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Refractory Rhabdoid Tumor

MedGen UID:
1647845
•Concept ID:
C4684871
•
Neoplastic Process

Definition

Rhabdoid tumor that does not respond to treatment. [from NCI]

Professional guidelines

PubMed

Palliative endoscopic third ventriculostomy for pediatric primary brain tumors: a single-institution case series.
Rennert RC, Brandel MG, Srinivas S, Prajapati D, Al Jammal OM, Brown NJ, Diaz-Aguilar LD, Elster J, Gonda DD, Crawford JR, Levy ML
J Neurosurg Pediatr 2021 Oct 1;28(4):387-394. Epub 2021 Aug 6 doi: 10.3171/2021.3.PEDS20952. PMID: 34359046
Nimotuzumab therapy in the treatment of pediatric central nervous system tumors: single-center experience.
Susam-Sen H, Varan A, Bajin İ, Göçmen R, Aydın B, Yalcin B, Kurucu N, Kutluk T, Bayhan T, Akyuz C
Naunyn Schmiedebergs Arch Pharmacol 2021 Aug;394(8):1769-1777. Epub 2021 Jun 21 doi: 10.1007/s00210-021-02109-y. PMID: 34151393
Autologous Stem Cell Transplantation for Children With Renal Tumors, and Adults With Wilms Tumor: Retrospective Analysis of the Japanese Transplant Registry Unified Management Program.
Kobayashi R, Inoue M, Takahashi Y, Kikuta A, Ogawa A, Koga Y, Koh K, Hashii Y, Hara J, Kato K, Tabuchi K, Matsumoto K
J Pediatr Hematol Oncol 2020 May;42(4):251-255. doi: 10.1097/MPH.0000000000001779. PMID: 32134841

Recent clinical studies

Etiology

Tazemetostat for tumors harboring SMARCB1/SMARCA4 or EZH2 alterations: results from NCI-COG pediatric MATCH APEC1621C.
Chi SN, Yi JS, Williams PM, Roy-Chowdhuri S, Patton DR, Coffey BD, Reid JM, Piao J, Saguilig L, Alonzo TA, Berg SL, Ramirez NC, Jaju A, Mhlanga JC, Fox E, Hawkins DS, Mooney MM, Takebe N, Tricoli JV, Janeway KA, Seibel NL, Parsons DW
J Natl Cancer Inst 2023 Nov 8;115(11):1355-1363. doi: 10.1093/jnci/djad085. PMID: 37228094Free PMC Article
Clinical evidence for a biological effect of epigenetically active decitabine in relapsed or progressive rhabdoid tumors.
Steinbügl M, Nemes K, Johann P, Kröncke T, Tüchert S, da Costa MJG, Ebinger M, Schüller U, Sehested A, Hauser P, Reinhard H, Sumerauer D, Hettmer S, Jakob M, Hasselblatt M, Siebert R, Witt O, Gerss J, Kerl K, Frühwald MC
Pediatr Blood Cancer 2021 Dec;68(12):e29267. Epub 2021 Aug 4 doi: 10.1002/pbc.29267. PMID: 34347371
Progress Update in Pediatric Renal Tumors.
Jain J, Sutton KS, Hong AL
Curr Oncol Rep 2021 Feb 16;23(3):33. doi: 10.1007/s11912-021-01016-y. PMID: 33591402
SMARCB1/INI1-deficient tumors of adulthood.
Parker NA, Al-Obaidi A, Deutsch JM
F1000Res 2020;9:662. Epub 2020 Jun 30 doi: 10.12688/f1000research.24808.2. PMID: 33796273Free 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

Diagnosis

Tazemetostat for tumors harboring SMARCB1/SMARCA4 or EZH2 alterations: results from NCI-COG pediatric MATCH APEC1621C.
Chi SN, Yi JS, Williams PM, Roy-Chowdhuri S, Patton DR, Coffey BD, Reid JM, Piao J, Saguilig L, Alonzo TA, Berg SL, Ramirez NC, Jaju A, Mhlanga JC, Fox E, Hawkins DS, Mooney MM, Takebe N, Tricoli JV, Janeway KA, Seibel NL, Parsons DW
J Natl Cancer Inst 2023 Nov 8;115(11):1355-1363. doi: 10.1093/jnci/djad085. PMID: 37228094Free PMC Article
Progress Update in Pediatric Renal Tumors.
Jain J, Sutton KS, Hong AL
Curr Oncol Rep 2021 Feb 16;23(3):33. doi: 10.1007/s11912-021-01016-y. PMID: 33591402
SMARCB1/INI1-deficient tumors of adulthood.
Parker NA, Al-Obaidi A, Deutsch JM
F1000Res 2020;9:662. Epub 2020 Jun 30 doi: 10.12688/f1000research.24808.2. PMID: 33796273Free PMC Article
Novel Two MRT Cell Lines Established from Multiple Sites of a Synchronous MRT Patient.
Kuwahara Y, Iehara T, Ichise E, Katsumi Y, Ouchi K, Tsuchiya K, Miyachi M, Konishi E, Sasajima H, Nakamura S, Fumino S, Tajiri T, Johann PD, FrÜhwald MC, Yoshida T, Okuda T, Hosoi H
Anticancer Res 2020 Nov;40(11):6159-6170. doi: 10.21873/anticanres.14636. PMID: 33109553
Loss of CDKN1C in a Recurrent Atypical Teratoid/Rhabdoid Tumor.
Tran D, Camelo-Piragua S, Gupta A, Gowans K, Robertson PL, Mody R, Koschmann C
J Pediatr Hematol Oncol 2017 Nov;39(8):e466-e469. doi: 10.1097/MPH.0000000000000873. PMID: 28731921Free PMC Article

