U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Malignant rhabdoid tumor of liver

MedGen UID:
1841657
•Concept ID:
C5826480
•
Neoplastic Process
HPO: HP:0034561

Definition

A malginant rhabdoid tumor (MRT) originating in the liver. MRT are best characterized by the presence of round or polygonal cells with abundant eosinophilic cytoplasm, typical eosinophilic perinuclear inclusions, vesicular nuclei and prominent nucleoli. Immunohistochemical expression of vimentin and epithelial markers and lack of staining for S-100, myoglobin and desmin are commonly found, but are non-specific. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMalignant rhabdoid tumor of liver

Professional guidelines

PubMed

Current recommendations for clinical surveillance and genetic testing in rhabdoid tumor predisposition: a report from the SIOPE Host Genome Working Group.
Frühwald MC, Nemes K, Boztug H, Cornips MCA, Evans DG, Farah R, Glentis S, Jorgensen M, Katsibardi K, Hirsch S, Jahnukainen K, Kventsel I, Kerl K, Kratz CP, Pajtler KW, Kordes U, Ridola V, Stutz E, Bourdeaut F
Fam Cancer 2021 Oct;20(4):305-316. Epub 2021 Feb 3 doi: 10.1007/s10689-021-00229-1. PMID: 33532948Free PMC Article
Patient-derived mouse xenografts from pediatric liver cancer predict tumor recurrence and advise clinical management.
Nicolle D, Fabre M, Simon-Coma M, Gorse A, Kappler R, Nonell L, Mallo M, Haidar H, Déas O, Mussini C, Guettier C, Redon MJ, Brugières L, Ghigna MR, Fadel E, Galmiche-Rolland L, Chardot C, Judde JG, Armengol C, Branchereau S, Cairo S
Hepatology 2016 Oct;64(4):1121-35. Epub 2016 Jun 16 doi: 10.1002/hep.28621. PMID: 27115099
Preclinical evaluation of engineered oncolytic herpes simplex virus for the treatment of pediatric solid tumors.
Megison ML, Gillory LA, Stewart JE, Nabers HC, Mroczek-Musulman E, Waters AM, Coleman JM, Kelly V, Markert JM, Gillespie GY, Friedman GK, Beierle EA
PLoS One 2014;9(1):e86843. Epub 2014 Jan 30 doi: 10.1371/journal.pone.0086843. PMID: 24497984Free PMC Article

Recent clinical studies

Etiology

Extracranial rhabdoid tumours: Results of a SFCE series of patients treated with a dose compression strategy according to European Paediatric Soft tisue sarcoma Study Group recommendations.
Enault M, Minard-Colin V, Corradini N, Leverger G, Thebaud E, Rome A, Proust S, Marie-Cardine A, Defachelles AS, Sarnacki S, Philippe-Chomette P, Delattre O, Masliah-Planchon J, Lacour B, Ferrari A, Brennan B, Orbach D, Bourdeaut F
Eur J Cancer 2022 Jan;161:64-78. Epub 2021 Dec 18 doi: 10.1016/j.ejca.2021.10.025. PMID: 34929472
Liver Tumors in Pediatric Patients.
Meyers R, Hiyama E, Czauderna P, Tiao GM
Surg Oncol Clin N Am 2021 Apr;30(2):253-274. doi: 10.1016/j.soc.2020.11.006. PMID: 33706899
Small cell undifferentiated (SCUD) hepatoblastomas: All malignant rhabdoid tumors?
Vokuhl C, Oyen F, Häberle B, von Schweinitz D, Schneppenheim R, Leuschner I
Genes Chromosomes Cancer 2016 Dec;55(12):925-931. Epub 2016 Jul 29 doi: 10.1002/gcc.22390. PMID: 27356182
Primary malignant liver tumors in children.
Agarwala S
Indian J Pediatr 2012 Jun;79(6):793-800. Epub 2012 Mar 1 doi: 10.1007/s12098-012-0704-1. PMID: 22382512
Liver tumors in children.
Litten JB, Tomlinson GE
Oncologist 2008 Jul;13(7):812-20. Epub 2008 Jul 21 doi: 10.1634/theoncologist.2008-0011. PMID: 18644850

Diagnosis

Mesenchymal Neoplasms of the Liver.
Papke DJ Jr
Surg Pathol Clin 2023 Sep;16(3):609-634. Epub 2023 Jun 11 doi: 10.1016/j.path.2023.04.013. PMID: 37536892
Liver Tumors in Pediatric Patients.
Meyers R, Hiyama E, Czauderna P, Tiao GM
Surg Oncol Clin N Am 2021 Apr;30(2):253-274. doi: 10.1016/j.soc.2020.11.006. PMID: 33706899
Rhabdoid tumors of the liver: rare, aggressive, and poorly responsive to standard cytotoxic chemotherapy.
Trobaugh-Lotrario AD, Finegold MJ, Feusner JH
Pediatr Blood Cancer 2011 Sep;57(3):423-8. Epub 2010 Nov 5 doi: 10.1002/pbc.22857. PMID: 21744471
Liver tumors in children.
Litten JB, Tomlinson GE
Oncologist 2008 Jul;13(7):812-20. Epub 2008 Jul 21 doi: 10.1634/theoncologist.2008-0011. PMID: 18644850
Tumors of the liver in children.
Meyers RL
Surg Oncol 2007 Nov;16(3):195-203. Epub 2007 Aug 21 doi: 10.1016/j.suronc.2007.07.002. PMID: 17714939

