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Advanced Rhabdoid Tumor

MedGen UID:
1732275
•Concept ID:
C5420859
•
Neoplastic Process

Definition

Rhabdoid tumor that has spread extensively to other anatomic sites or is no longer responding to treatment. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdvanced Rhabdoid Tumor

Professional guidelines

PubMed

Epidemiology, treatment and outcomes of primary renal sarcomas in adult patients.
Uhlig J, Uhlig A, Deshpande H, Ströbel P, Trojan L, Lotz J, Hurwitz M, Hafez O, Humphrey P, Grünwald V, Kim HS
Sci Rep 2024 May 2;14(1):10038. doi: 10.1038/s41598-024-60174-8. PMID: 38693188Free PMC Article
New developments in existing WHO entities and evolving molecular concepts: The Genitourinary Pathology Society (GUPS) update on renal neoplasia.
Trpkov K, Hes O, Williamson SR, Adeniran AJ, Agaimy A, Alaghehbandan R, Amin MB, Argani P, Chen YB, Cheng L, Epstein JI, Cheville JC, Comperat E, da Cunha IW, Gordetsky JB, Gupta S, He H, Hirsch MS, Humphrey PA, Kapur P, Kojima F, Lopez JI, Maclean F, Magi-Galluzzi C, McKenney JK, Mehra R, Menon S, Netto GJ, Przybycin CG, Rao P, Rao Q, Reuter VE, Saleeb RM, Shah RB, Smith SC, Tickoo S, Tretiakova MS, True L, Verkarre V, Wobker SE, Zhou M, Gill AJ
Mod Pathol 2021 Jul;34(7):1392-1424. Epub 2021 Mar 4 doi: 10.1038/s41379-021-00779-w. PMID: 33664427
Treatment of posterior fossa tumors in children.
Muzumdar D, Ventureyra EC
Expert Rev Neurother 2010 Apr;10(4):525-46. doi: 10.1586/ern.10.28. PMID: 20367206

Recent clinical studies

Etiology

Update on Cancer Predisposition Syndromes and Surveillance Guidelines for Childhood Brain Tumors.
Hansford JR, Das A, McGee RB, Nakano Y, Brzezinski J, Scollon SR, Rednam SP, Schienda J, Michaeli O, Kim SY, Greer MC, Weksberg R, Stewart DR, Foulkes WD, Tabori U, Pajtler KW, Pfister SM, Brodeur GM, Kamihara J
Clin Cancer Res 2024 Jun 3;30(11):2342-2350. doi: 10.1158/1078-0432.CCR-23-4033. PMID: 38573059Free PMC Article
NSD1 mediates antagonism between SWI/SNF and polycomb complexes and is required for transcriptional activation upon EZH2 inhibition.
Drosos Y, Myers JA, Xu B, Mathias KM, Beane EC, Radko-Juettner S, Mobley RJ, Larsen ME, Piccioni F, Ma X, Low J, Hansen BS, Peters ST, Bhanu NV, Dhanda SK, Chen T, Upadhyaya SA, Pruett-Miller SM, Root DE, Garcia BA, Partridge JF, Roberts CWM
Mol Cell 2022 Jul 7;82(13):2472-2489.e8. Epub 2022 May 9 doi: 10.1016/j.molcel.2022.04.015. PMID: 35537449Free PMC Article
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
Pembrolizumab in paediatric patients with advanced melanoma or a PD-L1-positive, advanced, relapsed, or refractory solid tumour or lymphoma (KEYNOTE-051): interim analysis of an open-label, single-arm, phase 1-2 trial.
Geoerger B, Kang HJ, Yalon-Oren M, Marshall LV, Vezina C, Pappo A, Laetsch TW, Petrilli AS, Ebinger M, Toporski J, Glade-Bender J, Nicholls W, Fox E, DuBois SG, Macy ME, Cohn SL, Pathiraja K, Diede SJ, Ebbinghaus S, Pinto N
Lancet Oncol 2020 Jan;21(1):121-133. Epub 2019 Dec 4 doi: 10.1016/S1470-2045(19)30671-0. PMID: 31812554
Pediatric Brain Tumors.
Dang M, Phillips PC
Continuum (Minneap Minn) 2017 Dec;23(6, Neuro-oncology):1727-1757. doi: 10.1212/CON.0000000000000545. PMID: 29200119

