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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.

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.

Professional guidelines

PubMed

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
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
Gosiengfiao Y, Reichek J, Walterhouse D
Paediatr Drugs 2012 Dec 1;14(6):389-400. doi: 10.2165/11599440-000000000-00000. PMID: 23050743

Curated

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

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
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
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
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
Kikuchi K, Rubin BP, Keller C
Curr Top Dev Biol 2011;96:33-56. doi: 10.1016/B978-0-12-385940-2.00002-4. PMID: 21621066Free PMC Article

Diagnosis

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
Leiner J, Le Loarer F
Virchows Arch 2020 Jan;476(1):97-108. Epub 2019 Nov 6 doi: 10.1007/s00428-019-02676-9. PMID: 31696361
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
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
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

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
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
Lu H, Qin J, Wang Z
Clin Lab 2017 Oct 1;63(10):1745-1748. doi: 10.7754/Clin.Lab.2017.170529. PMID: 29035460
Katoh M, Nakagama H
Med Res Rev 2014 Mar;34(2):280-300. Epub 2013 May 21 doi: 10.1002/med.21288. PMID: 23696246
Curry S, Bacon CL, Robinson I, Capra M, Smith OP
Br J Haematol 2010 Nov;151(3):208. doi: 10.1111/j.1365-2141.2010.08338.x. PMID: 20813011

Prognosis

Bharathy N, Cleary MM, Kim JA, Nagamori K, Crawford KA, Wang E, Saha D, Settelmeyer TP, Purohit R, Skopelitis D, Chang K, Doran JA, Kirschbaum CW, Bharathy S, Crews DW, Randolph ME, Karnezis AN, Hudson-Price L, Dhawan J, Michalek JE, Ciulli A, Vakoc CR, Keller C
Oncogene 2022 Mar;41(11):1647-1656. Epub 2022 Jan 29 doi: 10.1038/s41388-022-02205-0. PMID: 35094009Free PMC Article
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
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
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
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

Clay MR, Patel A, Tran Q, Hedges DJ, Chang TC, Stewart E, Charville G, Cline C, Dyer MA, Orr BA
Sci Rep 2021 Nov 15;11(1):22213. doi: 10.1038/s41598-021-01649-w. PMID: 34782706Free PMC Article
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
Forgó E, Hornick JL, Charville GW
Histopathology 2021 May;78(6):905-908. Epub 2021 Mar 16 doi: 10.1111/his.14321. PMID: 33368602Free PMC Article
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
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

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
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
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
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
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

Table of contents

    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.

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