U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Childhood pleomorphic rhabdomyosarcoma

MedGen UID:
76002
•Concept ID:
C0279614
•
Neoplastic Process
Synonyms: childhood anaplastic rhabdomyosarcoma; Childhood Pleomorphic Rhabdomyosarcoma; childhood pleomorphic rhabdomyosarcoma; paediatric pleomorphic rhabdomyosarcoma; Pediatric Pleomorphic Rhabdomyosarcoma; pediatric pleomorphic rhabdomyosarcoma; Pleomorphic Childhood Rhabdomyosarcoma; pleomorphic childhood rhabdomyosarcoma; Pleomorphic rhabdomyosarcoma; pleomorphic rhabdomyosarcoma of childhood
 
Monarch Initiative: MONDO:0004233

Definition

A rare aggressive rhabdomyosarcoma occurring in children. The neoplasm is characterized by the presence of bizarre round, spindle, and polygonal cells. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChildhood pleomorphic rhabdomyosarcoma

Professional guidelines

PubMed

Rhabdomyosarcoma: From Obscurity to Clarity in Diagnosis … But With Ongoing Challenges in Management: The Farber-Landing Lecture of 2020.
Parham DM, Giannikopoulos P
Pediatr Dev Pathol 2021 Mar-Apr;24(2):87-95. Epub 2021 Jan 13 doi: 10.1177/1093526620977720. PMID: 33439112
Adult rhabdomyosarcoma: outcome following multimodality treatment.
Little DJ, Ballo MT, Zagars GK, Pisters PW, Patel SR, El-Naggar AK, Garden AS, Benjamin RS
Cancer 2002 Jul 15;95(2):377-88. doi: 10.1002/cncr.10669. PMID: 12124838
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

Childhood rhabdomyosarcoma: a review of 35 cases and literature.
Mandong BM, Ngbea JA
Niger J Med 2011 Oct-Dec;20(4):466-9. PMID: 22288325
Rhabdomyosarcomas in adults and children: an update.
Parham DM, Ellison DA
Arch Pathol Lab Med 2006 Oct;130(10):1454-65. doi: 10.5858/2006-130-1454-RIAACA. PMID: 17090187
Pathologic classification of rhabdomyosarcomas and correlations with molecular studies.
Parham DM
Mod Pathol 2001 May;14(5):506-14. doi: 10.1038/modpathol.3880339. PMID: 11353062
Childhood rhabdomyosarcoma with anaplastic (pleomorphic) features. A report of the Intergroup Rhabdomyosarcoma Study.
Kodet R, Newton WA Jr, Hamoudi AB, Asmar L, Jacobs DL, Maurer HM
Am J Surg Pathol 1993 May;17(5):443-53. doi: 10.1097/00000478-199305000-00002. PMID: 8470759
Rhabdomyosarcoma in childhood.
Bale PM, Reye RD
Pathology 1975 Apr;7(2):101-11. doi: 10.3109/00313027509092704. PMID: 1153222

Diagnosis

Primary alveolar rhabdomyosarcoma of bone.
Tordecilla C J, Mosso Ch C, Franco S C, Díaz P E, Lobos R G
Andes Pediatr 2021 Jun;92(3):440-445. doi: 10.32641/andespediatr.v92i3.2613. PMID: 34479252
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
Rhabdomyosarcoma in an adult hand.
Seyhan N, Keskin M, Tosun Z, Savaci N
J Plast Surg Hand Surg 2013 Oct;47(5):422-4. Epub 2013 Jan 29 doi: 10.3109/2000656X.2012.731580. PMID: 23356943
The diagnosis and classification of childhood rhabdomyosarcoma.
Tsokos M
Semin Diagn Pathol 1994 Feb;11(1):26-38. PMID: 8202644
Childhood rhabdomyosarcoma with anaplastic (pleomorphic) features. A report of the Intergroup Rhabdomyosarcoma Study.
Kodet R, Newton WA Jr, Hamoudi AB, Asmar L, Jacobs DL, Maurer HM
Am J Surg Pathol 1993 May;17(5):443-53. doi: 10.1097/00000478-199305000-00002. PMID: 8470759

