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Refractory Malignant Peripheral Nerve Sheath Tumor

MedGen UID:
1665858
•Concept ID:
C4744490
•
Neoplastic Process

Definition

Malignant peripheral nerve sheath tumor that is resistant to treatment. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Molecular-guided therapy predictions reveal drug resistance phenotypes and treatment alternatives in malignant peripheral nerve sheath tumors.
Peacock JD, Cherba D, Kampfschulte K, Smith MK, Monks NR, Webb CP, Steensma M
J Transl Med 2013 Sep 17;11:213. doi: 10.1186/1479-5876-11-213. PMID: 24040940Free PMC Article
Expanded spectrum of viral therapy in the treatment of nervous system tumors.
Markert JM, Coen DM, Malick A, Mineta T, Martuza RL
J Neurosurg 1992 Oct;77(4):590-4. doi: 10.3171/jns.1992.77.4.0590. PMID: 1326612

Recent clinical studies

Etiology

ADVL1522: A phase 2 study of lorvotuzumab mertansine (IMGN901) in children with relapsed or refractory wilms tumor, rhabdomyosarcoma, neuroblastoma, pleuropulmonary blastoma, malignant peripheral nerve sheath tumor, or synovial sarcoma-A Children's Oncology Group study.
Geller JI, Pressey JG, Smith MA, Kudgus RA, Cajaiba M, Reid JM, Hall D, Barkauskas DA, Voss SD, Cho SY, Berg SL, Dome JS, Fox E, Weigel BJ
Cancer 2020 Dec 15;126(24):5303-5310. Epub 2020 Sep 11 doi: 10.1002/cncr.33195. PMID: 32914879Free PMC Article
A phase I study of sirolimus in combination with metronomic therapy (CHOAnome) in children with recurrent or refractory solid and brain tumors.
Qayed M, Cash T, Tighiouart M, MacDonald TJ, Goldsmith KC, Tanos R, Kean L, Watkins B, Suessmuth Y, Wetmore C, Katzenstein HM
Pediatr Blood Cancer 2020 Apr;67(4):e28134. Epub 2019 Dec 25 doi: 10.1002/pbc.28134. PMID: 31876107
A study of axitinib, a VEGF receptor tyrosine kinase inhibitor, in children and adolescents with recurrent or refractory solid tumors: A Children's Oncology Group phase 1 and pilot consortium trial (ADVL1315).
Geller JI, Fox E, Turpin BK, Goldstein SL, Liu X, Minard CG, Kudgus RA, Reid JM, Berg SL, Weigel BJ
Cancer 2018 Dec 1;124(23):4548-4555. Epub 2018 Nov 5 doi: 10.1002/cncr.31725. PMID: 30394521Free PMC Article
Phase II trial of ixabepilone administered daily for five days in children and young adults with refractory solid tumors: a report from the children's oncology group.
Jacobs S, Fox E, Krailo M, Hartley G, Navid F, Wexler L, Blaney SM, Goodwin A, Goodspeed W, Balis FM, Adamson PC, Widemann BC
Clin Cancer Res 2010 Jan 15;16(2):750-4. Epub 2010 Jan 12 doi: 10.1158/1078-0432.CCR-09-1906. PMID: 20068084Free PMC Article
Expanded spectrum of viral therapy in the treatment of nervous system tumors.
Markert JM, Coen DM, Malick A, Mineta T, Martuza RL
J Neurosurg 1992 Oct;77(4):590-4. doi: 10.3171/jns.1992.77.4.0590. PMID: 1326612

Diagnosis

Malignant peripheral nerve sheath tumor on a patient with a maternally inherited novel NF1 gene pathogenic germline variant: Case report.
Moreno-Salgado R, Rios-Lozano YZ, Tamayo-Palacio AC, Castillo AI, Hidalgo-Martínez MF
Cancer Genet 2023 Jun;274-275:72-74. Epub 2023 Apr 15 doi: 10.1016/j.cancergen.2023.04.003. PMID: 37087941
Activation of Receptor Tyrosine Kinases Mediates Acquired Resistance to MEK Inhibition in Malignant Peripheral Nerve Sheath Tumors.
Wang J, Pollard K, Calizo A, Pratilas CA
Cancer Res 2021 Feb 1;81(3):747-762. Epub 2020 Nov 17 doi: 10.1158/0008-5472.CAN-20-1992. PMID: 33203698Free PMC Article
ADVL1522: A phase 2 study of lorvotuzumab mertansine (IMGN901) in children with relapsed or refractory wilms tumor, rhabdomyosarcoma, neuroblastoma, pleuropulmonary blastoma, malignant peripheral nerve sheath tumor, or synovial sarcoma-A Children's Oncology Group study.
Geller JI, Pressey JG, Smith MA, Kudgus RA, Cajaiba M, Reid JM, Hall D, Barkauskas DA, Voss SD, Cho SY, Berg SL, Dome JS, Fox E, Weigel BJ
Cancer 2020 Dec 15;126(24):5303-5310. Epub 2020 Sep 11 doi: 10.1002/cncr.33195. PMID: 32914879Free PMC Article
A phase I study of sirolimus in combination with metronomic therapy (CHOAnome) in children with recurrent or refractory solid and brain tumors.
Qayed M, Cash T, Tighiouart M, MacDonald TJ, Goldsmith KC, Tanos R, Kean L, Watkins B, Suessmuth Y, Wetmore C, Katzenstein HM
Pediatr Blood Cancer 2020 Apr;67(4):e28134. Epub 2019 Dec 25 doi: 10.1002/pbc.28134. PMID: 31876107
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

