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Extrarenal Rhabdoid Tumor of the Liver

MedGen UID:
474709
•Concept ID:
C3273076
•
Neoplastic Process
Synonym: Extrarenal Malignant Rhabdoid Tumor of the Liver

Definition

A rare, aggressive primary malignant tumor of the liver that occurs in childhood. It is characterized by the presence of a diffuse infiltrate of undifferentiated cells with rhabdoid features. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVExtrarenal Rhabdoid Tumor of the Liver

Recent clinical studies

Etiology

Expression of WT1 in pediatric small cell tumors: report of two cases with a possible mesothelial origin.
Thorner P, Squire J, Plavsic N, Jong R, Greenberg M, Zielenska M
Pediatr Dev Pathol 1999 Jan-Feb;2(1):33-41. doi: 10.1007/s100249900087. PMID: 9841704
The clinicopathologic spectrum of putative extrarenal rhabdoid tumors. An analysis of 42 cases studied with immunohistochemistry or electron microscopy.
Parham DM, Weeks DA, Beckwith JB
Am J Surg Pathol 1994 Oct;18(10):1010-29. doi: 10.1097/00000478-199410000-00005. PMID: 8092393
Clinical observation of Wilms' tumor.
Lin DT, Hou JW, Lin KH, Chuu WM, Lin KS
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi 1990 Sep-Oct;31(5):299-306. PMID: 2175535

Diagnosis

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
Primary Malignant Rhabdoid Tumor of the Liver in a 5-Month-Old Female Who Presented with Abdominal Distension.
Abu Rass HB, Sweileh MW, Abuawad M
Am J Case Rep 2023 Nov 21;24:e941968. doi: 10.12659/AJCR.941968. PMID: 37988333Free PMC Article
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
Extraordinary disease-free survival in a rare malignant extrarenal rhabdoid tumor: a case report and review of the literature.
D'Amico F, Bertacco A, Cesari M, Mescoli C, Caturegli G, Gondolesi G, Cillo U
J Med Case Rep 2018 Feb 17;12(1):39. doi: 10.1186/s13256-017-1554-2. PMID: 29452605Free PMC Article
Malignant rhabdoid tumor of the small intestine in adults: a brief review of the literature and report of a case.
Voglino C, Scheiterle M, Di Mare G, Mariani F, De Stefano A, Ginori A, De Franco L, Ferrara F
Surg Today 2015 Jul;45(7):924-8. Epub 2014 Jun 25 doi: 10.1007/s00595-014-0955-8. PMID: 24957511

Therapy

Malignant extrarenal rhabdoid tumour (MERT) with liver metastases as a rare cause of an esophageal tumor in a 57 years old patient.
Kaechele V, Vogelpohl J, Boeck W, Riecke A, Eisele R, Barth T
Z Gastroenterol 2015 Jul;53(7):660-3. Epub 2015 Jul 13 doi: 10.1055/s-0034-1399694. PMID: 26167696
Immunohistochemical study as a tool in differential diagnosis of pediatric malignant rhabdoid tumor.
Machado I, Noguera R, Santonja N, Donat J, Fernandez-Delgado R, Acevedo A, Baragaño M, Navarro S
Appl Immunohistochem Mol Morphol 2010 Mar;18(2):150-8. doi: 10.1097/PAI.0b013e3181b91a51. PMID: 19770707

Prognosis

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
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
Extraordinary disease-free survival in a rare malignant extrarenal rhabdoid tumor: a case report and review of the literature.
D'Amico F, Bertacco A, Cesari M, Mescoli C, Caturegli G, Gondolesi G, Cillo U
J Med Case Rep 2018 Feb 17;12(1):39. doi: 10.1186/s13256-017-1554-2. PMID: 29452605Free PMC Article
Malignant rhabdoid tumor of the small intestine in adults: a brief review of the literature and report of a case.
Voglino C, Scheiterle M, Di Mare G, Mariani F, De Stefano A, Ginori A, De Franco L, Ferrara F
Surg Today 2015 Jul;45(7):924-8. Epub 2014 Jun 25 doi: 10.1007/s00595-014-0955-8. PMID: 24957511
Renal and extrarenal rhabdoid tumors in children: a clinicopathologic study of 14 patients.
Sotelo-Avila C, Gonzalez-Crussi F, deMello D, Vogler C, Gooch WM 3rd, Gale G, Pena R
Semin Diagn Pathol 1986 May;3(2):151-63. PMID: 3616219

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
Epithelioid leiomyosarcoma with rhabdoid features.
Yorulmaz G, Erdogan G, Pestereli HE, Savas B, Karaveli FS
Wien Klin Wochenschr 2007;119(17-18):557-60. doi: 10.1007/s00508-007-0822-3. PMID: 17943409
Clinical observation of Wilms' tumor.
Lin DT, Hou JW, Lin KH, Chuu WM, Lin KS
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi 1990 Sep-Oct;31(5):299-306. PMID: 2175535

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