Therapy

Rhabdomyosarcoma associated with germline TP53 alteration in children and adolescents: The French experience.
Pondrom M, Bougeard G, Karanian M, Bonneau-Lagacherie J, Boulanger C, Boutroux H, Briandet C, Chevreau C, Corradini N, Coze C, Defachelles AS, Galmiche-Roland L, Orbach D, Piguet C, Scoazec JY, Vérité C, Willems M, Frebourg T, Minard V, Brugières L
Pediatr Blood Cancer 2020 Sep;67(9):e28486. Epub 2020 Jul 13 doi: 10.1002/pbc.28486. PMID: 32658383
Defining an embryonal rhabdomyosarcoma endotype.
Ricker CA, Crawford K, Matlock K, Lathara M, Seguin B, Rudzinski ER, Berlow NE, Keller C
Cold Spring Harb Mol Case Stud 2020 Apr;6(2) Epub 2020 Apr 1 doi: 10.1101/mcs.a005066. PMID: 32238403Free PMC Article
Anaplastic (pleomorphic) subtype embryonal rhabdomyosarcoma of the cervix.
Caruso RA, Napoli P, Villari D, Starrantino M
Arch Gynecol Obstet 2004 Dec;270(4):278-80. Epub 2003 Aug 26 doi: 10.1007/s00404-003-0504-y. PMID: 12942264
Histopathology of childhood sarcomas, Intergroup Rhabdomyosarcoma Studies I and II: clinicopathologic correlation.
Newton WA Jr, Soule EH, Hamoudi AB, Reiman HM, Shimada H, Beltangady M, Maurer H
J Clin Oncol 1988 Jan;6(1):67-75. doi: 10.1200/JCO.1988.6.1.67. PMID: 3275751

Prognosis

Primary alveolar rhabdomyosarcoma of bone.
Tordecilla C J, Mosso Ch C, Franco S C, Díaz P E, Lobos R G
Andes Pediatr 2021 Jun;92(3):440-445. doi: 10.32641/andespediatr.v92i3.2613. PMID: 34479252
Defining an embryonal rhabdomyosarcoma endotype.
Ricker CA, Crawford K, Matlock K, Lathara M, Seguin B, Rudzinski ER, Berlow NE, Keller C
Cold Spring Harb Mol Case Stud 2020 Apr;6(2) Epub 2020 Apr 1 doi: 10.1101/mcs.a005066. PMID: 32238403Free PMC Article
Rhabdomyosarcoma in an adult hand.
Seyhan N, Keskin M, Tosun Z, Savaci N
J Plast Surg Hand Surg 2013 Oct;47(5):422-4. Epub 2013 Jan 29 doi: 10.3109/2000656X.2012.731580. PMID: 23356943
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
Rhabdomyosarcoma in childhood.
Bale PM, Reye RD
Pathology 1975 Apr;7(2):101-11. doi: 10.3109/00313027509092704. PMID: 1153222

Clinical prediction guides

Anaplasia in childhood rhabdomyosarcoma: An under reported entity.
Ahuja M, Tomar R, Thakur S, Mallya V, Mandal S, Khurana N, Sarin YK
Indian J Pathol Microbiol 2022 Oct-Dec;65(4):864-868. doi: 10.4103/ijpm.ijpm_178_21. PMID: 36308195
Rhabdomyosarcoma: From Obscurity to Clarity in Diagnosis … But With Ongoing Challenges in Management: The Farber-Landing Lecture of 2020.
Parham DM, Giannikopoulos P
Pediatr Dev Pathol 2021 Mar-Apr;24(2):87-95. Epub 2021 Jan 13 doi: 10.1177/1093526620977720. PMID: 33439112
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
Comparing adult and pediatric rhabdomyosarcoma in the surveillance, epidemiology and end results program, 1973 to 2005: an analysis of 2,600 patients.
Sultan I, Qaddoumi I, Yaser S, Rodriguez-Galindo C, Ferrari A
J Clin Oncol 2009 Jul 10;27(20):3391-7. Epub 2009 Apr 27 doi: 10.1200/JCO.2008.19.7483. PMID: 19398574
Histopathology of childhood sarcomas, Intergroup Rhabdomyosarcoma Studies I and II: clinicopathologic correlation.
Newton WA Jr, Soule EH, Hamoudi AB, Reiman HM, Shimada H, Beltangady M, Maurer H
J Clin Oncol 1988 Jan;6(1):67-75. doi: 10.1200/JCO.1988.6.1.67. PMID: 3275751

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.

    Consumer resources

    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...