Therapy

Systematic characterization of antibody-drug conjugate targets in central nervous system tumors.
Coy S, Lee JS, Chan SJ, Woo T, Jones J, Alexandrescu S, Wen PY, Sorger PK, Ligon KL, Santagata S
Neuro Oncol 2024 Mar 4;26(3):458-472. doi: 10.1093/neuonc/noad205. PMID: 37870091Free PMC Article
Tazemetostat for tumors harboring SMARCB1/SMARCA4 or EZH2 alterations: results from NCI-COG pediatric MATCH APEC1621C.
Chi SN, Yi JS, Williams PM, Roy-Chowdhuri S, Patton DR, Coffey BD, Reid JM, Piao J, Saguilig L, Alonzo TA, Berg SL, Ramirez NC, Jaju A, Mhlanga JC, Fox E, Hawkins DS, Mooney MM, Takebe N, Tricoli JV, Janeway KA, Seibel NL, Parsons DW
J Natl Cancer Inst 2023 Nov 8;115(11):1355-1363. doi: 10.1093/jnci/djad085. PMID: 37228094Free 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
Non-Malignant Cerebrospinal Fluid Ascites in a Patient with Atypical Teratoid Rhabdoid Tumor.
Upadhyaya SA, Klimo P Jr, Robinson GW
Oncol Res Treat 2017;40(4):216-219. Epub 2017 Mar 21 doi: 10.1159/000458441. PMID: 28365696
Successful Treatment of Extra-Renal Noncerebral Rhabdoid Tumors with VIDE.
Yasui N, Yoshida A, Kobayashi E, Nakatani F, Kawamoto H
Pediatr Blood Cancer 2016 Feb;63(2):352-4. Epub 2015 Oct 15 doi: 10.1002/pbc.25777. PMID: 26469354

Prognosis

Clinical evidence for a biological effect of epigenetically active decitabine in relapsed or progressive rhabdoid tumors.
Steinbügl M, Nemes K, Johann P, Kröncke T, Tüchert S, da Costa MJG, Ebinger M, Schüller U, Sehested A, Hauser P, Reinhard H, Sumerauer D, Hettmer S, Jakob M, Hasselblatt M, Siebert R, Witt O, Gerss J, Kerl K, Frühwald MC
Pediatr Blood Cancer 2021 Dec;68(12):e29267. Epub 2021 Aug 4 doi: 10.1002/pbc.29267. PMID: 34347371
Nimotuzumab therapy in the treatment of pediatric central nervous system tumors: single-center experience.
Susam-Sen H, Varan A, Bajin İ, Göçmen R, Aydın B, Yalcin B, Kurucu N, Kutluk T, Bayhan T, Akyuz C
Naunyn Schmiedebergs Arch Pharmacol 2021 Aug;394(8):1769-1777. Epub 2021 Jun 21 doi: 10.1007/s00210-021-02109-y. PMID: 34151393
Progress Update in Pediatric Renal Tumors.
Jain J, Sutton KS, Hong AL
Curr Oncol Rep 2021 Feb 16;23(3):33. doi: 10.1007/s11912-021-01016-y. PMID: 33591402
SMARCB1/INI1-deficient tumors of adulthood.
Parker NA, Al-Obaidi A, Deutsch JM
F1000Res 2020;9:662. Epub 2020 Jun 30 doi: 10.12688/f1000research.24808.2. PMID: 33796273Free 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

Clinical prediction guides

B7-H3 in Brain Malignancies: Immunology and Immunotherapy.
Guo X, Chang M, Wang Y, Xing B, Ma W
Int J Biol Sci 2023;19(12):3762-3780. Epub 2023 Jul 24 doi: 10.7150/ijbs.85813. PMID: 37564196Free PMC Article
Tazemetostat for tumors harboring SMARCB1/SMARCA4 or EZH2 alterations: results from NCI-COG pediatric MATCH APEC1621C.
Chi SN, Yi JS, Williams PM, Roy-Chowdhuri S, Patton DR, Coffey BD, Reid JM, Piao J, Saguilig L, Alonzo TA, Berg SL, Ramirez NC, Jaju A, Mhlanga JC, Fox E, Hawkins DS, Mooney MM, Takebe N, Tricoli JV, Janeway KA, Seibel NL, Parsons DW
J Natl Cancer Inst 2023 Nov 8;115(11):1355-1363. doi: 10.1093/jnci/djad085. PMID: 37228094Free PMC Article
A phase I trial of metformin in combination with vincristine, irinotecan, and temozolomide in children with relapsed or refractory solid and central nervous system tumors: A report from the national pediatric cancer foundation.
Metts JL, Trucco M, Weiser DA, Thompson P, Sandler E, Smith T, Crimella J, Sansil S, Thapa R, Fridley BL, Llosa N, Badgett T, Gorlick R, Reed D, Gill J
Cancer Med 2023 Feb;12(4):4270-4281. Epub 2022 Sep 23 doi: 10.1002/cam4.5297. PMID: 36151773Free PMC Article
Clinical evidence for a biological effect of epigenetically active decitabine in relapsed or progressive rhabdoid tumors.
Steinbügl M, Nemes K, Johann P, Kröncke T, Tüchert S, da Costa MJG, Ebinger M, Schüller U, Sehested A, Hauser P, Reinhard H, Sumerauer D, Hettmer S, Jakob M, Hasselblatt M, Siebert R, Witt O, Gerss J, Kerl K, Frühwald MC
Pediatr Blood Cancer 2021 Dec;68(12):e29267. Epub 2021 Aug 4 doi: 10.1002/pbc.29267. PMID: 34347371
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

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