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Rhabdoid tumor(RDT)

MedGen UID:
64646
Concept ID:
C0206743
Neoplastic Process
Synonyms: Malignant rhabdoid tumor; RDT
SNOMED CT: Rhabdoid sarcoma (83118000); Rhabdoid tumor (83118000); Malignant rhabdoid tumor (83118000); Malignant rhabdoid tumor (1156418001)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Related genes: SMARCB1, SMARCA4
 
HPO: HP:0034557
Monarch Initiative: MONDO:0002728
OMIM®: 609322
Orphanet: ORPHA69077

Definition

Rhabdoid tumors are rare aggressive malignancies in infants and young children with a poor prognosis. The most common anatomic localizations are the central nervous system, the kidneys, and other soft tissues. [from HPO]

Professional guidelines

PubMed

Gholamin S, Mitra SS, Feroze AH, Liu J, Kahn SA, Zhang M, Esparza R, Richard C, Ramaswamy V, Remke M, Volkmer AK, Willingham S, Ponnuswami A, McCarty A, Lovelace P, Storm TA, Schubert S, Hutter G, Narayanan C, Chu P, Raabe EH, Harsh G 4th, Taylor MD, Monje M, Cho YJ, Majeti R, Volkmer JP, Fisher PG, Grant G, Steinberg GK, Vogel H, Edwards M, Weissman IL, Cheshier SH
Sci Transl Med 2017 Mar 15;9(381) doi: 10.1126/scitranslmed.aaf2968. PMID: 28298418
Geller JI, Roth JJ, Biegel JA
Crit Rev Oncog 2015;20(3-4):199-216. doi: 10.1615/critrevoncog.2015013566. PMID: 26349416Free PMC Article
Chokoeva AA, Tchernev G, Cardoso JC, Patterson JW, Dechev I, Valkanov S, Zanardelli M, Lotti T, Wollina U
Int J Immunopathol Pharmacol 2015 Jun;28(2):168-77. Epub 2015 Mar 26 doi: 10.1177/0394632015576029. PMID: 25816394

Recent clinical studies

Etiology

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
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
Reddy AT, Strother DR, Judkins AR, Burger PC, Pollack IF, Krailo MD, Buxton AB, Williams-Hughes C, Fouladi M, Mahajan A, Merchant TE, Ho B, Mazewski CM, Lewis VA, Gajjar A, Vezina LG, Booth TN, Parsons KW, Poss VL, Zhou T, Biegel JA, Huang A
J Clin Oncol 2020 Apr 10;38(11):1175-1185. Epub 2020 Feb 27 doi: 10.1200/JCO.19.01776. PMID: 32105509Free PMC Article
Ho B, Johann PD, Grabovska Y, De Dieu Andrianteranagna MJ, Yao F, Frühwald M, Hasselblatt M, Bourdeaut F, Williamson D, Huang A, Kool M
Neuro Oncol 2020 May 15;22(5):613-624. doi: 10.1093/neuonc/noz235. PMID: 31889194Free PMC Article
Kristensen BW, Priesterbach-Ackley LP, Petersen JK, Wesseling P
Ann Oncol 2019 Aug 1;30(8):1265-1278. doi: 10.1093/annonc/mdz164. PMID: 31124566Free PMC Article

Diagnosis

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
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
Udaka YT, Packer RJ
Neurol Clin 2018 Aug;36(3):533-556. doi: 10.1016/j.ncl.2018.04.009. PMID: 30072070
Geller JI, Roth JJ, Biegel JA
Crit Rev Oncog 2015;20(3-4):199-216. doi: 10.1615/critrevoncog.2015013566. PMID: 26349416Free PMC Article
James AW, Dry SM
Surg Pathol Clin 2015 Sep;8(3):309-29. doi: 10.1016/j.path.2015.05.002. PMID: 26297059

Therapy

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
Reddy AT, Strother DR, Judkins AR, Burger PC, Pollack IF, Krailo MD, Buxton AB, Williams-Hughes C, Fouladi M, Mahajan A, Merchant TE, Ho B, Mazewski CM, Lewis VA, Gajjar A, Vezina LG, Booth TN, Parsons KW, Poss VL, Zhou T, Biegel JA, Huang A
J Clin Oncol 2020 Apr 10;38(11):1175-1185. Epub 2020 Feb 27 doi: 10.1200/JCO.19.01776. PMID: 32105509Free PMC Article
Kristensen BW, Priesterbach-Ackley LP, Petersen JK, Wesseling P
Ann Oncol 2019 Aug 1;30(8):1265-1278. doi: 10.1093/annonc/mdz164. PMID: 31124566Free PMC Article
Gholamin S, Mitra SS, Feroze AH, Liu J, Kahn SA, Zhang M, Esparza R, Richard C, Ramaswamy V, Remke M, Volkmer AK, Willingham S, Ponnuswami A, McCarty A, Lovelace P, Storm TA, Schubert S, Hutter G, Narayanan C, Chu P, Raabe EH, Harsh G 4th, Taylor MD, Monje M, Cho YJ, Majeti R, Volkmer JP, Fisher PG, Grant G, Steinberg GK, Vogel H, Edwards M, Weissman IL, Cheshier SH
Sci Transl Med 2017 Mar 15;9(381) doi: 10.1126/scitranslmed.aaf2968. PMID: 28298418
McGovern SL, Grosshans D, Mahajan A
Cancer J 2014 Nov-Dec;20(6):397-402. doi: 10.1097/PPO.0000000000000081. PMID: 25415685

