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Alveolar rhabdomyosarcoma(RMS2)

MedGen UID:
61651
•Concept ID:
C0206655
•
Neoplastic Process
Synonyms: Alveolar rhabdomyosarcoma (disease); RHABDOMYOSARCOMA 2; RMS2
SNOMED CT: Alveolar rhabdomyosarcoma (63449009); Alveolar rhabdomyosarcoma (404053004)
 
Genes (locations): FOXO1 (13q14.11); PAX3 (2q36.1); PAX7 (1p36.13)
 
HPO: HP:0006779
Monarch Initiative: MONDO:0009994
OMIM®: 268220
Orphanet: ORPHA99756

Definition

A rapidly growing malignant mesenchymal neoplasm. It is characterized by the presence of round cells with myoblastic differentiation and a fibrovascular stroma resembling an alveolar growth pattern. The tumor usually presents in the extremities. [from NCI]

Clinical features

From HPO
Alveolar rhabdomyosarcoma
MedGen UID:
61651
•Concept ID:
C0206655
•
Neoplastic Process
A rapidly growing malignant mesenchymal neoplasm. It is characterized by the presence of round cells with myoblastic differentiation and a fibrovascular stroma resembling an alveolar growth pattern. The tumor usually presents in the extremities.
See: Feature record | Search on this feature

Conditions with this feature

Alveolar rhabdomyosarcoma
MedGen UID:
61651
•Concept ID:
C0206655
•
Neoplastic Process
A rapidly growing malignant mesenchymal neoplasm. It is characterized by the presence of round cells with myoblastic differentiation and a fibrovascular stroma resembling an alveolar growth pattern. The tumor usually presents in the extremities.
See: Condition Record
Alveolar rhabdomyosarcoma

Professional guidelines

PubMed

GEIS-SEHOP clinical practice guidelines for the treatment of rhabdomyosarcoma.
Gallego S, Bernabeu D, Garrido-Pontnou M, Guillen G, Hindi N, Juan-Ribelles A, Márquez C, Mata C, Orcajo J, Ramírez G, Ramos M, Romagosa C, Ruano D, Rubio P, Vergés R, Valverde C; GEIS (Grupo Español de Investigación en Sarcomas), SEHOP (Sociedad Española de Hematología y Oncología Pediátrica)
Clin Transl Oncol 2021 Dec;23(12):2460-2473. Epub 2021 Jul 1 doi: 10.1007/s12094-021-02654-1. PMID: 34212338
Novel pathways and molecular targets for the treatment of sarcoma.
Frith AE, Hirbe AC, Van Tine BA
Curr Oncol Rep 2013 Aug;15(4):378-85. doi: 10.1007/s11912-013-0319-3. PMID: 23661264
What is new in rhabdomyosarcoma management in children?
Gosiengfiao Y, Reichek J, Walterhouse D
Paediatr Drugs 2012 Dec 1;14(6):389-400. doi: 10.2165/11599440-000000000-00000. PMID: 23050743

Curated

Clinical utility gene card for: Alveolar rhabdomyosarcoma.
Yu Z, Kelsey A, Alaggio R, Parham D
Eur J Hum Genet 2012 Jan;20(1) Epub 2011 Aug 10 doi: 10.1038/ejhg.2011.147. PMID: 21829230Free PMC Article

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

Recent clinical studies

Etiology

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
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
Vinorelbine and continuous low-dose cyclophosphamide as maintenance chemotherapy in patients with high-risk rhabdomyosarcoma (RMS 2005): a multicentre, open-label, randomised, phase 3 trial.
Bisogno G, De Salvo GL, Bergeron C, Gallego Melcón S, Merks JH, Kelsey A, Martelli H, Minard-Colin V, Orbach D, Glosli H, Chisholm J, Casanova M, Zanetti I, Devalck C, Ben-Arush M, Mudry P, Ferman S, Jenney M, Ferrari A; European paediatric Soft tissue sarcoma Study Group
Lancet Oncol 2019 Nov;20(11):1566-1575. Epub 2019 Sep 24 doi: 10.1016/S1470-2045(19)30617-5. PMID: 31562043
Rhabdomyosarcoma.
Skapek SX, Ferrari A, Gupta AA, Lupo PJ, Butler E, Shipley J, Barr FG, Hawkins DS
Nat Rev Dis Primers 2019 Jan 7;5(1):1. doi: 10.1038/s41572-018-0051-2. PMID: 30617281Free PMC Article
Dysregulated Myogenesis in Rhabdomyosarcoma.
Yu PY, Guttridge DC
Curr Top Dev Biol 2018;126:285-297. Epub 2017 Oct 31 doi: 10.1016/bs.ctdb.2017.10.007. PMID: 29305002

