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Malignant Peripheral Nerve Sheath Tumor of the Skin

MedGen UID:
1735957
•Concept ID:
C5419747
•
Neoplastic Process
Synonyms: Cutaneous Malignant Peripheral Nerve Sheath Tumor; Dermal Malignant Peripheral Nerve Sheath Tumor; Skin Malignant Peripheral Nerve Sheath Tumor

Definition

A malignant peripheral nerve sheath tumor that arises from the skin. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMalignant Peripheral Nerve Sheath Tumor of the Skin

Professional guidelines

PubMed

Unplanned excision of soft-tissue sarcomas: current concepts for management and prognosis.
Pretell-Mazzini J, Barton MD Jr, Conway SA, Temple HT
J Bone Joint Surg Am 2015 Apr 1;97(7):597-603. doi: 10.2106/JBJS.N.00649. PMID: 25834085
Neurofibromatosis type 1 (NF1): diagnosis and management.
Ferner RE, Gutmann DH
Handb Clin Neurol 2013;115:939-55. doi: 10.1016/B978-0-444-52902-2.00053-9. PMID: 23931823
Epithelioid angiosarcoma: a brief diagnostic review and differential diagnosis.
Hart J, Mandavilli S
Arch Pathol Lab Med 2011 Feb;135(2):268-72. doi: 10.5858/135.2.268. PMID: 21284449

Recent clinical studies

Etiology

Estimated Prevalence, Tumor Spectrum, and Neurofibromatosis Type 1-Like Phenotype of CDKN2A-Related Melanoma-Astrocytoma Syndrome.
Sargen MR, Kim J, Potjer TP, Velthuizen ME, Martir-Negron AE, Odia Y, Helgadottir H, Hatton JN, Haley JS, Thone G, Widemann BC, Gross AM, Yohe ME, Kaplan RN, Shern JF, Sundby RT, Astiazaran-Symonds E, Yang XR, Carey DJ, Tucker MA, Stewart DR, Goldstein AM
JAMA Dermatol 2023 Oct 1;159(10):1112-1118. doi: 10.1001/jamadermatol.2023.2621. PMID: 37585199Free PMC Article
Genomic Landscape of Malignant Peripheral Nerve Sheath Tumor‒Like Melanoma.
Muniz TP, Sorotsky H, Kanjanapan Y, Rose AAN, Araujo DV, Fortuna A, Ghazarian D, Kamil ZS, Pugh T, Mah M, Thiagarajah M, Torti D, Spreafico A, Hogg D
J Invest Dermatol 2021 Oct;141(10):2470-2479. Epub 2021 Apr 6 doi: 10.1016/j.jid.2021.03.016. PMID: 33831431
Diagnostic Pathology of Tumors of Peripheral Nerve.
Belakhoua SM, Rodriguez FJ
Neurosurgery 2021 Feb 16;88(3):443-456. doi: 10.1093/neuros/nyab021. PMID: 33588442Free PMC Article
Neurofibromatosis type 1.
Anderson JL, Gutmann DH
Handb Clin Neurol 2015;132:75-86. doi: 10.1016/B978-0-444-62702-5.00004-4. PMID: 26564071
Superficial or cutaneous malignant peripheral nerve sheath tumor--clinical experience at Taipei Veterans General Hospital.
Feng CJ, Ma H, Liao WC
Ann Plast Surg 2015 May;74 Suppl 2:S85-8. doi: 10.1097/SAP.0000000000000465. PMID: 25695445

Diagnosis

Diagnostic Pathology of Tumors of Peripheral Nerve.
Belakhoua SM, Rodriguez FJ
Neurosurgery 2021 Feb 16;88(3):443-456. doi: 10.1093/neuros/nyab021. PMID: 33588442Free PMC Article
Phakomatoses.
Becker B, Strowd RE 3rd
Dermatol Clin 2019 Oct;37(4):583-606. doi: 10.1016/j.det.2019.05.015. PMID: 31466597
Cutaneous Malignant Peripheral Nerve Sheath Tumor.
Luzar B, Falconieri G
Surg Pathol Clin 2017 Jun;10(2):337-343. Epub 2017 Mar 14 doi: 10.1016/j.path.2017.01.008. PMID: 28477884
Neurofibromatosis type 1 (NF1): diagnosis and management.
Ferner RE, Gutmann DH
Handb Clin Neurol 2013;115:939-55. doi: 10.1016/B978-0-444-52902-2.00053-9. PMID: 23931823
Neurofibromatosis.
Spira M, Riccardi V
Clin Plast Surg 1987 Apr;14(2):315-25. PMID: 3107867

