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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

Warden D, Cieply K, Armstrong B, Nelson M
BMJ Case Rep 2023 Mar 28;16(3) doi: 10.1136/bcr-2022-250699. PMID: 36977508Free PMC Article
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
Hornick JL
Ann Diagn Pathol 2018 Dec;37:118-124. Epub 2018 Oct 11 doi: 10.1016/j.anndiagpath.2018.10.006. PMID: 30340082
Liebner DA
J Surg Oncol 2015 Apr;111(5):622-31. Epub 2015 Jan 8 doi: 10.1002/jso.23866. PMID: 25581912
Akyol MU, Sözeri B, Küçükali T, Oğretmenoğlu O
Eur Arch Otorhinolaryngol 1998;255(6):307-10. doi: 10.1007/s004050050065. PMID: 9693927

Diagnosis

Cammareri C, Beltzung F, Michal M, Vanhersecke L, Coindre JM, Velasco V, Le Loarer F, Vergier B, Perret R
Virchows Arch 2023 Aug;483(2):145-156. Epub 2023 Jul 21 doi: 10.1007/s00428-023-03606-6. PMID: 37477762
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
Akyol MU, Sözeri B, Küçükali T, Oğretmenoğlu O
Eur Arch Otorhinolaryngol 1998;255(6):307-10. doi: 10.1007/s004050050065. PMID: 9693927
Hollowood K, Fletcher CD
Semin Diagn Pathol 1994 Feb;11(1):47-57. PMID: 8202646
Mohan Kumar K, Ramachandran P, Thyagarajan MP, Ramachandran G
J Indian Med Assoc 1971 Oct 1;57(7):248-50. PMID: 5141451

Therapy

Wu J, Wang Y, Zhang C
Clin Nucl Med 2021 Dec 1;46(12):1018-1019. doi: 10.1097/RLU.0000000000003716. PMID: 34034317
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
Cranmer LD, Chen CC, Morgan S, Martino G, Ray J
J Clin Oncol 2013 Mar 1;31(7):e108-10. Epub 2013 Jan 14 doi: 10.1200/JCO.2012.43.4910. PMID: 23319697
Scott RS, Jagirdar J
Ann Diagn Pathol 2007 Aug;11(4):274-6. doi: 10.1016/j.anndiagpath.2006.03.001. PMID: 17630111
Okada DH, Rowland JB, Petrovic LM
Gynecol Oncol 1999 Dec;75(3):509-13. doi: 10.1006/gyno.1999.5604. PMID: 10600318

Prognosis

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
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
Demir A, Onol FF, Türkeri L
Int Urol Nephrol 2004;36(4):577-8. doi: 10.1007/s11255-004-0853-x. PMID: 15787340
Akyol MU, Sözeri B, Küçükali T, Oğretmenoğlu O
Eur Arch Otorhinolaryngol 1998;255(6):307-10. doi: 10.1007/s004050050065. PMID: 9693927
Bramwell VH, Littley MB, Chang J, Crowther D
Eur J Cancer Clin Oncol 1982 Nov;18(11):1099-106. doi: 10.1016/0277-5379(82)90090-6. PMID: 6297916

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
Wu J, Wang Y, Zhang C
Clin Nucl Med 2021 Dec 1;46(12):1018-1019. doi: 10.1097/RLU.0000000000003716. PMID: 34034317
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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