Diagnosis

Therapeutic targeting of ATR in alveolar rhabdomyosarcoma.
Dorado García H, Pusch F, Bei Y, von Stebut J, Ibáñez G, Guillan K, Imami K, Gürgen D, Rolff J, Helmsauer K, Meyer-Liesener S, Timme N, Bardinet V, Chamorro González R, MacArthur IC, Chen CY, Schulz J, Wengner AM, Furth C, Lala B, Eggert A, Seifert G, Hundsoerfer P, Kirchner M, Mertins P, Selbach M, Lissat A, Dubois F, Horst D, Schulte JH, Spuler S, You D, Dela Cruz F, Kung AL, Haase K, DiVirgilio M, Scheer M, Ortiz MV, Henssen AG
Nat Commun 2022 Jul 25;13(1):4297. doi: 10.1038/s41467-022-32023-7. PMID: 35879366Free PMC Article
Ewing sarcoma and Ewing-like tumors.
Sbaraglia M, Righi A, Gambarotti M, Dei Tos AP
Virchows Arch 2020 Jan;476(1):109-119. Epub 2019 Dec 4 doi: 10.1007/s00428-019-02720-8. PMID: 31802230
Rhabdomyosarcoma.
Skapek SX, Ferrari A, Gupta AA, Lupo PJ, Butler E, Shipley J, Barr FG, Hawkins DS
Nat Rev Dis Primers 2019 Jan 7;5(1):1. doi: 10.1038/s41572-018-0051-2. PMID: 30617281Free PMC Article
Childhood rhabdomyosarcoma.
Córdoba Rovira SM, Inarejos Clemente EJ
Radiologia 2016 Nov-Dec;58(6):481-490. Epub 2016 Nov 1 doi: 10.1016/j.rx.2016.09.003. PMID: 27810092
FGF receptors: cancer biology and therapeutics.
Katoh M, Nakagama H
Med Res Rev 2014 Mar;34(2):280-300. Epub 2013 May 21 doi: 10.1002/med.21288. PMID: 23696246

Therapy

Entinostat as a combinatorial therapeutic for rhabdomyosarcoma.
Chauhan S, Lian E, Habib I, Liu Q, Anders NM, Bugg MM, Federman NC, Reid JM, Stewart CF, Cates T, Michalek JE, Keller C
Sci Rep 2024 Aug 15;14(1):18936. doi: 10.1038/s41598-024-66545-5. PMID: 39147820Free PMC Article
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
Vinorelbine and continuous low-dose cyclophosphamide as maintenance chemotherapy in patients with high-risk rhabdomyosarcoma (RMS 2005): a multicentre, open-label, randomised, phase 3 trial.
Bisogno G, De Salvo GL, Bergeron C, Gallego Melcón S, Merks JH, Kelsey A, Martelli H, Minard-Colin V, Orbach D, Glosli H, Chisholm J, Casanova M, Zanetti I, Devalck C, Ben-Arush M, Mudry P, Ferman S, Jenney M, Ferrari A; European paediatric Soft tissue sarcoma Study Group
Lancet Oncol 2019 Nov;20(11):1566-1575. Epub 2019 Sep 24 doi: 10.1016/S1470-2045(19)30617-5. PMID: 31562043
Pleural Alveolar Rhabdomyosarcoma.
Lu H, Qin J, Wang Z
Clin Lab 2017 Oct 1;63(10):1745-1748. doi: 10.7754/Clin.Lab.2017.170529. PMID: 29035460
FGF receptors: cancer biology and therapeutics.
Katoh M, Nakagama H
Med Res Rev 2014 Mar;34(2):280-300. Epub 2013 May 21 doi: 10.1002/med.21288. PMID: 23696246