Therapy

Fungating malignant peripheral nerve sheath tumor arising from a slow-growing mass in the forearm: a case report and review of the literature.
Abdel Al S, Abou Chaar MK, Asha W, Al-Najjar H, Al-Hussaini M
J Med Case Rep 2020 Jul 7;14(1):91. doi: 10.1186/s13256-020-02427-4. PMID: 32631436Free PMC Article
Integrative Analysis Identifies Candidate Tumor Microenvironment and Intracellular Signaling Pathways that Define Tumor Heterogeneity in NF1.
Banerjee J, Allaway RJ, Taroni JN, Baker A, Zhang X, Moon CI, Pratilas CA, Blakeley JO, Guinney J, Hirbe A, Greene CS, Gosline SJ
Genes (Basel) 2020 Feb 21;11(2) doi: 10.3390/genes11020226. PMID: 32098059Free PMC Article
Phenotypic and genotypic overlap between mosaic NF2 and schwannomatosis in patients with multiple non-intradermal schwannomas.
Kehrer-Sawatzki H, Kluwe L, Friedrich RE, Summerer A, Schäfer E, Wahlländer U, Matthies C, Gugel I, Farschtschi S, Hagel C, Cooper DN, Mautner VF
Hum Genet 2018 Jul;137(6-7):543-552. Epub 2018 Jul 13 doi: 10.1007/s00439-018-1909-9. PMID: 30006736
Vemurafenib treatment of BRAF V600E-mutated malignant peripheral nerve sheath tumor.
Kaplan HG
J Natl Compr Canc Netw 2013 Dec 1;11(12):1466-70. doi: 10.6004/jnccn.2013.0173. PMID: 24335681
Radiation-induced malignant and atypical peripheral nerve sheath tumors.
Foley KM, Woodruff JM, Ellis FT, Posner JB
Ann Neurol 1980 Apr;7(4):311-8. doi: 10.1002/ana.410070405. PMID: 7377756

Prognosis

Cancer and Central Nervous System Tumor Surveillance in Pediatric Neurofibromatosis 2 and Related Disorders.
Evans DGR, Salvador H, Chang VY, Erez A, Voss SD, Druker H, Scott HS, Tabori U
Clin Cancer Res 2017 Jun 15;23(12):e54-e61. doi: 10.1158/1078-0432.CCR-17-0590. PMID: 28620005
Unplanned excision of soft-tissue sarcomas: current concepts for management and prognosis.
Pretell-Mazzini J, Barton MD Jr, Conway SA, Temple HT
J Bone Joint Surg Am 2015 Apr 1;97(7):597-603. doi: 10.2106/JBJS.N.00649. PMID: 25834085
Superficial or cutaneous malignant peripheral nerve sheath tumor--clinical experience at Taipei Veterans General Hospital.
Feng CJ, Ma H, Liao WC
Ann Plast Surg 2015 May;74 Suppl 2:S85-8. doi: 10.1097/SAP.0000000000000465. PMID: 25695445
Intravascular schwannoma.
Gaudi S, Mills O, Goyette EF, Morgan MB
Am J Dermatopathol 2011 Dec;33(8):850-4. doi: 10.1097/DAD.0b013e3181f5df90. PMID: 22042258
Glandular peripheral nerve sheath tumors.
Woodruff JM, Christensen WN
Cancer 1993 Dec 15;72(12):3618-28. doi: 10.1002/1097-0142(19931215)72:12<3618::aid-cncr2820721212>3.0.co;2-#. PMID: 8252477

Clinical prediction guides

Incidence of second malignancies in individuals diagnosed with malignant peripheral nerve sheath tumors.
Williams LA, Moertel CL, Richardson M, Marcotte EL
J Neurooncol 2020 May;147(3):701-709. Epub 2020 Apr 1 doi: 10.1007/s11060-020-03478-9. PMID: 32239432Free PMC Article
Cancer and Central Nervous System Tumor Surveillance in Pediatric Neurofibromatosis 2 and Related Disorders.
Evans DGR, Salvador H, Chang VY, Erez A, Voss SD, Druker H, Scott HS, Tabori U
Clin Cancer Res 2017 Jun 15;23(12):e54-e61. doi: 10.1158/1078-0432.CCR-17-0590. PMID: 28620005
Clinicopathological features of peripheral nerve sheath tumors involving the eye and ocular adnexa.
Zhang ML, Suarez MJ, Bosley TM, Rodriguez FJ
Hum Pathol 2017 May;63:70-78. Epub 2017 Feb 22 doi: 10.1016/j.humpath.2017.02.006. PMID: 28235631Free PMC Article
An Update on Neurofibromatosis Type 1: Not Just Café-au-Lait Spots and Freckling. Part II. Other Skin Manifestations Characteristic of NF1. NF1 and Cancer.
Hernández-Martín A, Duat-Rodríguez A
Actas Dermosifiliogr 2016 Jul-Aug;107(6):465-73. Epub 2016 Mar 5 doi: 10.1016/j.ad.2016.01.009. PMID: 26956402
Intravascular schwannoma.
Gaudi S, Mills O, Goyette EF, Morgan MB
Am J Dermatopathol 2011 Dec;33(8):850-4. doi: 10.1097/DAD.0b013e3181f5df90. PMID: 22042258

Recent systematic reviews

Impact of CT and MRI in the diagnostic workup of malignant triton tumour-a monocentric analysis and review of the literature.
Drews MA, Milosevic A, Hamacher R, Grüneisen JS, Haubold J, Opitz MK, Bauer S, Umutlu L, Forsting M, Schaarschmidt BM
Br J Radiol 2024 Feb 2;97(1154):430-438. doi: 10.1093/bjr/tqad035. PMID: 38308031
Programmed cell death 1 protein and programmed death-ligand 1 inhibitors in the treatment of nonmelanoma skin cancer: A systematic review.
Choi FD, Kraus CN, Elsensohn AN, Carley SK, Lehmer LM, Nguyen RT, Linden KG, Shiu J
J Am Acad Dermatol 2020 Feb;82(2):440-459. Epub 2019 Jun 1 doi: 10.1016/j.jaad.2019.05.077. PMID: 31163235

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