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Childhood Alveolar Rhabdomyosarcoma

MedGen UID:
83531
•Concept ID:
C0279613
•
Neoplastic Process
Synonyms: Alveolar Childhood Rhabdomyosarcoma; Pediatric Alveolar Rhabdomyosarcoma

Definition

An aggressive malignant mesenchymal neoplasm occurring in children. It is characterized by the presence of round cells with myoblastic differentiation, and a fibrovascular stroma resembling an alveolar growth pattern. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChildhood Alveolar Rhabdomyosarcoma

Professional guidelines

PubMed

Diagnosis and Management of Rhabdomyosarcoma in Children and Adolescents: ICMR Consensus Document.
Panda SP, Chinnaswamy G, Vora T, Prasad M, Bansal D, Kapoor G, Radhakrishnan V, Agarwala S, Laskar S, Arora B, Kaur T, Rath GK, Bakhshi S
Indian J Pediatr 2017 May;84(5):393-402. Epub 2017 Apr 5 doi: 10.1007/s12098-017-2315-3. PMID: 28378141
Molecular diagnostics in the management of rhabdomyosarcoma.
Arnold MA, Barr FG
Expert Rev Mol Diagn 2017 Feb;17(2):189-194. Epub 2017 Jan 6 doi: 10.1080/14737159.2017.1275965. PMID: 28058850Free PMC Article
Childhood rhabdomyosarcoma: new insight on biology and treatment.
Huh WW, Skapek SX
Curr Oncol Rep 2010 Nov;12(6):402-10. doi: 10.1007/s11912-010-0130-3. PMID: 20820958

Recent clinical studies

Therapy

Single-cell profiling of alveolar rhabdomyosarcoma reveals RAS pathway inhibitors as cell-fate hijackers with therapeutic relevance.
Danielli SG, Porpiglia E, De Micheli AJ, Navarro N, Zellinger MJ, Bechtold I, Kisele S, Volken L, Marques JG, Kasper S, Bode PK, Henssen AG, Gürgen D, Delattre O, Surdez D, Roma J, Bühlmann P, Blau HM, Wachtel M, Schäfer BW
Sci Adv 2023 Feb 10;9(6):eade9238. Epub 2023 Feb 8 doi: 10.1126/sciadv.ade9238. PMID: 36753540Free PMC Article
Neomorphic DNA-binding enables tumor-specific therapeutic gene expression in fusion-addicted childhood sarcoma.
Hölting TLB, Cidre-Aranaz F, Matzek D, Popper B, Jacobi SJ, Funk CM, Geyer FH, Li J, Piseddu I, Cadilha BL, Ledderose S, Zwilling J, Ohmura S, Anz D, Künkele A, Klauschen F, Grünewald TGP, Knott MML
Mol Cancer 2022 Oct 13;21(1):199. doi: 10.1186/s12943-022-01641-6. PMID: 36229873Free PMC Article
A Phase I Trial of TB-403 in Relapsed Medulloblastoma, Neuroblastoma, Ewing Sarcoma, and Alveolar Rhabdomyosarcoma.
Saulnier-Sholler G, Duda DG, Bergendahl G, Ebb D, Snuderl M, Laetsch TW, Michlitsch J, Hanson D, Isakoff MS, Bielamowicz K, Kraveka JM, Ferguson W, Carmeliet P, De Deene A, Gijsen L, Jain RK
Clin Cancer Res 2022 Sep 15;28(18):3950-3957. doi: 10.1158/1078-0432.CCR-22-1169. PMID: 35833850Free PMC Article
Outcome and Patterns of Relapse in Childhood Parameningeal Rhabdomyosarcoma Treated With Proton Beam Therapy.
Doyen J, Jazmati D, Geismar D, Frisch S, Schleithoff SS, Vermeren X, Scheer M, Blase C, Tippelt S, Timmermann B
Int J Radiat Oncol Biol Phys 2019 Dec 1;105(5):1043-1054. Epub 2019 Aug 13 doi: 10.1016/j.ijrobp.2019.08.005. PMID: 31419513
Childhood rhabdomyosarcoma: new insight on biology and treatment.
Huh WW, Skapek SX
Curr Oncol Rep 2010 Nov;12(6):402-10. doi: 10.1007/s11912-010-0130-3. PMID: 20820958