Prognosis

Entinostat as a combinatorial therapeutic for rhabdomyosarcoma.
Chauhan S, Lian E, Habib I, Liu Q, Anders NM, Bugg MM, Federman NC, Reid JM, Stewart CF, Cates T, Michalek JE, Keller C
Sci Rep 2024 Aug 15;14(1):18936. doi: 10.1038/s41598-024-66545-5. PMID: 39147820Free 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
Ewing sarcoma and Ewing-like tumors.
Sbaraglia M, Righi A, Gambarotti M, Dei Tos AP
Virchows Arch 2020 Jan;476(1):109-119. Epub 2019 Dec 4 doi: 10.1007/s00428-019-02720-8. PMID: 31802230
Dysregulated Myogenesis in Rhabdomyosarcoma.
Yu PY, Guttridge DC
Curr Top Dev Biol 2018;126:285-297. Epub 2017 Oct 31 doi: 10.1016/bs.ctdb.2017.10.007. PMID: 29305002
Pleural Alveolar Rhabdomyosarcoma.
Lu H, Qin J, Wang Z
Clin Lab 2017 Oct 1;63(10):1745-1748. doi: 10.7754/Clin.Lab.2017.170529. PMID: 29035460

Clinical prediction guides

Entinostat as a combinatorial therapeutic for rhabdomyosarcoma.
Chauhan S, Lian E, Habib I, Liu Q, Anders NM, Bugg MM, Federman NC, Reid JM, Stewart CF, Cates T, Michalek JE, Keller C
Sci Rep 2024 Aug 15;14(1):18936. doi: 10.1038/s41598-024-66545-5. PMID: 39147820Free 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
Ewing sarcoma and Ewing-like tumors.
Sbaraglia M, Righi A, Gambarotti M, Dei Tos AP
Virchows Arch 2020 Jan;476(1):109-119. Epub 2019 Dec 4 doi: 10.1007/s00428-019-02720-8. PMID: 31802230
Rhabdomyosarcoma in children - current pathologic and molecular classification.
Dziuba I, Kurzawa P, Dopierała M, Larque AB, Januszkiewicz-Lewandowska D
Pol J Pathol 2018;69(1):20-32. doi: 10.5114/pjp.2018.75333. PMID: 29895123

Recent systematic reviews

A systematic review of the prevalence of pathogenic or likely pathogenic germline variants in individuals with FOXO1 fusion-positive rhabdomyosarcoma.
Freycon C, Lupo PJ, Witkowski L, Budd C, Foulkes WD, Goudie C
Pediatr Blood Cancer 2023 Nov;70(11):e30651. Epub 2023 Aug 28 doi: 10.1002/pbc.30651. PMID: 37638828
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
Anesthetic Management During Pediatric Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy With Cisplatin in a Small Child: A Case Report and Systematic Literature Review.
Zajonz TS, Padberg W, Mann STW, Gehron J, Sander M, Mann V
A A Pract 2020 Jan 1;14(1):1-5. doi: 10.1213/XAA.0000000000001122. PMID: 31651415
Change of Circulating and Tissue-Based miR-20a in Human Cancers and Associated Prognostic Implication: A Systematic Review and Meta-Analysis.
Zhang Q, Wang Q, Sun W, Gao F, Liu L, Cheng L, Li Z
Biomed Res Int 2018;2018:6124927. Epub 2018 Nov 25 doi: 10.1155/2018/6124927. PMID: 30596096Free PMC Article
Prognostic value of PAX3/7-FOXO1 fusion status in alveolar rhabdomyosarcoma: Systematic review and meta-analysis.
Kubo T, Shimose S, Fujimori J, Furuta T, Ochi M
Crit Rev Oncol Hematol 2015 Oct;96(1):46-53. Epub 2015 May 14 doi: 10.1016/j.critrevonc.2015.04.012. PMID: 26008753

Supplemental Content

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    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024
    • EuroGenetest, 2011
      Clinical utility gene card for: Alveolar rhabdomyosarcoma.

    Reviews

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