U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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

Dedifferentiated and Undifferentiated Melanomas: Report of 35 New Cases With Literature Review and Proposal of Diagnostic Criteria.
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
The indications and efficacy of conventional chemotherapy in primary and recurrent sarcoma.
Liebner DA
J Surg Oncol 2015 Apr;111(5):622-31. Epub 2015 Jan 8 doi: 10.1002/jso.23866. PMID: 25581912
Multiplex RT-PCR assay for the differential diagnosis of alveolar rhabdomyosarcoma and Ewing's sarcoma.
Downing JR, Khandekar A, Shurtleff SA, Head DR, Parham DM, Webber BL, Pappo AS, Hulshof MG, Conn WP, Shapiro DN
Am J Pathol 1995 Mar;146(3):626-34. PMID: 7887445Free PMC Article

Recent clinical studies

Etiology

An Integrative Morphologic and Molecular Approach for Diagnosis and Subclassification of Rhabdomyosarcoma.
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
Methylation profiling reveals novel molecular classes of rhabdomyosarcoma.
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
Subclassification of pleomorphic sarcomas: How and why should we care?
Hornick JL
Ann Diagn Pathol 2018 Dec;37:118-124. Epub 2018 Oct 11 doi: 10.1016/j.anndiagpath.2018.10.006. PMID: 30340082
Developmental origins of fusion-negative rhabdomyosarcomas.
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
Laryngeal pleomorphic rhabdomyosarcoma.
Akyol MU, Sözeri B, Küçükali T, Oğretmenoğlu O
Eur Arch Otorhinolaryngol 1998;255(6):307-10. doi: 10.1007/s004050050065. PMID: 9693927

Diagnosis

PRAME immunohistochemistry in soft tissue tumors and mimics: a study of 350 cases highlighting its imperfect specificity but potentially useful diagnostic applications.
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
An Integrative Morphologic and Molecular Approach for Diagnosis and Subclassification of Rhabdomyosarcoma.
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
The current landscape of rhabdomyosarcomas: an update.
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
Subclassification of pleomorphic sarcomas: How and why should we care?
Hornick JL
Ann Diagn Pathol 2018 Dec;37:118-124. Epub 2018 Oct 11 doi: 10.1016/j.anndiagpath.2018.10.006. PMID: 30340082
Rhabdomyosarcoma in adults.
Hollowood K, Fletcher CD
Semin Diagn Pathol 1994 Feb;11(1):47-57. PMID: 8202646

Therapy

Successful treatment of synchronous chemoresistant pulmonary metastasis from pleomorphic rhabdomyosarcoma with stereotaxic body radiation therapy: A case report and a review of the literature.
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
Pleomorphic rhabdomyosarcoma with an impressive response to chemotherapy: case report and review of the literature.
Kollár A, Langer R, Ionescu C, Cullmann JL, Klenke FM
Tumori 2016 Nov 11;102(Suppl. 2) doi: 10.5301/tj.5000476. PMID: 26952562
Adult Pleomorphic Rhabdomyosarcoma: A Multicentre Retrospective Study.
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
Para testicular rhabdomyosarcoma in adults: three case reports and review of literature.
Boudahna L, Benbrahim Z, Amaadour L, Mazouz A, Benhayoune K, Tahiri Y, Farih MH, Amarti A, Arifi S, Mellas N
Pan Afr Med J 2014;19:279. Epub 2014 Nov 14 doi: 10.11604/pamj.2014.19.279.4784. PMID: 25870734Free PMC Article
New systemic therapy options for advanced sarcomas.
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

Prognosis

Methylation profiling reveals novel molecular classes of rhabdomyosarcoma.
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
Heterologous and rare homologous sarcomas of the uterine corpus: a clinicopathologic review.
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739
Pleomorphic rhabdomyosarcoma of the diaphragm.
Chatterjee JS, Powell AP, Chatterjee D
J Natl Med Assoc 2005 Jan;97(1):95-8. PMID: 15719879Free PMC Article
Laryngeal pleomorphic rhabdomyosarcoma.
Akyol MU, Sözeri B, Küçükali T, Oğretmenoğlu O
Eur Arch Otorhinolaryngol 1998;255(6):307-10. doi: 10.1007/s004050050065. PMID: 9693927
Tumors of skeletal muscle.
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

Prognostic factors and treatment outcomes in patients with pleomorphic rhabdomyosarcoma: a population-based cohort study.
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
Complete loss of TP53 and RB1 is associated with complex genome and low immune infiltrate in pleomorphic rhabdomyosarcoma.
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
Methylation profiling reveals novel molecular classes of rhabdomyosarcoma.
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
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
Heterologous and rare homologous sarcomas of the uterine corpus: a clinicopathologic review.
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739

Recent systematic reviews

Special features of sarcomas developed in patients with Lynch syndrome: A systematic review.
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
Pleomorphic Rhabdomyosarcoma: A Systematic Review with Outcome Analysis and Report of a Rare Abdominal Wall Lesion.
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
Diagnosis and treatment of pleomorphic rhabdomyosarcoma of the uterus: a rare case report and review of the literature.
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
Pleomorphic Rhabdomyosarcoma of the Uterus - Case Report and a Systematic Review of the Literature.
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

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.

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...