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Epithelioid Malignant Peripheral Nerve Sheath Tumor of the Skin

MedGen UID:
1727912
•Concept ID:
C5419748
•
Neoplastic Process
Synonyms: Cutaneous Epithelioid Malignant Peripheral Nerve Sheath Tumor; Dermal Epithelioid Malignant Peripheral Nerve Sheath Tumor; Skin Epithelioid Malignant Peripheral Nerve Sheath Tumor

Definition

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

Term Hierarchy

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

Professional guidelines

PubMed

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

An NTRK-1-associated soft tissue tumor showing distinctive nodules with peripheral accentuation of cellularity.
Czaja R, Layng KV, Barrett A, Hooper P, Alomari A, Warren S
J Cutan Pathol 2023 Apr;50(4):343-348. Epub 2022 Nov 23 doi: 10.1111/cup.14355. PMID: 36335562
Methylation-based classification of benign and malignant peripheral nerve sheath tumors.
Röhrich M, Koelsche C, Schrimpf D, Capper D, Sahm F, Kratz A, Reuss J, Hovestadt V, Jones DT, Bewerunge-Hudler M, Becker A, Weis J, Mawrin C, Mittelbronn M, Perry A, Mautner VF, Mechtersheimer G, Hartmann C, Okuducu AF, Arp M, Seiz-Rosenhagen M, Hänggi D, Heim S, Paulus W, Schittenhelm J, Ahmadi R, Herold-Mende C, Unterberg A, Pfister SM, von Deimling A, Reuss DE
Acta Neuropathol 2016 Jun;131(6):877-87. Epub 2016 Feb 8 doi: 10.1007/s00401-016-1540-6. PMID: 26857854
Epithelioid Schwannomas: An Analysis of 58 Cases Including Atypical Variants.
Hart J, Gardner JM, Edgar M, Weiss SW
Am J Surg Pathol 2016 May;40(5):704-13. doi: 10.1097/PAS.0000000000000589. PMID: 26752543
Cutaneous epithelioid malignant peripheral nerve sheath tumour: a clinicopathological analysis of 11 cases.
Luzar B, Shanesmith R, Ramakrishnan R, Fisher C, Calonje E
Histopathology 2016 Jan;68(2):286-96. Epub 2015 Jul 28 doi: 10.1111/his.12756. PMID: 26096054
Low-affinity nerve growth factor receptor (p75) in dermatofibrosarcoma protuberans and other nonneural tumors: a study of 1,150 tumors and fetal and adult normal tissues.
Fanburg-Smith JC, Miettinen M
Hum Pathol 2001 Sep;32(9):976-83. doi: 10.1053/hupa.2001.27602. PMID: 11567228

Diagnosis

PRAME immunohistochemistry in soft tissue tumors and mimics: a study of 350 cases highlighting its imperfect specificity but potentially useful diagnostic applications.
Cammareri C, Beltzung F, Michal M, Vanhersecke L, Coindre JM, Velasco V, Le Loarer F, Vergier B, Perret R
Virchows Arch 2023 Aug;483(2):145-156. Epub 2023 Jul 21 doi: 10.1007/s00428-023-03606-6. PMID: 37477762
Epithelioid Sarcoma of the Peripheral Nerve: Clinicopathologic Series of Three Cases and Literature Review.
Diaz-Perez JA, Spasic S, Velez-Torres JM, McCarthy EF, Rosenberg AE
Am J Clin Pathol 2021 Apr 26;155(5):729-737. doi: 10.1093/ajcp/aqaa180. PMID: 33247294
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
Methylation-based classification of benign and malignant peripheral nerve sheath tumors.
Röhrich M, Koelsche C, Schrimpf D, Capper D, Sahm F, Kratz A, Reuss J, Hovestadt V, Jones DT, Bewerunge-Hudler M, Becker A, Weis J, Mawrin C, Mittelbronn M, Perry A, Mautner VF, Mechtersheimer G, Hartmann C, Okuducu AF, Arp M, Seiz-Rosenhagen M, Hänggi D, Heim S, Paulus W, Schittenhelm J, Ahmadi R, Herold-Mende C, Unterberg A, Pfister SM, von Deimling A, Reuss DE
Acta Neuropathol 2016 Jun;131(6):877-87. Epub 2016 Feb 8 doi: 10.1007/s00401-016-1540-6. PMID: 26857854
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

Prognosis

Epithelioid malignant peripheral nerve sheath tumour of the ulnar nerve around the elbow: a diagnostic and therapeutic challenge.
Acharya AM, Bhat AK, Pai G M, Bansal E
BMJ Case Rep 2023 Nov 24;16(11) doi: 10.1136/bcr-2023-256900. PMID: 38000811Free PMC Article
Cutaneous soft tissue tumors: diagnostically disorienting epithelioid tumors that are not epithelial, and other perplexing mesenchymal lesions.
Carter CS, Patel RM
Mod Pathol 2020 Jan;33(Suppl 1):66-82. Epub 2019 Nov 4 doi: 10.1038/s41379-019-0387-5. PMID: 31685962
Epithelioid angiosarcoma arising in schwannoma of the kidney: report of the first case and review of the literature.
Iannaci G, Crispino M, Cifarelli P, Montella M, Panarese I, Ronchi A, Russo R, Tremiterra G, Luise R, Sapere P
World J Surg Oncol 2016 Feb 3;14(1):29. doi: 10.1186/s12957-016-0789-5. PMID: 26842370Free PMC Article
Cutaneous epithelioid malignant peripheral nerve sheath tumour: a clinicopathological analysis of 11 cases.
Luzar B, Shanesmith R, Ramakrishnan R, Fisher C, Calonje E
Histopathology 2016 Jan;68(2):286-96. Epub 2015 Jul 28 doi: 10.1111/his.12756. PMID: 26096054
Low-grade malignant perineurioma of the paravertebral column, transforming into a high-grade malignancy.
Karaki S, Mochida J, Lee YH, Nishimura K, Tsutsumi Y
Pathol Int 1999 Sep;49(9):820-5. doi: 10.1046/j.1440-1827.1999.00948.x. PMID: 10504555

Clinical prediction guides

Cutaneous soft tissue tumors: diagnostically disorienting epithelioid tumors that are not epithelial, and other perplexing mesenchymal lesions.
Carter CS, Patel RM
Mod Pathol 2020 Jan;33(Suppl 1):66-82. Epub 2019 Nov 4 doi: 10.1038/s41379-019-0387-5. PMID: 31685962
Cutaneous epithelioid malignant peripheral nerve sheath tumour: a clinicopathological analysis of 11 cases.
Luzar B, Shanesmith R, Ramakrishnan R, Fisher C, Calonje E
Histopathology 2016 Jan;68(2):286-96. Epub 2015 Jul 28 doi: 10.1111/his.12756. PMID: 26096054

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