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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 peripheral nerve sheath tumour with rhabdomyosarcoma; malignant peripheral nerve sheath tumour with rhabdomyosarcomatous differenciation; Malignant schwannoma with rhabdomyoblastic differentiation; Malignant Triton tumor; 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; malignant tumour of peripheral nerve sheath with rhabdomyosarcoma; malignant tumour 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

NF1-Driven Rhabdomyosarcoma Phenotypes: A Comparative Clinical and Molecular Study of NF1-Mutant Rhabdomyosarcoma and NF1-Associated Malignant Triton Tumor.
de Traux de Wardin H, Dermawan JK, Vanoli F, Jiang SC, Singer S, Chi P, Tap W, Wexler LH, Antonescu CR
JCO Precis Oncol 2024 Apr;8:e2300597. doi: 10.1200/PO.23.00597. PMID: 38603649
Surgical management of craniospinal axis malignant peripheral nerve sheath tumors: a single-institution experience and literature review.
Chowdhury A, Vivanco-Suarez J, Teferi N, Belzer A, Al-Kaylani H, Challa M, Lee S, Buatti JM, Hitchon P
World J Surg Oncol 2023 Oct 26;21(1):338. doi: 10.1186/s12957-023-03227-y. PMID: 37880773Free PMC Article
Loss of H3K27me3 occurs in a large subset of embryonal rhabdomyosarcomas: Immunohistochemical and molecular analysis of 25 cases.
Tomassen T, Kester LA, Tops BB, Driehuis E, van Noesel MM, van Ewijk R, van Gorp JM, Hulsker CC, Terwisscha-van Scheltinga SEJ, Merks HHM, Flucke U, Hiemcke-Jiwa LS
Ann Diagn Pathol 2021 Jun;52:151735. Epub 2021 Mar 22 doi: 10.1016/j.anndiagpath.2021.151735. PMID: 33770660
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
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
A-Z of malignant peripheral nerve sheath tumors.
Kamran SC, Shinagare AB, Howard SA, Hornick JL, Ramaiya NH
Cancer Imaging 2012 Oct 26;12(3):475-83. doi: 10.1102/1470-7330.2012.0043. PMID: 23108260Free PMC Article
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
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 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

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 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

Surgical management of craniospinal axis malignant peripheral nerve sheath tumors: a single-institution experience and literature review.
Chowdhury A, Vivanco-Suarez J, Teferi N, Belzer A, Al-Kaylani H, Challa M, Lee S, Buatti JM, Hitchon P
World J Surg Oncol 2023 Oct 26;21(1):338. doi: 10.1186/s12957-023-03227-y. PMID: 37880773Free PMC Article
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

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