Diagnosis

Update on Cancer Predisposition Syndromes and Surveillance Guidelines for Childhood Brain Tumors.
Hansford JR, Das A, McGee RB, Nakano Y, Brzezinski J, Scollon SR, Rednam SP, Schienda J, Michaeli O, Kim SY, Greer MC, Weksberg R, Stewart DR, Foulkes WD, Tabori U, Pajtler KW, Pfister SM, Brodeur GM, Kamihara J
Clin Cancer Res 2024 Jun 3;30(11):2342-2350. doi: 10.1158/1078-0432.CCR-23-4033. PMID: 38573059Free PMC Article
New developments in existing WHO entities and evolving molecular concepts: The Genitourinary Pathology Society (GUPS) update on renal neoplasia.
Trpkov K, Hes O, Williamson SR, Adeniran AJ, Agaimy A, Alaghehbandan R, Amin MB, Argani P, Chen YB, Cheng L, Epstein JI, Cheville JC, Comperat E, da Cunha IW, Gordetsky JB, Gupta S, He H, Hirsch MS, Humphrey PA, Kapur P, Kojima F, Lopez JI, Maclean F, Magi-Galluzzi C, McKenney JK, Mehra R, Menon S, Netto GJ, Przybycin CG, Rao P, Rao Q, Reuter VE, Saleeb RM, Shah RB, Smith SC, Tickoo S, Tretiakova MS, True L, Verkarre V, Wobker SE, Zhou M, Gill AJ
Mod Pathol 2021 Jul;34(7):1392-1424. Epub 2021 Mar 4 doi: 10.1038/s41379-021-00779-w. PMID: 33664427
Renal Medullary Carcinoma.
Elliott A, Bruner E
Arch Pathol Lab Med 2019 Dec;143(12):1556-1561. Epub 2019 Mar 11 doi: 10.5858/arpa.2017-0492-RS. PMID: 30855171
Pediatric Brain Tumors.
Dang M, Phillips PC
Continuum (Minneap Minn) 2017 Dec;23(6, Neuro-oncology):1727-1757. doi: 10.1212/CON.0000000000000545. PMID: 29200119
Posterior Fossa Tumors.
Brandão LA, Young Poussaint T
Neuroimaging Clin N Am 2017 Feb;27(1):1-37. doi: 10.1016/j.nic.2016.08.001. PMID: 27889018

