Concept ID:
C1334005
•
Neoplastic Process
Synonym: Poorly Differentiated Malignant Peripheral Nerve Sheath Tumor

Definition

A malignant peripheral nerve sheath tumor that is high grade. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 2.
Chokoeva AA, Tchernev G, Cardoso JC, Patterson JW, Dechev I, Valkanov S, Zanardelli M, Lotti T, Wollina U
Int J Immunopathol Pharmacol 2015 Jun;28(2):178-86. Epub 2015 Mar 26 doi: 10.1177/0394632015575977. PMID: 25816393
Management of childhood malignant peripheral nerve sheath tumor.
Ferrari A, Bisogno G, Carli M
Paediatr Drugs 2007;9(4):239-48. doi: 10.2165/00148581-200709040-00005. PMID: 17705563
Retroperitoneal soft tissue sarcomas: prognosis and treatment of primary and recurrent disease in 117 patients.
Alldinger I, Yang Q, Pilarsky C, Saeger HD, Knoefel WT, Peiper M
Anticancer Res 2006 Mar-Apr;26(2B):1577-81. PMID: 16619574

Recent clinical studies

Etiology

Role of perioperative chemotherapy and radiotherapy for localized high-grade malignant peripheral nerve sheath tumor at the extremities and trunk wall: a population-based cohort study.
Kobayashi H, Zhang L, Okajima K, Hirai T, Tsuda Y, Ikegami M, Kawai A, Tanaka S
Jpn J Clin Oncol 2023 Jan 28;53(2):138-145. doi: 10.1093/jjco/hyac178. PMID: 36461784
Deep-seated plexiform schwannoma: a pathologic study of 16 cases and comparative analysis with the superficial variety.
Agaram NP, Prakash S, Antonescu CR
Am J Surg Pathol 2005 Aug;29(8):1042-8. PMID: 16006798

Diagnosis

PRC2-Inactivating Mutations in Cancer Enhance Cytotoxic Response to DNMT1-Targeted Therapy via Enhanced Viral Mimicry.
Patel AJ, Warda S, Maag JLV, Misra R, Miranda-Román MA, Pachai MR, Lee CJ, Li D, Wang N, Bayshtok G, Fishinevich E, Meng Y, Wong EWP, Yan J, Giff E, Pappalardi MB, McCabe MT, Fletcher JA, Rudin CM, Chandarlapaty S, Scandura JM, Koche RP, Glass JL, Antonescu CR, Zheng D, Chen Y, Chi P
Cancer Discov 2022 Sep 2;12(9):2120-2139. doi: 10.1158/2159-8290.CD-21-1671. PMID: 35789380Free PMC Article
Sporadic High-Grade Malignant Peripheral Nerve Sheath Tumor of the Hypoglossal Nerve.
Colizza A, D'Aguanno V, Greco A, De Seta D, Gianno F, Riminucci M, de Vincentiis M, Corsi A
Ear Nose Throat J 2022 Jan;101(1):23-26. Epub 2020 Jul 1 doi: 10.1177/0145561320936973. PMID: 32609545

Prognosis

Role of perioperative chemotherapy and radiotherapy for localized high-grade malignant peripheral nerve sheath tumor at the extremities and trunk wall: a population-based cohort study.
Kobayashi H, Zhang L, Okajima K, Hirai T, Tsuda Y, Ikegami M, Kawai A, Tanaka S
Jpn J Clin Oncol 2023 Jan 28;53(2):138-145. doi: 10.1093/jjco/hyac178. PMID: 36461784
PRC2-Inactivating Mutations in Cancer Enhance Cytotoxic Response to DNMT1-Targeted Therapy via Enhanced Viral Mimicry.
Patel AJ, Warda S, Maag JLV, Misra R, Miranda-Román MA, Pachai MR, Lee CJ, Li D, Wang N, Bayshtok G, Fishinevich E, Meng Y, Wong EWP, Yan J, Giff E, Pappalardi MB, McCabe MT, Fletcher JA, Rudin CM, Chandarlapaty S, Scandura JM, Koche RP, Glass JL, Antonescu CR, Zheng D, Chen Y, Chi P
Cancer Discov 2022 Sep 2;12(9):2120-2139. doi: 10.1158/2159-8290.CD-21-1671. PMID: 35789380Free PMC Article
Deep-seated plexiform schwannoma: a pathologic study of 16 cases and comparative analysis with the superficial variety.
Agaram NP, Prakash S, Antonescu CR
Am J Surg Pathol 2005 Aug;29(8):1042-8. PMID: 16006798

Clinical prediction guides

Role of perioperative chemotherapy and radiotherapy for localized high-grade malignant peripheral nerve sheath tumor at the extremities and trunk wall: a population-based cohort study.
Kobayashi H, Zhang L, Okajima K, Hirai T, Tsuda Y, Ikegami M, Kawai A, Tanaka S
Jpn J Clin Oncol 2023 Jan 28;53(2):138-145. doi: 10.1093/jjco/hyac178. PMID: 36461784
PRC2-Inactivating Mutations in Cancer Enhance Cytotoxic Response to DNMT1-Targeted Therapy via Enhanced Viral Mimicry.
Patel AJ, Warda S, Maag JLV, Misra R, Miranda-Román MA, Pachai MR, Lee CJ, Li D, Wang N, Bayshtok G, Fishinevich E, Meng Y, Wong EWP, Yan J, Giff E, Pappalardi MB, McCabe MT, Fletcher JA, Rudin CM, Chandarlapaty S, Scandura JM, Koche RP, Glass JL, Antonescu CR, Zheng D, Chen Y, Chi P
Cancer Discov 2022 Sep 2;12(9):2120-2139. doi: 10.1158/2159-8290.CD-21-1671. PMID: 35789380Free PMC Article

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