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Low Grade Malignant Peripheral Nerve Sheath Tumor

MedGen UID:
232702
•Concept ID:
C1334416
•
Neoplastic Process
Synonym: Well Differentiated Malignant Peripheral Nerve Sheath Tumor

Definition

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

Term Hierarchy

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

Professional guidelines

PubMed

Myxoid solitary fibrous tumor: a study of seven cases with emphasis on differential diagnosis.
de Saint Aubain Somerhausen N, Rubin BP, Fletcher CD
Mod Pathol 1999 May;12(5):463-71. PMID: 10349983

Recent clinical studies

Etiology

ALK-rearranged Mesenchymal Neoplasms: A Report of 9 cases Further Expanding the Clinicopathologic Spectrum of Emerging Kinase Fusion Positive Group of Tumors.
Dermawan JK, DiNapoli SE, Mullaney KA, Sukhadia P, Agaram NP, Dickson BC, Antonescu CR
Genes Chromosomes Cancer 2023 Feb;62(2):75-84. Epub 2022 Oct 7 doi: 10.1002/gcc.23097. PMID: 36125853Free PMC Article
Temporary skin closure in extremity soft tissue sarcoma - our indications.
J M, P C, V Š, M F, Z M
Acta Chir Plast 2022 Summer;64(2):69-75. doi: 10.48095/ccachp202269. PMID: 36068081
Atypical Spindle Cell Lipomatous Tumor: Clinicopathologic Characterization of 232 Cases Demonstrating a Morphologic Spectrum.
Mariño-Enriquez A, Nascimento AF, Ligon AH, Liang C, Fletcher CD
Am J Surg Pathol 2017 Feb;41(2):234-244. doi: 10.1097/PAS.0000000000000770. PMID: 27879515
Malignant peripheral nerve sheath tumor of the abducens nerve and a review of the literature.
Voorhies J, Hattab EM, Cohen-Gadol AA
World Neurosurg 2013 Nov;80(5):654.e1-8. Epub 2012 Sep 25 doi: 10.1016/j.wneu.2012.08.007. PMID: 23022650
Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases.
Dei Tos AP, Mentzel T, Newman PL, Fletcher CD
Am J Surg Pathol 1994 Sep;18(9):913-21. doi: 10.1097/00000478-199409000-00006. PMID: 8067512

Diagnosis

Low-Grade Malignant Peripheral Nerve Sheath Tumor Mimicking Schwannoma: Role and Importance of Trimethylated H3K27M Staining.
Gilder HE, Puffer RC, Spinner RJ, Raghunathan A, Bydon M
World Neurosurg 2018 Sep;117:178-181. Epub 2018 Jun 14 doi: 10.1016/j.wneu.2018.06.035. PMID: 29909207
Low-grade fibromyxoid sarcoma arising within the median nerve.
Swanson AA, Giannini C, Folpe AL, Van Dyke DL, Amrami KK, Michalak WA, Vaubel RA
Neuropathology 2018 Jun;38(3):309-314. Epub 2018 Jan 3 doi: 10.1111/neup.12453. PMID: 29314300
Superficial malignant peripheral nerve sheath tumor with overlying intradermal melanocytic nevus mimicking spindle cell melanoma.
Jackson CR, Minca EC, Kapil JP, Smith SC, Billings SD
J Cutan Pathol 2016 Dec;43(12):1220-1225. Epub 2016 Oct 24 doi: 10.1111/cup.12834. PMID: 27687011
Low grade malignant peripheral nerve sheath tumor with mesenchymal differentiation: a case report.
Janczar K, Tybor K, Józefowicz M, Papierz W
Pol J Pathol 2011 Dec;62(4):278-81. PMID: 22246916
Low grade malignant peripheral nerve sheath tumor with smooth muscle differentiation.
Rodriguez FJ, Scheithauer BW, Abell-Aleff PC, Elamin E, Erlandson RA
Acta Neuropathol 2007 Jun;113(6):705-9. Epub 2006 Nov 21 doi: 10.1007/s00401-006-0171-8. PMID: 17119986

Prognosis

Low-Grade Malignant Peripheral Nerve Sheath Tumor Mimicking Schwannoma: Role and Importance of Trimethylated H3K27M Staining.
Gilder HE, Puffer RC, Spinner RJ, Raghunathan A, Bydon M
World Neurosurg 2018 Sep;117:178-181. Epub 2018 Jun 14 doi: 10.1016/j.wneu.2018.06.035. PMID: 29909207
Low-grade Schwann cell neoplasms with leptomeningeal dissemination: clinicopathologic and autopsy findings.
Rodriguez EF, Blakeley J, Langmead S, Olivi A, Tufaro A, Tabbarah A, Berkenblit G, Sacks JM, Newsome SD, Montgomery E, Rodriguez FJ
Hum Pathol 2017 Feb;60:121-128. Epub 2016 Sep 22 doi: 10.1016/j.humpath.2016.08.010. PMID: 27666764Free PMC Article
Malignant peripheral nerve sheath tumor of the abducens nerve and a review of the literature.
Voorhies J, Hattab EM, Cohen-Gadol AA
World Neurosurg 2013 Nov;80(5):654.e1-8. Epub 2012 Sep 25 doi: 10.1016/j.wneu.2012.08.007. PMID: 23022650
Low grade malignant peripheral nerve sheath tumor with smooth muscle differentiation.
Rodriguez FJ, Scheithauer BW, Abell-Aleff PC, Elamin E, Erlandson RA
Acta Neuropathol 2007 Jun;113(6):705-9. Epub 2006 Nov 21 doi: 10.1007/s00401-006-0171-8. PMID: 17119986

Clinical prediction guides

Atypical Spindle Cell Lipomatous Tumor: Clinicopathologic Characterization of 232 Cases Demonstrating a Morphologic Spectrum.
Mariño-Enriquez A, Nascimento AF, Ligon AH, Liang C, Fletcher CD
Am J Surg Pathol 2017 Feb;41(2):234-244. doi: 10.1097/PAS.0000000000000770. PMID: 27879515
Superficial malignant peripheral nerve sheath tumor with overlying intradermal melanocytic nevus mimicking spindle cell melanoma.
Jackson CR, Minca EC, Kapil JP, Smith SC, Billings SD
J Cutan Pathol 2016 Dec;43(12):1220-1225. Epub 2016 Oct 24 doi: 10.1111/cup.12834. PMID: 27687011
A neurogenic tumor containing a low-grade malignant peripheral nerve sheath tumor (MPNST) component with loss of p16 expression and homozygous deletion of CDKN2A/p16: a case report showing progression from a neurofibroma to a high-grade MPNST.
Tajima S, Koda K
Int J Clin Exp Pathol 2015;8(5):5113-20. Epub 2015 May 1 PMID: 26191206Free PMC Article
Fibroblastic low-grade malignant peripheral nerve sheath tumor in the orbit.
Han JC, Kim YD, Suh YL, Kwon MJ, Woo KI
Ophthalmic Plast Reconstr Surg 2012 Jul-Aug;28(4):e97-8. doi: 10.1097/IOP.0b013e318236857c. PMID: 22186985
Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases.
Dei Tos AP, Mentzel T, Newman PL, Fletcher CD
Am J Surg Pathol 1994 Sep;18(9):913-21. doi: 10.1097/00000478-199409000-00006. PMID: 8067512

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