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Pleomorphic rhabdomyosarcoma

MedGen UID:
137770
Concept ID:
C0334480
Neoplastic Process
Synonyms: anaplastic rhabdomyosarcoma; Pleomorphic Rhabdomyosarcoma; pleomorphic rhabdomyosarcoma; pleomorphic rhabdomyosarcoma, adult type
SNOMED CT: Pleomorphic rhabdomyosarcoma (404054005)
 
Monarch Initiative: MONDO:0017386
Orphanet: ORPHA293199

Definition

A rare soft tissue sarcoma characterized by a high-grade lesion occurring almost exclusively in adults, composed of bizarre polygonal, round, and spindle cells with evidence of skeletal muscle differentiation. Patients usually present with a rapidly growing, painful mass located in the deep soft tissues of the extremities, but also other anatomic regions. Prognosis is generally poor. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPleomorphic rhabdomyosarcoma
Follow this link to review classifications for Pleomorphic rhabdomyosarcoma in Orphanet.

Professional guidelines

PubMed

Agaimy A, Stoehr R, Hornung A, Popp J, Erdmann M, Heinzerling L, Hartmann A
Am J Surg Pathol 2021 Feb 1;45(2):240-254. doi: 10.1097/PAS.0000000000001645. PMID: 33428337
Liebner DA
J Surg Oncol 2015 Apr;111(5):622-31. Epub 2015 Jan 8 doi: 10.1002/jso.23866. PMID: 25581912
Gaffney EF, Dervan PA, Fletcher CD
Am J Surg Pathol 1993 Jun;17(6):601-9. PMID: 8333559

Recent clinical studies

Etiology

Fan R, Parham DM, Wang LL
Arch Pathol Lab Med 2022 Aug 1;146(8):953-959. doi: 10.5858/arpa.2021-0183-RA. PMID: 35051261
Steiner D, Smolle MA, Brcic I, Leithner A
Wien Klin Wochenschr 2022 Jan;134(1-2):76-79. Epub 2021 Apr 27 doi: 10.1007/s00508-021-01872-5. PMID: 33905030Free PMC Article
Hornick JL
Ann Diagn Pathol 2018 Dec;37:118-124. Epub 2018 Oct 11 doi: 10.1016/j.anndiagpath.2018.10.006. PMID: 30340082
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
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739

Diagnosis

Poumeaud F, Valentin T, Vande Perre P, Jaffrelot M, Bonnet D, Chibon F, Chevreau C, Selves J, Guimbaud R, Fares N
Crit Rev Oncol Hematol 2023 Aug;188:104055. Epub 2023 Jun 8 doi: 10.1016/j.critrevonc.2023.104055. PMID: 37301271
Fan R, Parham DM, Wang LL
Arch Pathol Lab Med 2022 Aug 1;146(8):953-959. doi: 10.5858/arpa.2021-0183-RA. PMID: 35051261
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
Hornick JL
Ann Diagn Pathol 2018 Dec;37:118-124. Epub 2018 Oct 11 doi: 10.1016/j.anndiagpath.2018.10.006. PMID: 30340082
Hollowood K, Fletcher CD
Semin Diagn Pathol 1994 Feb;11(1):47-57. PMID: 8202646

Therapy

Benkhaled S, Mané M, Jungels C, Shumelinsky F, Aubain NS, Van Gestel D
Cancer Treat Res Commun 2021;26:100282. Epub 2020 Dec 11 doi: 10.1016/j.ctarc.2020.100282. PMID: 33360328
Kollár A, Langer R, Ionescu C, Cullmann JL, Klenke FM
Tumori 2016 Nov 11;102(Suppl. 2) doi: 10.5301/tj.5000476. PMID: 26952562
Noujaim J, Thway K, Jones RL, Miah A, Khabra K, Langer R, Kasper B, Judson I, Benson C, Kollàr A
Anticancer Res 2015 Nov;35(11):6213-7. PMID: 26504053
van der Graaf WT, Gelderblom H
Curr Treat Options Oncol 2012 Sep;13(3):306-17. doi: 10.1007/s11864-012-0196-2. PMID: 22661203Free PMC Article
Harlow PJ, Kaufman FR, Siegel SE, Quevedo E
Med Pediatr Oncol 1979;7(2):123-6. doi: 10.1002/mpo.2950070205. PMID: 388175

Prognosis

Beird HC, Wu CC, Nakazawa M, Ingram D, Daniele JR, Lazcano R, Little L, Davies C, Daw NC, Wani K, Wang WL, Song X, Gumbs C, Zhang J, Rubin B, Conley A, Flanagan AM, Lazar AJ, Futreal PA
HGG Adv 2023 Oct 12;4(4):100224. Epub 2023 Jul 19 doi: 10.1016/j.xhgg.2023.100224. PMID: 37593416Free PMC Article
Steiner D, Smolle MA, Brcic I, Leithner A
Wien Klin Wochenschr 2022 Jan;134(1-2):76-79. Epub 2021 Apr 27 doi: 10.1007/s00508-021-01872-5. PMID: 33905030Free PMC Article
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739
Schrock A, Jakob M, Zhou H, Bootz F
Auris Nasus Larynx 2007 Dec;34(4):553-6. Epub 2007 May 3 doi: 10.1016/j.anl.2007.02.009. PMID: 17481838
Agamanolis DP, Dasu S, Krill CE Jr
Hum Pathol 1986 Aug;17(8):778-95. doi: 10.1016/s0046-8177(86)80198-8. PMID: 3525381

Clinical prediction guides

Kobayashi H, Okajima K, Zhang L, Hirai T, Ishibashi Y, Tsuda Y, Ikegami M, Kawai A, Tanaka S
Jpn J Clin Oncol 2024 Apr 6;54(4):471-478. doi: 10.1093/jjco/hyad188. PMID: 38183215
Beird HC, Wu CC, Nakazawa M, Ingram D, Daniele JR, Lazcano R, Little L, Davies C, Daw NC, Wani K, Wang WL, Song X, Gumbs C, Zhang J, Rubin B, Conley A, Flanagan AM, Lazar AJ, Futreal PA
HGG Adv 2023 Oct 12;4(4):100224. Epub 2023 Jul 19 doi: 10.1016/j.xhgg.2023.100224. PMID: 37593416Free PMC Article
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
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
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739

Recent systematic reviews

Poumeaud F, Valentin T, Vande Perre P, Jaffrelot M, Bonnet D, Chibon F, Chevreau C, Selves J, Guimbaud R, Fares N
Crit Rev Oncol Hematol 2023 Aug;188:104055. Epub 2023 Jun 8 doi: 10.1016/j.critrevonc.2023.104055. PMID: 37301271
Deb PQ, Chokshi RJ, Li S, Suster DI
Int J Surg Pathol 2023 Aug;31(5):548-556. Epub 2022 Jun 16 doi: 10.1177/10668969221105622. PMID: 35707991
Li ZJ, Li CL, Wang W, Fu XY, Zhen YQ
J Int Med Res 2021 May;49(5):3000605211014360. doi: 10.1177/03000605211014360. PMID: 34034549Free PMC Article
Alavi S, Eckes L, Kratschell R, Taube ET, Rosseck F, Wieners G, Sehouli J
Anticancer Res 2017 May;37(5):2509-2514. doi: 10.21873/anticanres.11592. PMID: 28476820

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