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Malignant rhabdoid tumor, somatic

MedGen UID:
442465
•Concept ID:
C2750405
•
Neoplastic Process
Synonym: Malignant Rhabdoid Tumor, Somatic
 
OMIM®: 601607; 609322

Professional guidelines

PubMed

Current Management of Fetal and Neonatal Renal Tumors.
Berger M, von Schweinitz D
Curr Pediatr Rev 2015;11(3):188-94. doi: 10.2174/1573396311666150714105403. PMID: 26168945

Recent clinical studies

Etiology

Hereditary ovarian tumour syndromes: current update on genetics and imaging.
Shanbhogue KP, Prasad AS, Ucisik-Keser FE, Katabathina VS, Morani AC
Clin Radiol 2021 Apr;76(4):313.e15-313.e26. Epub 2021 Jan 19 doi: 10.1016/j.crad.2020.11.116. PMID: 33353730
Tumor mutation burden, DNA mismatch repair status and checkpoint immunotherapy markers in primary and relapsed malignant rhabdoid tumors.
Abro B, Kaushal M, Chen L, Wu R, Dehner LP, Pfeifer JD, He M
Pathol Res Pract 2019 Jun;215(6):152395. Epub 2019 Apr 18 doi: 10.1016/j.prp.2019.03.023. PMID: 31047727
Cancer Surveillance in Gorlin Syndrome and Rhabdoid Tumor Predisposition Syndrome.
Foulkes WD, Kamihara J, Evans DGR, Brugières L, Bourdeaut F, Molenaar JJ, Walsh MF, Brodeur GM, Diller L
Clin Cancer Res 2017 Jun 15;23(12):e62-e67. doi: 10.1158/1078-0432.CCR-17-0595. PMID: 28620006Free PMC Article
Immunoreactivity using anti-ERG monoclonal antibodies in sarcomas is influenced by clone selection.
Machado I, Mayordomo-Aranda E, Scotlandi K, Picci P, Llombart-Bosch A
Pathol Res Pract 2014 Aug;210(8):508-13. Epub 2014 Apr 18 doi: 10.1016/j.prp.2014.04.005. PMID: 24906228
PAX immunoreactivity in poorly differentiated small round cell tumors of childhood.
Fan R
Fetal Pediatr Pathol 2014 Aug;33(4):244-52. Epub 2014 Jun 4 doi: 10.3109/15513815.2014.920441. PMID: 24897005

Diagnosis

Rhabdoid tumor predisposition syndrome with renal tumor 10 years after brain tumor.
Fukushima H, Yamasaki K, Sakaida M, Tsujio N, Okuno T, Ishii N, Okada K, Fujisaki H, Matsusaka Y, Sakamoto H, Yoneda A, Hara J, Inoue T
Pathol Int 2021 Feb;71(2):155-160. Epub 2020 Dec 30 doi: 10.1111/pin.13056. PMID: 33378586
Hereditary ovarian tumour syndromes: current update on genetics and imaging.
Shanbhogue KP, Prasad AS, Ucisik-Keser FE, Katabathina VS, Morani AC
Clin Radiol 2021 Apr;76(4):313.e15-313.e26. Epub 2021 Jan 19 doi: 10.1016/j.crad.2020.11.116. PMID: 33353730
Cancer Surveillance in Gorlin Syndrome and Rhabdoid Tumor Predisposition Syndrome.
Foulkes WD, Kamihara J, Evans DGR, Brugières L, Bourdeaut F, Molenaar JJ, Walsh MF, Brodeur GM, Diller L
Clin Cancer Res 2017 Jun 15;23(12):e62-e67. doi: 10.1158/1078-0432.CCR-17-0595. PMID: 28620006Free PMC Article
All things rhabdoid and SMARC: An enigmatic exploration with Dr. Louis P. Dehner.
Fuller CE
Semin Diagn Pathol 2016 Nov;33(6):427-440. Epub 2016 Aug 31 doi: 10.1053/j.semdp.2016.08.003. PMID: 27745735
Current Management of Fetal and Neonatal Renal Tumors.
Berger M, von Schweinitz D
Curr Pediatr Rev 2015;11(3):188-94. doi: 10.2174/1573396311666150714105403. PMID: 26168945