Therapy

Rhabdoid tumor of the liver: Report of 6 pediatric cases treated at a single institute.
Cornet M, De Lambert G, Pariente D, Planchon JM, Guettier C, Martelli H, Guérin F, Branchereau S
J Pediatr Surg 2018 Mar;53(3):567-571. Epub 2017 Sep 14 doi: 10.1016/j.jpedsurg.2017.09.005. PMID: 28966010
Patient-derived mouse xenografts from pediatric liver cancer predict tumor recurrence and advise clinical management.
Nicolle D, Fabre M, Simon-Coma M, Gorse A, Kappler R, Nonell L, Mallo M, Haidar H, Déas O, Mussini C, Guettier C, Redon MJ, Brugières L, Ghigna MR, Fadel E, Galmiche-Rolland L, Chardot C, Judde JG, Armengol C, Branchereau S, Cairo S
Hepatology 2016 Oct;64(4):1121-35. Epub 2016 Jun 16 doi: 10.1002/hep.28621. PMID: 27115099
Malignant rhabdoid tumor of the liver presented with initial tumor rupture.
Kachanov D, Teleshova M, Kim E, Dobrenkov K, Moiseenko R, Usychkina A, Filin A, Semenkov A, Mitrofanova A, Konovalov D, Shamanskaya T, Novichkova G, Varfolomeeva S
Cancer Genet 2014 Sep;207(9):412-4. Epub 2014 Apr 21 doi: 10.1016/j.cancergen.2014.04.006. PMID: 24894493
Rhabdoid tumors of the liver: rare, aggressive, and poorly responsive to standard cytotoxic chemotherapy.
Trobaugh-Lotrario AD, Finegold MJ, Feusner JH
Pediatr Blood Cancer 2011 Sep;57(3):423-8. Epub 2010 Nov 5 doi: 10.1002/pbc.22857. PMID: 21744471
Malignant rhabdoid tumor of the liver: case report and literature review.
Yuri T, Danbara N, Shikata N, Fujimoto S, Nakano T, Sakaida N, Uemura Y, Tsubura A
Pathol Int 2004 Aug;54(8):623-9. doi: 10.1111/j.1440-1827.2004.01672.x. PMID: 15260854

Prognosis

Malignant rhabdoid tumor of the liver in a middle-aged woman: a case report and literature review.
Ye H, Liu Z, Zhang Y
BMC Gastroenterol 2022 Jan 21;22(1):28. doi: 10.1186/s12876-022-02102-6. PMID: 35062870Free PMC Article
Malignant Rhabdoid Tumor of Soft Tissue.
Guilmette J, Laverdière C, Soulières D, Patey N, Soucy G, Trudel D, Bouron-Dal Soglio D
Pediatr Dev Pathol 2017 Jun;20(3):262-266. Epub 2017 Apr 21 doi: 10.1177/1093526617706814. PMID: 28429647
Cytogenetics of hepatoblastoma.
Tomlinson GE
Front Biosci (Elite Ed) 2012 Jan 1;4(4):1287-92. doi: 10.2741/459. PMID: 22201954
Rhabdoid tumors of the liver: rare, aggressive, and poorly responsive to standard cytotoxic chemotherapy.
Trobaugh-Lotrario AD, Finegold MJ, Feusner JH
Pediatr Blood Cancer 2011 Sep;57(3):423-8. Epub 2010 Nov 5 doi: 10.1002/pbc.22857. PMID: 21744471
Nestin in gastrointestinal and other cancers: effects on cells and tumor angiogenesis.
Ishiwata T, Matsuda Y, Naito Z
World J Gastroenterol 2011 Jan 28;17(4):409-18. doi: 10.3748/wjg.v17.i4.409. PMID: 21274370Free PMC Article

Clinical prediction guides

Primary extrarenal rhabdoid tumour of the liver: a case report and literature review.
Meyers Michel, Demetter Pieter, De Roo An-Katrien, Pezzullo Martina, Jungels Christiane, Brichard Bénédicte, De Magnee Catherine, De Krijger Ronald R, Verset Gontran
Acta Gastroenterol Belg 2023 Oct-Dec;86(4):555-562. doi: 10.51821/86.4.11504. PMID: 38240550
MIR17HG: A Cancerogenic Long-Noncoding RNA in Different Cancers.
Tan F, Chen J, Du Z, Zhao F, Liu Y, Zhang Q, Yuan C
Curr Pharm Des 2022;28(15):1272-1281. doi: 10.2174/1381612828666220310144500. PMID: 35272588
Molecular features of primary hepatic undifferentiated carcinoma.
Tsai JH, Jeng YM, Lee CH, Liau JY
Mod Pathol 2022 May;35(5):680-687. Epub 2021 Dec 23 doi: 10.1038/s41379-021-00970-z. PMID: 34949765
Patient-derived mouse xenografts from pediatric liver cancer predict tumor recurrence and advise clinical management.
Nicolle D, Fabre M, Simon-Coma M, Gorse A, Kappler R, Nonell L, Mallo M, Haidar H, Déas O, Mussini C, Guettier C, Redon MJ, Brugières L, Ghigna MR, Fadel E, Galmiche-Rolland L, Chardot C, Judde JG, Armengol C, Branchereau S, Cairo S
Hepatology 2016 Oct;64(4):1121-35. Epub 2016 Jun 16 doi: 10.1002/hep.28621. PMID: 27115099
Cytogenetics of hepatoblastoma.
Tomlinson GE
Front Biosci (Elite Ed) 2012 Jan 1;4(4):1287-92. doi: 10.2741/459. PMID: 22201954

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

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...