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Epithelioid malignant peripheral nerve sheath tumor

MedGen UID:
272104
Concept ID:
C1321427
Neoplastic Process
Synonyms: Epithelioid malignant nerve sheath tumor; Epithelioid Malignant Peripheral Nerve Sheath Tumor; epithelioid malignant peripheral nerve sheath tumor; Epithelioid MPNST; epithelioid MPNST; Malignant Epithelioid Neoplasm of Peripheral Nerve Sheath; malignant epithelioid neoplasm of peripheral nerve sheath; Malignant Epithelioid Neoplasm of the Peripheral Nerve Sheath; malignant epithelioid neoplasm of the peripheral nerve sheath; Malignant Epithelioid Peripheral Nerve Sheath Neoplasm; malignant epithelioid peripheral nerve sheath neoplasm; Malignant Epithelioid Peripheral Nerve Sheath Tumor; malignant epithelioid peripheral nerve sheath tumor; malignant epithelioid peripheral nerve sheath tumour; Malignant Epithelioid Tumor of Peripheral Nerve Sheath; malignant epithelioid tumor of peripheral nerve sheath; Malignant Epithelioid Tumor of the Peripheral Nerve Sheath; malignant epithelioid tumor of the peripheral nerve sheath; malignant epithelioid tumour of peripheral nerve sheath; malignant epithelioid tumour of the peripheral nerve sheath; Malignant peripheral nerve sheath tumor, epithelioid
SNOMED CT: Epithelioid malignant nerve sheath tumor (404038007); Malignant peripheral nerve sheath tumor, epithelioid (253093000); Epithelioid MPNST (253093000); Epithelioid malignant nerve sheath tumor (253093000); Epithelioid malignant peripheral nerve sheath tumor (404038007)
 
Monarch Initiative: MONDO:0004540

Definition

A rare variant of malignant peripheral nerve sheath tumor composed predominantly or exclusively of epithelioid cells. [from NCI]

Professional guidelines

PubMed

Rutkowski PL, Mullen JT
J Surg Oncol 2018 Jan;117(1):79-86. Epub 2017 Nov 11 doi: 10.1002/jso.24893. PMID: 29127695
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
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

Gözdaşoğlu S, Pamir A, Ünal E, Gökçora İH, Uluoğlu Ö, Ceyhan K, Deda H, Işıkman E, Yavuz G, Taçyıldız N, Çavdar A
Turk J Haematol 2016 Mar 5;33(1):66-70. Epub 2015 Aug 6 doi: 10.4274/tjh.2015.0027. PMID: 26377258Free PMC Article
Jo VY, Fletcher CD
Am J Surg Pathol 2015 May;39(5):673-82. doi: 10.1097/PAS.0000000000000379. PMID: 25602794
McMenamin ME, Fletcher CD
Am J Surg Pathol 2001 Jan;25(1):13-25. doi: 10.1097/00000478-200101000-00002. PMID: 11145248
Guillou L, Wadden C, Coindre JM, Krausz T, Fletcher CD
Am J Surg Pathol 1997 Feb;21(2):130-46. doi: 10.1097/00000478-199702000-00002. PMID: 9042279

Diagnosis

Li S, Hong R, Wang X, Yang J
Int J Gynaecol Obstet 2023 Nov;163(2):521-527. Epub 2023 May 7 doi: 10.1002/ijgo.14831. PMID: 37151162
Yamashita K, Funauchi Y, Hayakawa K, Ae K, Matsumoto S, Ikuta K, Nishida Y, Ueno T, Shimoyama Y, Hiruta N, Machinami R, Kawachi H, Takeuchi K
Virchows Arch 2022 Jun;480(6):1269-1275. Epub 2021 Oct 12 doi: 10.1007/s00428-021-03218-y. PMID: 34635937
Schaefer IM, Al-Ibraheemi A, Qian X
Am J Clin Pathol 2021 Jul 6;156(2):229-245. doi: 10.1093/ajcp/aqaa223. PMID: 33608696Free PMC Article
Pawel BR
Pediatr Dev Pathol 2018 Jan-Feb;21(1):6-28. Epub 2017 Dec 27 doi: 10.1177/1093526617749671. PMID: 29280680
James AW, Dry SM
Surg Pathol Clin 2015 Sep;8(3):309-29. doi: 10.1016/j.path.2015.05.002. PMID: 26297059

Therapy

Gözdaşoğlu S, Pamir A, Ünal E, Gökçora İH, Uluoğlu Ö, Ceyhan K, Deda H, Işıkman E, Yavuz G, Taçyıldız N, Çavdar A
Turk J Haematol 2016 Mar 5;33(1):66-70. Epub 2015 Aug 6 doi: 10.4274/tjh.2015.0027. PMID: 26377258Free PMC Article

Prognosis

Li S, Hong R, Wang X, Yang J
Int J Gynaecol Obstet 2023 Nov;163(2):521-527. Epub 2023 May 7 doi: 10.1002/ijgo.14831. PMID: 37151162
Pawel BR
Pediatr Dev Pathol 2018 Jan-Feb;21(1):6-28. Epub 2017 Dec 27 doi: 10.1177/1093526617749671. PMID: 29280680
James AW, Dry SM
Surg Pathol Clin 2015 Sep;8(3):309-29. doi: 10.1016/j.path.2015.05.002. PMID: 26297059
Jo VY, Fletcher CD
Am J Surg Pathol 2015 May;39(5):673-82. doi: 10.1097/PAS.0000000000000379. PMID: 25602794
Folpe AL
Mod Pathol 2014 Jan;27 Suppl 1:S64-79. doi: 10.1038/modpathol.2013.175. PMID: 24384854

Clinical prediction guides

Schaefer IM, Hornick JL
Semin Diagn Pathol 2021 May;38(3):222-231. Epub 2020 Jun 5 doi: 10.1053/j.semdp.2020.05.005. PMID: 32646614Free PMC Article
Jo VY, Fletcher CDM
Am J Surg Pathol 2017 Aug;41(8):1013-1022. doi: 10.1097/PAS.0000000000000849. PMID: 28368924
Jo VY, Fletcher CD
Am J Surg Pathol 2015 May;39(5):673-82. doi: 10.1097/PAS.0000000000000379. PMID: 25602794
Folpe AL
Mod Pathol 2014 Jan;27 Suppl 1:S64-79. doi: 10.1038/modpathol.2013.175. PMID: 24384854
Tsuchiya D, Takamura H, Saito K, Kashiwa H, Maeda K, Yamashita H
Jpn J Ophthalmol 2004 Nov-Dec;48(6):565-9. doi: 10.1007/s10384-004-0121-8. PMID: 15592781

Recent systematic reviews

Patra S, Ayyanar P, Padhi S, Purkait S, Muduly DK, Samal SC
Am J Case Rep 2019 Aug 10;20:1175-1181. doi: 10.12659/AJCR.916251. PMID: 31399553Free PMC Article

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