Therapy

A Phase I Study of the CDK4/6 Inhibitor Ribociclib (LEE011) in Pediatric Patients with Malignant Rhabdoid Tumors, Neuroblastoma, and Other Solid Tumors.
Geoerger B, Bourdeaut F, DuBois SG, Fischer M, Geller JI, Gottardo NG, Marabelle A, Pearson ADJ, Modak S, Cash T, Robinson GW, Motta M, Matano A, Bhansali SG, Dobson JR, Parasuraman S, Chi SN
Clin Cancer Res 2017 May 15;23(10):2433-2441. Epub 2017 Apr 21 doi: 10.1158/1078-0432.CCR-16-2898. PMID: 28432176
Synchronous congenital malignant rhabdoid tumor of the orbit and atypical teratoid/rhabdoid tumor--feasibility and efficacy of multimodal therapy in a long-term survivor.
Seeringer A, Reinhard H, Hasselblatt M, Schneppenheim R, Siebert R, Bartelheim K, Leuschner I, Frühwald MC
Cancer Genet 2014 Sep;207(9):429-33. Epub 2014 Jul 3 doi: 10.1016/j.cancergen.2014.06.028. PMID: 25262118

Prognosis

Hereditary ovarian tumour syndromes: current update on genetics and imaging.
Shanbhogue KP, Prasad AS, Ucisik-Keser FE, Katabathina VS, Morani AC
Clin Radiol 2021 Apr;76(4):313.e15-313.e26. Epub 2021 Jan 19 doi: 10.1016/j.crad.2020.11.116. PMID: 33353730
Current Management of Fetal and Neonatal Renal Tumors.
Berger M, von Schweinitz D
Curr Pediatr Rev 2015;11(3):188-94. doi: 10.2174/1573396311666150714105403. PMID: 26168945

Clinical prediction guides

Rhabdoid tumor predisposition syndrome with renal tumor 10 years after brain tumor.
Fukushima H, Yamasaki K, Sakaida M, Tsujio N, Okuno T, Ishii N, Okada K, Fujisaki H, Matsusaka Y, Sakamoto H, Yoneda A, Hara J, Inoue T
Pathol Int 2021 Feb;71(2):155-160. Epub 2020 Dec 30 doi: 10.1111/pin.13056. PMID: 33378586
Tumor mutation burden, DNA mismatch repair status and checkpoint immunotherapy markers in primary and relapsed malignant rhabdoid tumors.
Abro B, Kaushal M, Chen L, Wu R, Dehner LP, Pfeifer JD, He M
Pathol Res Pract 2019 Jun;215(6):152395. Epub 2019 Apr 18 doi: 10.1016/j.prp.2019.03.023. PMID: 31047727
A Phase I Study of the CDK4/6 Inhibitor Ribociclib (LEE011) in Pediatric Patients with Malignant Rhabdoid Tumors, Neuroblastoma, and Other Solid Tumors.
Geoerger B, Bourdeaut F, DuBois SG, Fischer M, Geller JI, Gottardo NG, Marabelle A, Pearson ADJ, Modak S, Cash T, Robinson GW, Motta M, Matano A, Bhansali SG, Dobson JR, Parasuraman S, Chi SN
Clin Cancer Res 2017 May 15;23(10):2433-2441. Epub 2017 Apr 21 doi: 10.1158/1078-0432.CCR-16-2898. PMID: 28432176
SNF5 reexpression in malignant rhabdoid tumors regulates transcription of target genes by recruitment of SWI/SNF complexes and RNAPII to the transcription start site of their promoters.
Kuwahara Y, Wei D, Durand J, Weissman BE
Mol Cancer Res 2013 Mar;11(3):251-60. Epub 2013 Jan 30 doi: 10.1158/1541-7786.MCR-12-0390. PMID: 23364536Free PMC Article

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