U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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

Management of the "Other" retroperitoneal sarcomas.
Rutkowski PL, Mullen JT
J Surg Oncol 2018 Jan;117(1):79-86. Epub 2017 Nov 11 doi: 10.1002/jso.24893. PMID: 29127695
Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 2.
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
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

Therapy

Secondary Neoplasms in Children with Hodgkin's Lymphoma Receiving C-MOPP and Radiotherapy: Presentation of Four Cases.
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

Ovarian epithelioid malignant peripheral nerve sheath tumor with EWSR1-CREM fusion: A case report and literature review.
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
SMARCB1-deficient Tumors of Childhood: A Practical Guide.
Pawel BR
Pediatr Dev Pathol 2018 Jan-Feb;21(1):6-28. Epub 2017 Dec 27 doi: 10.1177/1093526617749671. PMID: 29280680
Diagnostically Challenging Epithelioid Soft Tissue Tumors.
James AW, Dry SM
Surg Pathol Clin 2015 Sep;8(3):309-29. doi: 10.1016/j.path.2015.05.002. PMID: 26297059
Epithelioid malignant peripheral nerve sheath tumor: clinicopathologic analysis of 63 cases.
Jo VY, Fletcher CD
Am J Surg Pathol 2015 May;39(5):673-82. doi: 10.1097/PAS.0000000000000379. PMID: 25602794
Selected topics in the pathology of epithelioid soft tissue tumors.
Folpe AL
Mod Pathol 2014 Jan;27 Suppl 1:S64-79. doi: 10.1038/modpathol.2013.175. PMID: 24384854

Clinical prediction guides

SWI/SNF complex-deficient soft tissue neoplasms: An update.
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
SMARCB1/INI1 Loss in Epithelioid Schwannoma: A Clinicopathologic and Immunohistochemical Study of 65 Cases.
Jo VY, Fletcher CDM
Am J Surg Pathol 2017 Aug;41(8):1013-1022. doi: 10.1097/PAS.0000000000000849. PMID: 28368924
Epithelioid malignant peripheral nerve sheath tumor: clinicopathologic analysis of 63 cases.
Jo VY, Fletcher CD
Am J Surg Pathol 2015 May;39(5):673-82. doi: 10.1097/PAS.0000000000000379. PMID: 25602794
Selected topics in the pathology of epithelioid soft tissue tumors.
Folpe AL
Mod Pathol 2014 Jan;27 Suppl 1:S64-79. doi: 10.1038/modpathol.2013.175. PMID: 24384854
"Proximal-type" epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Clinicopathologic, immunohistochemical, and ultrastructural study of a series.
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

Recent systematic reviews

Epithelioid Malignant Peripheral Nerve Sheath Tumor (Epithelioid-MPNST) Presenting as Bleeding Rectal Polyp: A Case Report with Systematic Literature Review.
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

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...