Therapy

Current Open Trials and Molecular Update for Pediatric Embryonal Tumors.
Rosenberg T, Cooney T
Pediatr Neurosurg 2023;58(5):299-306. Epub 2023 May 26 doi: 10.1159/000531256. PMID: 37245504
Tacedinaline (CI-994), a class I HDAC inhibitor, targets intrinsic tumor growth and leptomeningeal dissemination in MYC-driven medulloblastoma while making them susceptible to anti-CD47-induced macrophage phagocytosis via NF-kB-TGM2 driven tumor inflammation.
Marquardt V, Theruvath J, Pauck D, Picard D, Qin N, Blümel L, Maue M, Bartl J, Ahmadov U, Langini M, Meyer FD, Cole A, Cruz-Cruz J, Graef CM, Wölfl M, Milde T, Witt O, Erdreich-Epstein A, Leprivier G, Kahlert U, Stefanski A, Stühler K, Keir ST, Bigner DD, Hauer J, Beez T, Knobbe-Thomsen CB, Fischer U, Felsberg J, Hansen FK, Vibhakar R, Venkatraman S, Cheshier SH, Reifenberger G, Borkhardt A, Kurz T, Remke M, Mitra S
J Immunother Cancer 2023 Jan;11(1) doi: 10.1136/jitc-2022-005871. PMID: 36639156Free PMC Article
Pembrolizumab in paediatric patients with advanced melanoma or a PD-L1-positive, advanced, relapsed, or refractory solid tumour or lymphoma (KEYNOTE-051): interim analysis of an open-label, single-arm, phase 1-2 trial.
Geoerger B, Kang HJ, Yalon-Oren M, Marshall LV, Vezina C, Pappo A, Laetsch TW, Petrilli AS, Ebinger M, Toporski J, Glade-Bender J, Nicholls W, Fox E, DuBois SG, Macy ME, Cohn SL, Pathiraja K, Diede SJ, Ebbinghaus S, Pinto N
Lancet Oncol 2020 Jan;21(1):121-133. Epub 2019 Dec 4 doi: 10.1016/S1470-2045(19)30671-0. PMID: 31812554
New molecular targets in meningiomas: the present and the future.
Venur VA, Santagata S, Galanis E, Brastianos PK
Curr Opin Neurol 2018 Dec;31(6):740-746. doi: 10.1097/WCO.0000000000000615. PMID: 30379704
Atypical teratoid/rhabdoid tumors-current concepts, advances in biology, and potential future therapies.
Frühwald MC, Biegel JA, Bourdeaut F, Roberts CW, Chi SN
Neuro Oncol 2016 Jun;18(6):764-78. Epub 2016 Jan 10 doi: 10.1093/neuonc/nov264. PMID: 26755072Free PMC Article

Prognosis

Sarcomatoid and Rhabdoid Renal Cell Carcinoma: Clinical, Pathologic, and Molecular Genetic Features.
Adeniran AJ, Shuch B, Humphrey PA
Am J Surg Pathol 2024 Jul 1;48(7):e65-e88. Epub 2024 May 13 doi: 10.1097/PAS.0000000000002233. PMID: 38736105
Atypical Teratoid/Rhabdoid Tumor of the Nervous System in Adults: Location-Related Features and Outcome.
Corvino S, Del Basso De Caro M, Franca RA, Corazzelli G, Della Monica R, Chiariotti L, Maiuri F
World Neurosurg 2023 Nov;179:e404-e415. Epub 2023 Sep 1 doi: 10.1016/j.wneu.2023.08.107. PMID: 37659753
New developments in existing WHO entities and evolving molecular concepts: The Genitourinary Pathology Society (GUPS) update on renal neoplasia.
Trpkov K, Hes O, Williamson SR, Adeniran AJ, Agaimy A, Alaghehbandan R, Amin MB, Argani P, Chen YB, Cheng L, Epstein JI, Cheville JC, Comperat E, da Cunha IW, Gordetsky JB, Gupta S, He H, Hirsch MS, Humphrey PA, Kapur P, Kojima F, Lopez JI, Maclean F, Magi-Galluzzi C, McKenney JK, Mehra R, Menon S, Netto GJ, Przybycin CG, Rao P, Rao Q, Reuter VE, Saleeb RM, Shah RB, Smith SC, Tickoo S, Tretiakova MS, True L, Verkarre V, Wobker SE, Zhou M, Gill AJ
Mod Pathol 2021 Jul;34(7):1392-1424. Epub 2021 Mar 4 doi: 10.1038/s41379-021-00779-w. PMID: 33664427
Pembrolizumab in paediatric patients with advanced melanoma or a PD-L1-positive, advanced, relapsed, or refractory solid tumour or lymphoma (KEYNOTE-051): interim analysis of an open-label, single-arm, phase 1-2 trial.
Geoerger B, Kang HJ, Yalon-Oren M, Marshall LV, Vezina C, Pappo A, Laetsch TW, Petrilli AS, Ebinger M, Toporski J, Glade-Bender J, Nicholls W, Fox E, DuBois SG, Macy ME, Cohn SL, Pathiraja K, Diede SJ, Ebbinghaus S, Pinto N
Lancet Oncol 2020 Jan;21(1):121-133. Epub 2019 Dec 4 doi: 10.1016/S1470-2045(19)30671-0. PMID: 31812554
Novel therapy for pediatric and adolescent kidney cancer.
Walz AL, Fernandez CV, Geller JI
Cancer Metastasis Rev 2019 Dec;38(4):643-655. doi: 10.1007/s10555-019-09822-4. PMID: 31811552

