U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Malignant triton tumor

MedGen UID:
90816
•Concept ID:
C0334616
•
Neoplastic Process
Synonyms: Malignant Neoplasm of Peripheral Nerve Sheath with Rhabdomyosarcoma; malignant neoplasm of peripheral nerve sheath with rhabdomyosarcoma; Malignant Neoplasm of the Peripheral Nerve Sheath with Rhabdomyosarcoma; malignant neoplasm of the peripheral nerve sheath with rhabdomyosarcoma; Malignant neurilemmoma with rhabdomyoblastic differentiation; Malignant Peripheral Nerve Sheath Neoplasm with Rhabdomyosarcoma; malignant peripheral nerve sheath neoplasm with rhabdomyosarcoma; Malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation; Malignant Peripheral Nerve Sheath Tumor with Rhabdomyosarcoma; malignant peripheral nerve sheath tumor with rhabdomyosarcoma; Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differenciation; malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differenciation; Malignant schwannoma with rhabdomyoblastic differentiation; Malignant Triton tumor; Malignant Triton Tumor; malignant triton tumor; malignant Triton tumour; Malignant Tumor of Peripheral Nerve Sheath with Rhabdomyosarcoma; malignant tumor of peripheral nerve sheath with rhabdomyosarcoma; Malignant Tumor of the Peripheral Nerve Sheath with Rhabdomyosarcoma; malignant tumor of the peripheral nerve sheath with rhabdomyosarcoma; MPNST with rhabdomyoblastic differentiation; MPNST with Rhabdomyosarcoma; MPNST with rhabdomyosarcoma; MPNST with rhabdomyosarcomatous differentiation; MTT; Triton tumor, malignant
SNOMED CT: Triton tumor, malignant (354002); Malignant Triton tumor (404040002); Malignant schwannoma with rhabdomyoblastic differentiation (354002); MPNST with rhabdomyoblastic differentiation (354002); Malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation (354002); Malignant neurilemmoma with rhabdomyoblastic differentiation (354002)
 
Monarch Initiative: MONDO:0016757
Orphanet: ORPHA252212

Definition

Malignant triton tumor (MTT) is a rare aggressive subtype of malignant peripheral nerve sheath tumor (MPNST; see this term) characterized histopathologically by focal rhabdomyoblastic differentiation. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMalignant triton tumor
Follow this link to review classifications for Malignant triton tumor in Orphanet.

Professional guidelines

PubMed

Primary and Secondary Prevention of Lung Cancer: Tobacco Treatment.
Kathuria H, Neptune E
Clin Chest Med 2020 Mar;41(1):39-51. doi: 10.1016/j.ccm.2019.10.002. PMID: 32008628
Microneurosurgical treatment options in peripheral nerve compression syndromes after chemotherapy and radiation treatment.
Chang EI, Rose MI, Rossi K, Elkwood AI
J Surg Oncol 2018 Oct;118(5):793-799. Epub 2018 Sep 27 doi: 10.1002/jso.25254. PMID: 30261113
Treatment of tobacco dependence: current state of the art.
Kathuria H, Leone FT, Neptune ER
Curr Opin Pulm Med 2018 Jul;24(4):327-334. doi: 10.1097/MCP.0000000000000491. PMID: 29677028

Recent clinical studies

Etiology

A rare case of intracranial malignant triton tumor arising in the middle cranial fossa: a case report and review of the literature.
Bruzzone E, Melloni I, Barra S, Fraternali Orcioni G, Cocito L
Folia Neuropathol 2018;56(3):229-234. doi: 10.5114/fn.2018.78704. PMID: 30509044
Optimal surgical treatment and urological outcomes in boys with pelvic and urogenital rhabdomyosarcomas and soft tissue sarcomas.
Hishiki T, Saito T, Mitsunaga T, Nakata M, Terui E, Komatsu S, Mise N, Harada K, Iwai J, Higashimoto Y, Okimoto Y, Kakuda H, Ochiai H, Hino M, Homma S, Osa Y, Yoshida H
Pediatr Surg Int 2013 Oct;29(10):1077-82. doi: 10.1007/s00383-013-3400-y. PMID: 23989524
Malignant triton tumor in a patient with Li-Fraumeni syndrome and a novel TP53 mutation.
Chao MM, Levine JE, Ruiz RE, Kohlmann WK, Bower MA, Petty EM, Mody RJ
Pediatr Blood Cancer 2007 Dec;49(7):1000-4. doi: 10.1002/pbc.20700. PMID: 16333835
Malignant triton tumor of the lumbar spine.
Yimaz MR, Bek S, Bekmezci T, Gökduman C, Solak A AS
Spine (Phila Pa 1976) 2004 Sep 15;29(18):E399-401. doi: 10.1097/01.brs.0000138308.56051.c7. PMID: 15371720
Non-germ-cell or teratomatous malignant tumors showing additional rhabdomyoblastic differentiation, with emphasis on the malignant Triton tumor.
Woodruff JM, Perino G
Semin Diagn Pathol 1994 Feb;11(1):69-81. PMID: 8202648

Diagnosis

Triton tumor of the orbit.
Barh A, Mukherjee B, Koka K, Krishnakumar S
Orbit 2020 Dec;39(6):418-421. Epub 2019 Nov 20 doi: 10.1080/01676830.2019.1692873. PMID: 31746248
Primary epididymis malignant triton tumor: case report and review of the literature.
Gao L, Song H, Mu K, Wang J, Guo B, Shi B, Li G
Eur J Med Res 2015 Sep 21;20(1):79. doi: 10.1186/s40001-015-0172-y. PMID: 26392076Free PMC Article
Malignant triton tumor of the cervical spine: report of one case and review of the literature.
Jaing TH, Chuang CC, Jung SM, Wu CT, Tseng CK, Chen CS
Pediatr Neonatol 2015 Feb;56(1):58-61. Epub 2013 Mar 13 doi: 10.1016/j.pedneo.2013.01.013. PMID: 23597523
Malignant triton tumor of the retroperitoneum: a case report and review of the literature.
Li Z, Xiang J, Yan S, Gao F, Zheng S
World J Surg Oncol 2012 May 30;10:96. doi: 10.1186/1477-7819-10-96. PMID: 22647059Free PMC Article
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

