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Refractory Atypical Teratoid/Rhabdoid Tumor

MedGen UID:
1685327
•Concept ID:
C5206771
•
Neoplastic Process

Definition

An atypical teratoid/rhabdoid tumor that does not respond to treatment. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRefractory Atypical Teratoid/Rhabdoid Tumor

Professional guidelines

PubMed

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

Recent clinical studies

Etiology

Outcomes for children with recurrent/refractory atypical teratoid rhabdoid tumor: A single-institution study with molecular correlation.
Carey SS, Huang J, Myers JR, Mostafavi R, Orr BA, Dhanda SK, Michalik LH, Tatevossian RG, Klimo P Jr, Boop F, Lu C, Sioson E, Zhou X, Nichols KE, Merchant TE, Ellison DW, Robinson GW, Onar-Thomas A, Gajjar A, Upadhyaya SA
Pediatr Blood Cancer 2024 Oct;71(10):e31208. Epub 2024 Jul 21 doi: 10.1002/pbc.31208. PMID: 39034595Free PMC Article
Retrospective experience of children with relapsed brain tumors treated with oral combination of axitinib and metronomic etoposide.
Donzé C, Revon-Rivière G, Pondrom M, Verschuur A, Leblond P, André N
Pediatr Blood Cancer 2024 Aug;71(8):e31076. Epub 2024 May 22 doi: 10.1002/pbc.31076. PMID: 38778441
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
Optical genome mapping identifies a germline retrotransposon insertion in SMARCB1 in two siblings with atypical teratoid rhabdoid tumors.
Sabatella M, Mantere T, Waanders E, Neveling K, Mensenkamp AR, van Dijk F, Hehir-Kwa JY, Derks R, Kwint M, O'Gorman L, Tropa Martins M, Gidding CE, Lequin MH, Küsters B, Wesseling P, Nelen M, Biegel JA, Hoischen A, Jongmans MC, Kuiper RP
J Pathol 2021 Oct;255(2):202-211. Epub 2021 Jul 29 doi: 10.1002/path.5755. PMID: 34231212Free PMC Article
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

Diagnosis

Outcomes for children with recurrent/refractory atypical teratoid rhabdoid tumor: A single-institution study with molecular correlation.
Carey SS, Huang J, Myers JR, Mostafavi R, Orr BA, Dhanda SK, Michalik LH, Tatevossian RG, Klimo P Jr, Boop F, Lu C, Sioson E, Zhou X, Nichols KE, Merchant TE, Ellison DW, Robinson GW, Onar-Thomas A, Gajjar A, Upadhyaya SA
Pediatr Blood Cancer 2024 Oct;71(10):e31208. Epub 2024 Jul 21 doi: 10.1002/pbc.31208. PMID: 39034595Free 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
Optical genome mapping identifies a germline retrotransposon insertion in SMARCB1 in two siblings with atypical teratoid rhabdoid tumors.
Sabatella M, Mantere T, Waanders E, Neveling K, Mensenkamp AR, van Dijk F, Hehir-Kwa JY, Derks R, Kwint M, O'Gorman L, Tropa Martins M, Gidding CE, Lequin MH, Küsters B, Wesseling P, Nelen M, Biegel JA, Hoischen A, Jongmans MC, Kuiper RP
J Pathol 2021 Oct;255(2):202-211. Epub 2021 Jul 29 doi: 10.1002/path.5755. PMID: 34231212Free PMC Article
Oncolytic measles virus efficacy in murine xenograft models of atypical teratoid rhabdoid tumors.
Studebaker AW, Hutzen B, Pierson CR, Shaffer TA, Raffel C, Jackson EM
Neuro Oncol 2015 Dec;17(12):1568-77. Epub 2015 Apr 2 doi: 10.1093/neuonc/nov058. PMID: 25838138Free 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
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
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
Resveratrol-induced apoptosis and increased radiosensitivity in CD133-positive cells derived from atypical teratoid/rhabdoid tumor.
Kao CL, Huang PI, Tsai PH, Tsai ML, Lo JF, Lee YY, Chen YJ, Chen YW, Chiou SH
Int J Radiat Oncol Biol Phys 2009 May 1;74(1):219-28. doi: 10.1016/j.ijrobp.2008.12.035. PMID: 19362240

Prognosis

Outcomes for children with recurrent/refractory atypical teratoid rhabdoid tumor: A single-institution study with molecular correlation.
Carey SS, Huang J, Myers JR, Mostafavi R, Orr BA, Dhanda SK, Michalik LH, Tatevossian RG, Klimo P Jr, Boop F, Lu C, Sioson E, Zhou X, Nichols KE, Merchant TE, Ellison DW, Robinson GW, Onar-Thomas A, Gajjar A, Upadhyaya SA
Pediatr Blood Cancer 2024 Oct;71(10):e31208. Epub 2024 Jul 21 doi: 10.1002/pbc.31208. PMID: 39034595Free PMC Article
Retrospective experience of children with relapsed brain tumors treated with oral combination of axitinib and metronomic etoposide.
Donzé C, Revon-Rivière G, Pondrom M, Verschuur A, Leblond P, André N
Pediatr Blood Cancer 2024 Aug;71(8):e31076. Epub 2024 May 22 doi: 10.1002/pbc.31076. PMID: 38778441
Optical genome mapping identifies a germline retrotransposon insertion in SMARCB1 in two siblings with atypical teratoid rhabdoid tumors.
Sabatella M, Mantere T, Waanders E, Neveling K, Mensenkamp AR, van Dijk F, Hehir-Kwa JY, Derks R, Kwint M, O'Gorman L, Tropa Martins M, Gidding CE, Lequin MH, Küsters B, Wesseling P, Nelen M, Biegel JA, Hoischen A, Jongmans MC, Kuiper RP
J Pathol 2021 Oct;255(2):202-211. Epub 2021 Jul 29 doi: 10.1002/path.5755. PMID: 34231212Free PMC Article
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
Oncolytic measles virus efficacy in murine xenograft models of atypical teratoid rhabdoid tumors.
Studebaker AW, Hutzen B, Pierson CR, Shaffer TA, Raffel C, Jackson EM
Neuro Oncol 2015 Dec;17(12):1568-77. Epub 2015 Apr 2 doi: 10.1093/neuonc/nov058. PMID: 25838138Free PMC Article

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
Optical genome mapping identifies a germline retrotransposon insertion in SMARCB1 in two siblings with atypical teratoid rhabdoid tumors.
Sabatella M, Mantere T, Waanders E, Neveling K, Mensenkamp AR, van Dijk F, Hehir-Kwa JY, Derks R, Kwint M, O'Gorman L, Tropa Martins M, Gidding CE, Lequin MH, Küsters B, Wesseling P, Nelen M, Biegel JA, Hoischen A, Jongmans MC, Kuiper RP
J Pathol 2021 Oct;255(2):202-211. Epub 2021 Jul 29 doi: 10.1002/path.5755. PMID: 34231212Free PMC Article
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

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