U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Malignant Peripheral Nerve Sheath Tumor with Mesenchymal Differentiation

MedGen UID:
457456
•Concept ID:
C2985230
•
Neoplastic Process

Definition

A malignant peripheral nerve sheath tumor characterized by the presence of mesenchymal differentiation. Representative example is the malignant Triton tumor which contains a rhabdomyosarcomatous component. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMalignant Peripheral Nerve Sheath Tumor with Mesenchymal Differentiation

Professional guidelines

PubMed

NY-ESO-1 expression in synovial sarcoma and other mesenchymal tumors: significance for NY-ESO-1-based targeted therapy and differential diagnosis.
Lai JP, Robbins PF, Raffeld M, Aung PP, Tsokos M, Rosenberg SA, Miettinen MM, Lee CC
Mod Pathol 2012 Jun;25(6):854-8. Epub 2012 Mar 2 doi: 10.1038/modpathol.2012.31. PMID: 22388761Free PMC Article

Recent clinical studies

Etiology

ALK Immunoexpression is Specific for Inflammatory Myofibroblastic Tumor Among Vulvovaginal Mesenchymal Neoplasms.
Bowman CJ, Medeiros F, Fadare O, Sangoi AR, Horvai AE, Devine WP, McCluggage WG, Rabban JT
Int J Gynecol Pathol 2023 Jan 1;42(1):1-10. Epub 2022 Feb 18 doi: 10.1097/PGP.0000000000000858. PMID: 35180768
Mesenchymal Neoplasms of Salivary Glands: A Clinicopathologic Study of 68 Cases.
Velez Torres JM, Duarte EM, Diaz-Perez JA, Leibowitz J, Weed DT, Thomas G, Sargi Z, Civantos FJ, Arnold DJ, Gomez-Fernandez C, Montgomery EA, Rosenberg AE
Head Neck Pathol 2022 Jun;16(2):353-365. Epub 2021 Jul 12 doi: 10.1007/s12105-021-01360-2. PMID: 34251596Free PMC Article
Malignant epithelioid peripheral nerve sheath tumor with prominent reticular/microcystic pattern in a child: a low-grade neoplasm with 18-years follow-up.
Agaimy A, Stachel KD, Jüngert J, Radkow T, Carbon R, Metzler M, Holter W
Appl Immunohistochem Mol Morphol 2014 Sep;22(8):627-33. doi: 10.1097/PAI.0b013e318224751f. PMID: 21768875
Immunohistochemical expression and cluster analysis of mesenchymal and neural stem cell-associated proteins in pediatric soft tissue sarcomas.
Sadikovic B, Graham C, Ho M, Zielenska M, Somers GR
Pediatr Dev Pathol 2011 Jul-Aug;14(4):259-72. Epub 2010 Dec 16 doi: 10.2350/10-08-0890-OA.1. PMID: 21162641
Phosphaturic mesenchymal tumor diagnosed by fine-needle aspiration and core biopsy: a case report and review of literature.
Policarpio-Nicolas ML, Abbott TE, Dalkin AC, Bennett-Wick J, Frierson HF Jr
Diagn Cytopathol 2008 Feb;36(2):115-9. doi: 10.1002/dc.20772. PMID: 18181193

Diagnosis

Malignant peripheral nerve sheath tumor of the bladder A case report.
Petracco G, Patriarca C, Spasciani R, Parafioriti A
Pathologica 2019 Dec;111(4):365-368. doi: 10.32074/1591-951X-33-19. PMID: 31965114Free PMC Article
Malignant Peripheral Nerve Sheath Tumor With Divergent Glandular Differentiation.
Miki Y, Thway K
Int J Surg Pathol 2017 Jun;25(4):310-313. Epub 2017 Mar 1 doi: 10.1177/1066896917696749. PMID: 28381143
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
Synovial sarcoma of nerve.
Scheithauer BW, Amrami KK, Folpe AL, Silva AI, Edgar MA, Woodruff JM, Levi AD, Spinner RJ
Hum Pathol 2011 Apr;42(4):568-77. Epub 2011 Feb 4 doi: 10.1016/j.humpath.2010.08.019. PMID: 21295819
Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation (malignant triton tumor).
Stasik CJ, Tawfik O
Arch Pathol Lab Med 2006 Dec;130(12):1878-81. doi: 10.5858/2006-130-1878-MPNSTW. PMID: 17149968

Therapy

A Case of Inv(1)(q23q31) TPR-NTRK1 Fusion-Positive Spindle Cell Neoplasm in an Infant-Uncovered by Next-Generation Sequencing: Diagnostic Challenge, Review, and Therapeutic Implications.
Rekhi B, Shetty O, Bapat P, Gurav M, Qureshi S
Int J Surg Pathol 2021 Feb;29(1):102-108. Epub 2020 Jun 3 doi: 10.1177/1066896920927467. PMID: 32489127
Grade 4 unclassified renal cell carcinoma with sarcomatoid component expressing S-100 protein. A case report with peculiar diagnostic and therapeutic implications.
Miolo G, Ash A, Buonadonna A, Lo Re G, Torrisi E, Cervo S, Santeufemia DA, Tuzi A, Canzonieri V
Cancer Biol Ther 2014;15(11):1439-43. doi: 10.4161/15384047.2014.956642. PMID: 25482943Free PMC Article
NY-ESO-1 expression in synovial sarcoma and other mesenchymal tumors: significance for NY-ESO-1-based targeted therapy and differential diagnosis.
Lai JP, Robbins PF, Raffeld M, Aung PP, Tsokos M, Rosenberg SA, Miettinen MM, Lee CC
Mod Pathol 2012 Jun;25(6):854-8. Epub 2012 Mar 2 doi: 10.1038/modpathol.2012.31. PMID: 22388761Free PMC Article

Prognosis

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
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
Synovial sarcoma of nerve.
Scheithauer BW, Amrami KK, Folpe AL, Silva AI, Edgar MA, Woodruff JM, Levi AD, Spinner RJ
Hum Pathol 2011 Apr;42(4):568-77. Epub 2011 Feb 4 doi: 10.1016/j.humpath.2010.08.019. PMID: 21295819
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
Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation (malignant triton tumor).
Stasik CJ, Tawfik O
Arch Pathol Lab Med 2006 Dec;130(12):1878-81. doi: 10.5858/2006-130-1878-MPNSTW. PMID: 17149968

Clinical prediction guides

CDX2 expression in malignant peripheral nerve sheath tumour: a potential diagnostic pitfall associated with PRC2 inactivation.
Odeyemi OO, Ozawa MG, Charville GW
Histopathology 2022 May;80(6):995-1000. Epub 2022 Feb 28 doi: 10.1111/his.14626. PMID: 35122289Free PMC Article
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
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
Malignant peripheral nerve sheath tumor of the nasal cavity and nasopharynx in a child: A case report.
Li Q, Pan H, Li L, Cao J
Medicine (Baltimore) 2019 Jan;98(4):e14223. doi: 10.1097/MD.0000000000014223. PMID: 30681599Free PMC Article
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

Supplemental Content

Table of contents

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

    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...