Malignant triton tumor of trigeminal nerve-case report.
Mallah FA, Tanrıkulu B, Çorapçıoğlu F, Özek MM
Childs Nerv Syst 2018 May;34(5):983-986. Epub 2017 Dec 26 doi: 10.1007/s00381-017-3708-3. PMID: 29279963
Malignant triton tumor of the gluteal region in a patient unaffected by neurofibromatosis: A case report.
Merter A, Başarır K, Yıldız Y, Sağlık Y
Acta Orthop Traumatol Turc 2018 May;52(3):236-239. Epub 2017 Oct 27 doi: 10.1016/j.aott.2017.07.005. PMID: 29110923Free PMC Article
Malignant Triton Tumor (Malignant Peripheral Nerve Sheath Tumor With Rhabdomyoblastic Differentiation) Occurring in a Vascularized Free Flap Reconstruction Graft.
Ram R, Gardner J, Alapati S, Jambhekar K, Pandey T, Montgomery C, Nicholas R
Int J Surg Pathol 2017 Aug;25(5):462-467. Epub 2017 Apr 7 doi: 10.1177/1066896917700725. PMID: 28387148
Chemotherapy in metastatic malignant triton tumor: report on two cases.
Thoennissen NH, Schliemann C, Brunnberg U, Schmidt E, Staebler A, Stegger L, Bremer C, Schleicher C, Mesters RM, Müller-Tidow C, Berdel WE
Oncol Rep 2007 Oct;18(4):763-7. doi: 10.3892/or.18.4.763. PMID: 17786333
Malignant triton tumor of the head and neck: A case report and review of the literature.
Victoria L, McCulloch TM, Callaghan EJ, Bauman NM
Head Neck 1999 Oct;21(7):663-70. doi: 10.1002/(sici)1097-0347(199910)21:7<663::aid-hed12>3.0.co;2-4. PMID: 10487955

Prognosis

Primary epididymis malignant triton tumor: case report and review of the literature.
Gao L, Song H, Mu K, Wang J, Guo B, Shi B, Li G
Eur J Med Res 2015 Sep 21;20(1):79. doi: 10.1186/s40001-015-0172-y. PMID: 26392076Free PMC Article
Malignant triton tumor of the cervical spine: report of one case and review of the literature.
Jaing TH, Chuang CC, Jung SM, Wu CT, Tseng CK, Chen CS
Pediatr Neonatol 2015 Feb;56(1):58-61. Epub 2013 Mar 13 doi: 10.1016/j.pedneo.2013.01.013. PMID: 23597523
Malignant triton tumor of the retroperitoneum: a case report and review of the literature.
Li Z, Xiang J, Yan S, Gao F, Zheng S
World J Surg Oncol 2012 May 30;10:96. doi: 10.1186/1477-7819-10-96. PMID: 22647059Free PMC Article
Prognostic factors for the malignant triton tumor of the head and neck.
Terzic A, Bode B, Gratz KW, Stoeckli SJ
Head Neck 2009 May;31(5):679-88. doi: 10.1002/hed.21051. PMID: 19283843
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

Computed tomography imaging features of malignant 'triton' tumor to facilitate its clinical diagnosis: report of two cases.
Li Y, Peng Q, Jiang N, Molloy DP, Zeng C, Wu Q
BMC Med Imaging 2022 Jul 14;22(1):125. doi: 10.1186/s12880-022-00848-9. PMID: 35836141Free PMC Article
Clinicopathological and prognostic significance of H3K27 methylation status in malignant peripheral nerve sheath tumor: correlation with skeletal muscle differentiation.
Ito Y, Kohashi K, Endo M, Yoshimoto M, Ishihara S, Toda Y, Susuki Y, Kawaguchi K, Furukawa H, Tateishi Y, Yamada Y, Kinoshita I, Mori T, Yamamoto H, Nakashima Y, Oda Y
Virchows Arch 2021 Dec;479(6):1233-1244. Epub 2021 Aug 25 doi: 10.1007/s00428-021-03189-0. PMID: 34432163
Malignant triton tumor of the gluteal region in a patient unaffected by neurofibromatosis: A case report.
Merter A, Başarır K, Yıldız Y, Sağlık Y
Acta Orthop Traumatol Turc 2018 May;52(3):236-239. Epub 2017 Oct 27 doi: 10.1016/j.aott.2017.07.005. PMID: 29110923Free PMC Article
A case of recurrent malignant triton tumor successfully treated with radiotherapy.
Yasuda M, Muto Y, Kuremoto T, Murakami K, Onisihi T, Koida A, Inui T, Hisa Y
Auris Nasus Larynx 2016 Dec;43(6):710-4. Epub 2016 Apr 15 doi: 10.1016/j.anl.2016.04.003. PMID: 27146005
Novel PAX3-NCOA1 Fusions in Biphenotypic Sinonasal Sarcoma With Focal Rhabdomyoblastic Differentiation.
Huang SC, Ghossein RA, Bishop JA, Zhang L, Chen TC, Huang HY, Antonescu CR
Am J Surg Pathol 2016 Jan;40(1):51-9. doi: 10.1097/PAS.0000000000000492. PMID: 26371783Free 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.
    • 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.

    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...