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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

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: 37228094
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
Safety and toxicity of intrathecal liposomal cytarabine (Depocyte) in children and adolescents with recurrent or refractory brain tumors: a multi-institutional retrospective study.
Benesch M, Siegler N, Hoff Kv, Lassay L, Kropshofer G, Müller H, Sommer C, Rutkowski S, Fleischhack G, Urban C
Anticancer Drugs 2009 Oct;20(9):794-9. doi: 10.1097/CAD.0b013e32832f4abe. PMID: 19617818

Diagnosis

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
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
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
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
Identification of CD133-positive radioresistant cells in atypical teratoid/rhabdoid tumor.
Chiou SH, Kao CL, Chen YW, Chien CS, Hung SC, Lo JF, Chen YJ, Ku HH, Hsu MT, Wong TT
PLoS One 2008 May 7;3(5):e2090. doi: 10.1371/journal.pone.0002090. PMID: 18509505Free PMC Article

Therapy

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
Cytotoxicity and Target Modulation in Pediatric Solid Tumors by the Proteasome Inhibitor Carfilzomib.
Thakur S, Ruan Y, Jayanthan A, Boklan J, Narendran A
Curr Cancer Drug Targets 2021;21(9):804-811. doi: 10.2174/1568009621666210504085527. PMID: 33949932
Safety and toxicity of intrathecal liposomal cytarabine (Depocyte) in children and adolescents with recurrent or refractory brain tumors: a multi-institutional retrospective study.
Benesch M, Siegler N, Hoff Kv, Lassay L, Kropshofer G, Müller H, Sommer C, Rutkowski S, Fleischhack G, Urban C
Anticancer Drugs 2009 Oct;20(9):794-9. doi: 10.1097/CAD.0b013e32832f4abe. PMID: 19617818
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
Phase II study of oxaliplatin in children with recurrent or refractory medulloblastoma, supratentorial primitive neuroectodermal tumors, and atypical teratoid rhabdoid tumors: a pediatric brain tumor consortium study.
Fouladi M, Blaney SM, Poussaint TY, Freeman BB 3rd, McLendon R, Fuller C, Adesina AM, Hancock ML, Danks MK, Stewart C, Boyett JM, Gajjar A
Cancer 2006 Nov 1;107(9):2291-7. doi: 10.1002/cncr.22241. PMID: 17019740

Prognosis

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
Phase I trial of MK-0752 in children with refractory CNS malignancies: a pediatric brain tumor consortium study.
Fouladi M, Stewart CF, Olson J, Wagner LM, Onar-Thomas A, Kocak M, Packer RJ, Goldman S, Gururangan S, Gajjar A, Demuth T, Kun LE, Boyett JM, Gilbertson RJ
J Clin Oncol 2011 Sep 10;29(26):3529-34. Epub 2011 Aug 8 doi: 10.1200/JCO.2011.35.7806. PMID: 21825264Free 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
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
Safety and toxicity of intrathecal liposomal cytarabine (Depocyte) in children and adolescents with recurrent or refractory brain tumors: a multi-institutional retrospective study.
Benesch M, Siegler N, Hoff Kv, Lassay L, Kropshofer G, Müller H, Sommer C, Rutkowski S, Fleischhack G, Urban C
Anticancer Drugs 2009 Oct;20(9):794-9. doi: 10.1097/CAD.0b013e32832f4abe. PMID: 19617818
Phase II study of oxaliplatin in children with recurrent or refractory medulloblastoma, supratentorial primitive neuroectodermal tumors, and atypical teratoid rhabdoid tumors: a pediatric brain tumor consortium study.
Fouladi M, Blaney SM, Poussaint TY, Freeman BB 3rd, McLendon R, Fuller C, Adesina AM, Hancock ML, Danks MK, Stewart C, Boyett JM, Gajjar A
Cancer 2006 Nov 1;107(9):2291-7. doi: 10.1002/cncr.22241. PMID: 17019740

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