Activation of Receptor Tyrosine Kinases Mediates Acquired Resistance to MEK Inhibition in Malignant Peripheral Nerve Sheath Tumors.
Wang J, Pollard K, Calizo A, Pratilas CA
Cancer Res 2021 Feb 1;81(3):747-762. Epub 2020 Nov 17 doi: 10.1158/0008-5472.CAN-20-1992. PMID: 33203698Free PMC Article
ADVL1522: A phase 2 study of lorvotuzumab mertansine (IMGN901) in children with relapsed or refractory wilms tumor, rhabdomyosarcoma, neuroblastoma, pleuropulmonary blastoma, malignant peripheral nerve sheath tumor, or synovial sarcoma-A Children's Oncology Group study.
Geller JI, Pressey JG, Smith MA, Kudgus RA, Cajaiba M, Reid JM, Hall D, Barkauskas DA, Voss SD, Cho SY, Berg SL, Dome JS, Fox E, Weigel BJ
Cancer 2020 Dec 15;126(24):5303-5310. Epub 2020 Sep 11 doi: 10.1002/cncr.33195. PMID: 32914879Free PMC Article
A phase I study of sirolimus in combination with metronomic therapy (CHOAnome) in children with recurrent or refractory solid and brain tumors.
Qayed M, Cash T, Tighiouart M, MacDonald TJ, Goldsmith KC, Tanos R, Kean L, Watkins B, Suessmuth Y, Wetmore C, Katzenstein HM
Pediatr Blood Cancer 2020 Apr;67(4):e28134. Epub 2019 Dec 25 doi: 10.1002/pbc.28134. PMID: 31876107
A study of axitinib, a VEGF receptor tyrosine kinase inhibitor, in children and adolescents with recurrent or refractory solid tumors: A Children's Oncology Group phase 1 and pilot consortium trial (ADVL1315).
Geller JI, Fox E, Turpin BK, Goldstein SL, Liu X, Minard CG, Kudgus RA, Reid JM, Berg SL, Weigel BJ
Cancer 2018 Dec 1;124(23):4548-4555. Epub 2018 Nov 5 doi: 10.1002/cncr.31725. PMID: 30394521Free PMC Article
Molecular-guided therapy predictions reveal drug resistance phenotypes and treatment alternatives in malignant peripheral nerve sheath tumors.
Peacock JD, Cherba D, Kampfschulte K, Smith MK, Monks NR, Webb CP, Steensma M
J Transl Med 2013 Sep 17;11:213. doi: 10.1186/1479-5876-11-213. PMID: 24040940Free PMC Article

Prognosis

Malignant peripheral nerve sheath tumor on a patient with a maternally inherited novel NF1 gene pathogenic germline variant: Case report.
Moreno-Salgado R, Rios-Lozano YZ, Tamayo-Palacio AC, Castillo AI, Hidalgo-Martínez MF
Cancer Genet 2023 Jun;274-275:72-74. Epub 2023 Apr 15 doi: 10.1016/j.cancergen.2023.04.003. PMID: 37087941
Molecular-guided therapy predictions reveal drug resistance phenotypes and treatment alternatives in malignant peripheral nerve sheath tumors.
Peacock JD, Cherba D, Kampfschulte K, Smith MK, Monks NR, Webb CP, Steensma M
J Transl Med 2013 Sep 17;11:213. doi: 10.1186/1479-5876-11-213. PMID: 24040940Free PMC Article

Clinical prediction guides

A phase I study of sirolimus in combination with metronomic therapy (CHOAnome) in children with recurrent or refractory solid and brain tumors.
Qayed M, Cash T, Tighiouart M, MacDonald TJ, Goldsmith KC, Tanos R, Kean L, Watkins B, Suessmuth Y, Wetmore C, Katzenstein HM
Pediatr Blood Cancer 2020 Apr;67(4):e28134. Epub 2019 Dec 25 doi: 10.1002/pbc.28134. PMID: 31876107
Molecular-guided therapy predictions reveal drug resistance phenotypes and treatment alternatives in malignant peripheral nerve sheath tumors.
Peacock JD, Cherba D, Kampfschulte K, Smith MK, Monks NR, Webb CP, Steensma M
J Transl Med 2013 Sep 17;11:213. doi: 10.1186/1479-5876-11-213. PMID: 24040940Free PMC Article
Phase II trial of ixabepilone administered daily for five days in children and young adults with refractory solid tumors: a report from the children's oncology group.
Jacobs S, Fox E, Krailo M, Hartley G, Navid F, Wexler L, Blaney SM, Goodwin A, Goodspeed W, Balis FM, Adamson PC, Widemann BC
Clin Cancer Res 2010 Jan 15;16(2):750-4. Epub 2010 Jan 12 doi: 10.1158/1078-0432.CCR-09-1906. PMID: 20068084Free PMC Article
Phase I clinical evaluation of diaziquone in childhood cancer.
Ettinger LJ, Siegel SE, Belasco JB, Evans AE, Ruccione KS, Jamin DC, Rohrbaugh TM, Higgins GR
Cancer Treat Rep 1985 Mar;69(3):323-7. PMID: 3856480

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