Clinical prediction guides

Targeting EZH2 in SMARCB1-deficient sarcomas: Advances and opportunities to potentiate the efficacy of EZH2 inhibitors.
Lanzi C, Arrighetti N, Pasquali S, Cassinelli G
Biochem Pharmacol 2023 Sep;215:115727. Epub 2023 Aug 2 doi: 10.1016/j.bcp.2023.115727. PMID: 37541451
New developments in existing WHO entities and evolving molecular concepts: The Genitourinary Pathology Society (GUPS) update on renal neoplasia.
Trpkov K, Hes O, Williamson SR, Adeniran AJ, Agaimy A, Alaghehbandan R, Amin MB, Argani P, Chen YB, Cheng L, Epstein JI, Cheville JC, Comperat E, da Cunha IW, Gordetsky JB, Gupta S, He H, Hirsch MS, Humphrey PA, Kapur P, Kojima F, Lopez JI, Maclean F, Magi-Galluzzi C, McKenney JK, Mehra R, Menon S, Netto GJ, Przybycin CG, Rao P, Rao Q, Reuter VE, Saleeb RM, Shah RB, Smith SC, Tickoo S, Tretiakova MS, True L, Verkarre V, Wobker SE, Zhou M, Gill AJ
Mod Pathol 2021 Jul;34(7):1392-1424. Epub 2021 Mar 4 doi: 10.1038/s41379-021-00779-w. PMID: 33664427
SWI/SNF complex-deficient soft tissue neoplasms: An update.
Schaefer IM, Hornick JL
Semin Diagn Pathol 2021 May;38(3):222-231. Epub 2020 Jun 5 doi: 10.1053/j.semdp.2020.05.005. PMID: 32646614Free PMC Article
Pembrolizumab in paediatric patients with advanced melanoma or a PD-L1-positive, advanced, relapsed, or refractory solid tumour or lymphoma (KEYNOTE-051): interim analysis of an open-label, single-arm, phase 1-2 trial.
Geoerger B, Kang HJ, Yalon-Oren M, Marshall LV, Vezina C, Pappo A, Laetsch TW, Petrilli AS, Ebinger M, Toporski J, Glade-Bender J, Nicholls W, Fox E, DuBois SG, Macy ME, Cohn SL, Pathiraja K, Diede SJ, Ebbinghaus S, Pinto N
Lancet Oncol 2020 Jan;21(1):121-133. Epub 2019 Dec 4 doi: 10.1016/S1470-2045(19)30671-0. PMID: 31812554
Renal Medullary Carcinoma.
Elliott A, Bruner E
Arch Pathol Lab Med 2019 Dec;143(12):1556-1561. Epub 2019 Mar 11 doi: 10.5858/arpa.2017-0492-RS. PMID: 30855171

Recent systematic reviews

Atypical teratoid/rhabdoid tumor in adults: a systematic review of the literature with meta-analysis and additional reports of 4 cases.
Broggi G, Gianno F, Shemy DT, Massimino M, Milanaccio C, Mastronuzzi A, Rossi S, Arcella A, Giangaspero F, Antonelli M
J Neurooncol 2022 Mar;157(1):1-14. Epub 2022 Feb 25 doi: 10.1007/s11060-022-03959-z. PMID: 35217948

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