Prognosis

Genomic Classification and Clinical Outcome in Rhabdomyosarcoma: A Report From an International Consortium.
Shern JF, Selfe J, Izquierdo E, Patidar R, Chou HC, Song YK, Yohe ME, Sindiri S, Wei J, Wen X, Rudzinski ER, Barkauskas DA, Lo T, Hall D, Linardic CM, Hughes D, Jamal S, Jenney M, Chisholm J, Brown R, Jones K, Hicks B, Angelini P, George S, Chesler L, Hubank M, Kelsey A, Gatz SA, Skapek SX, Hawkins DS, Shipley JM, Khan J
J Clin Oncol 2021 Sep 10;39(26):2859-2871. Epub 2021 Jun 24 doi: 10.1200/JCO.20.03060. PMID: 34166060Free PMC Article
Transcription factor AP-2beta in development, differentiation and tumorigenesis.
Raap M, Gierendt L, Kreipe HH, Christgen M
Int J Cancer 2021 Sep 15;149(6):1221-1227. Epub 2021 Mar 26 doi: 10.1002/ijc.33558. PMID: 33720400
Diagnosis and Management of Rhabdomyosarcoma in Children and Adolescents: ICMR Consensus Document.
Panda SP, Chinnaswamy G, Vora T, Prasad M, Bansal D, Kapoor G, Radhakrishnan V, Agarwala S, Laskar S, Arora B, Kaur T, Rath GK, Bakhshi S
Indian J Pediatr 2017 May;84(5):393-402. Epub 2017 Apr 5 doi: 10.1007/s12098-017-2315-3. PMID: 28378141
Molecular diagnostics in the management of rhabdomyosarcoma.
Arnold MA, Barr FG
Expert Rev Mol Diagn 2017 Feb;17(2):189-194. Epub 2017 Jan 6 doi: 10.1080/14737159.2017.1275965. PMID: 28058850Free PMC Article
Childhood rhabdomyosarcoma: recent advances and prospective views.
Wang C
J Dent Res 2012 Apr;91(4):341-50. Epub 2011 Sep 13 doi: 10.1177/0022034511421490. PMID: 21917598Free PMC Article

Clinical prediction guides

A Phase I Trial of TB-403 in Relapsed Medulloblastoma, Neuroblastoma, Ewing Sarcoma, and Alveolar Rhabdomyosarcoma.
Saulnier-Sholler G, Duda DG, Bergendahl G, Ebb D, Snuderl M, Laetsch TW, Michlitsch J, Hanson D, Isakoff MS, Bielamowicz K, Kraveka JM, Ferguson W, Carmeliet P, De Deene A, Gijsen L, Jain RK
Clin Cancer Res 2022 Sep 15;28(18):3950-3957. doi: 10.1158/1078-0432.CCR-22-1169. PMID: 35833850Free PMC Article
Genomic Classification and Clinical Outcome in Rhabdomyosarcoma: A Report From an International Consortium.
Shern JF, Selfe J, Izquierdo E, Patidar R, Chou HC, Song YK, Yohe ME, Sindiri S, Wei J, Wen X, Rudzinski ER, Barkauskas DA, Lo T, Hall D, Linardic CM, Hughes D, Jamal S, Jenney M, Chisholm J, Brown R, Jones K, Hicks B, Angelini P, George S, Chesler L, Hubank M, Kelsey A, Gatz SA, Skapek SX, Hawkins DS, Shipley JM, Khan J
J Clin Oncol 2021 Sep 10;39(26):2859-2871. Epub 2021 Jun 24 doi: 10.1200/JCO.20.03060. PMID: 34166060Free PMC Article
Soft Tissue Special Issue: Skeletal Muscle Tumors: A Clinicopathological Review.
Kohashi K, Kinoshita I, Oda Y
Head Neck Pathol 2020 Mar;14(1):12-20. Epub 2020 Jan 16 doi: 10.1007/s12105-019-01113-2. PMID: 31950473Free PMC Article
Molecular diagnostics in the management of rhabdomyosarcoma.
Arnold MA, Barr FG
Expert Rev Mol Diagn 2017 Feb;17(2):189-194. Epub 2017 Jan 6 doi: 10.1080/14737159.2017.1275965. PMID: 28058850Free PMC Article
Classification of rhabdomyosarcoma and its molecular basis.
Parham DM, Barr FG
Adv Anat Pathol 2013 Nov;20(6):387-97. doi: 10.1097/PAP.0b013e3182a92d0d. PMID: 24113309Free PMC Article

Recent systematic reviews

Adult parameningial alveolar rhabdomyosarcoma: Case report and literature review.
Barthère X, Guillerm S, Quero L, Le Maignan C, Torossian N, Verillaud B, Itti R, Hennequin C
Cancer Radiother 2020 Dec;24(8):870-875. Epub 2020 Nov 7 doi: 10.1016/j.canrad.2020.03.015. PMID: 33172776

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