Prognosis

Reddy AT, Strother DR, Judkins AR, Burger PC, Pollack IF, Krailo MD, Buxton AB, Williams-Hughes C, Fouladi M, Mahajan A, Merchant TE, Ho B, Mazewski CM, Lewis VA, Gajjar A, Vezina LG, Booth TN, Parsons KW, Poss VL, Zhou T, Biegel JA, Huang A
J Clin Oncol 2020 Apr 10;38(11):1175-1185. Epub 2020 Feb 27 doi: 10.1200/JCO.19.01776. PMID: 32105509Free PMC Article
Kristensen BW, Priesterbach-Ackley LP, Petersen JK, Wesseling P
Ann Oncol 2019 Aug 1;30(8):1265-1278. doi: 10.1093/annonc/mdz164. PMID: 31124566Free PMC Article
James AW, Dry SM
Surg Pathol Clin 2015 Sep;8(3):309-29. doi: 10.1016/j.path.2015.05.002. PMID: 26297059
Isaacs H Jr
J Pediatr Surg 2010 Mar;45(3):619-26. doi: 10.1016/j.jpedsurg.2009.12.011. PMID: 20223330
Shamberger RC
Semin Surg Oncol 1999 Mar;16(2):105-20. doi: 10.1002/(sici)1098-2388(199903)16:2<105::aid-ssu4>3.0.co;2-t. PMID: 9988867

Clinical prediction guides

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
Calandrini C, Drost J
STAR Protoc 2022 Mar 18;3(1):101079. Epub 2022 Jan 10 doi: 10.1016/j.xpro.2021.101079. PMID: 35036959Free PMC Article
Nambirajan A, Jain D
Semin Diagn Pathol 2021 Sep;38(5):83-89. Epub 2021 Jun 4 doi: 10.1053/j.semdp.2021.06.001. PMID: 34147303
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
Petralia F, Tignor N, Reva B, Koptyra M, Chowdhury S, Rykunov D, Krek A, Ma W, Zhu Y, Ji J, Calinawan A, Whiteaker JR, Colaprico A, Stathias V, Omelchenko T, Song X, Raman P, Guo Y, Brown MA, Ivey RG, Szpyt J, Guha Thakurta S, Gritsenko MA, Weitz KK, Lopez G, Kalayci S, Gümüş ZH, Yoo S, da Veiga Leprevost F, Chang HY, Krug K, Katsnelson L, Wang Y, Kennedy JJ, Voytovich UJ, Zhao L, Gaonkar KS, Ennis BM, Zhang B, Baubet V, Tauhid L, Lilly JV, Mason JL, Farrow B, Young N, Leary S, Moon J, Petyuk VA, Nazarian J, Adappa ND, Palmer JN, Lober RM, Rivero-Hinojosa S, Wang LB, Wang JM, Broberg M, Chu RK, Moore RJ, Monroe ME, Zhao R, Smith RD, Zhu J, Robles AI, Mesri M, Boja E, Hiltke T, Rodriguez H, Zhang B, Schadt EE, Mani DR, Ding L, Iavarone A, Wiznerowicz M, Schürer S, Chen XS, Heath AP, Rokita JL, Nesvizhskii AI, Fenyö D, Rodland KD, Liu T, Gygi SP, Paulovich AG, Resnick AC, Storm PB, Rood BR, Wang P; Children’s Brain Tumor Network; Clinical Proteomic Tumor Analysis Consortium
Cell 2020 Dec 23;183(7):1962-1985.e31. Epub 2020 Nov 25 doi: 10.1016/j.cell.2020.10.044. PMID: 33242424Free PMC Article

Recent systematic reviews

Egiz A, Kannan S, Asl SF
World Neurosurg 2022 Aug;164:216-227. Epub 2022 Apr 22 doi: 10.1016/j.wneu.2022.04.073. PMID: 35470083
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
Bunevicius A, Matukevicius A, Deltuva V, Gudinaviciene I, Pranys D, Tamasauskas A
World Neurosurg 2018 May;113:129-134. Epub 2018 Feb 13 doi: 10.1016/j.wneu.2018.02.012. PMID: 29452330
Spina A, Gagliardi F, Boari N, Bailo M, Mortini P
World Neurosurg 2017 Aug;104:612-618. Epub 2017 Apr 28 doi: 10.1016/j.wneu.2017.04.132. PMID: 28461278
Isaacs H Jr
J Pediatr Surg 2010 Mar;45(3):619-26. doi: 10.1016/j.jpedsurg.2009.12.011. PMID: 20223330

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