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Cardiac Rhabdomyosarcoma

MedGen UID:
316475
•Concept ID:
C1704616
•
Neoplastic Process
Synonym: Heart Rhabdomyosarcoma

Definition

A rare malignant mesenchymal tumor with skeletal muscle differentiation arising within the myocardium. It is characterized by the presence of small round spindle cells. Most cardiac rhabodomyosarcomas are of embryonal type and usually present in children and young adults. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCardiac Rhabdomyosarcoma

Professional guidelines

PubMed

Cardiac Tumors: Clinical Presentation, Diagnosis, and Management.
Poterucha TJ, Kochav J, O'Connor DS, Rosner GF
Curr Treat Options Oncol 2019 Jun 27;20(8):66. doi: 10.1007/s11864-019-0662-1. PMID: 31250250
Treatment including anthracyclines versus treatment not including anthracyclines for childhood cancer.
van Dalen EC, Raphaël MF, Caron HN, Kremer LC
Cochrane Database Syst Rev 2014 Sep 4;2014(9):CD006647. doi: 10.1002/14651858.CD006647.pub4. PMID: 25188452Free PMC Article
Cardiac tumors--diagnosis and surgical treatment.
Hoffmeier A, Sindermann JR, Scheld HH, Martens S
Dtsch Arztebl Int 2014 Mar 21;111(12):205-11. doi: 10.3238/arztebl.2014.0205. PMID: 24717305Free PMC Article

Recent clinical studies

Etiology

A triad: cardiac rhabdomyosarcoma, stroke and tamponade.
Ashraf T, Day TG, Marek J, Hughes M, Giardini A
Pediatr Cardiol 2013 Mar;34(3):771-3. Epub 2012 Oct 7 doi: 10.1007/s00246-012-0549-4. PMID: 23052680
A rare cause of recurrent stroke in childhood: left atrial rhabdomyosarcoma.
Serdaroglu G, Yilmaz S, Ulger Z, Gökce S, Tekgul H, Gokben S
Acta Paediatr 2011 Oct;100(10):e189-91. Epub 2011 Apr 8 doi: 10.1111/j.1651-2227.2011.02214.x. PMID: 21352362
Primary cardiac rhabdomyosarcoma following a uterine leiomyosarcoma: double primary sarcomas.
Tsutsumi K, Aida Y, Ohno T, Ookura M
Jpn J Thorac Cardiovasc Surg 2005 Aug;53(8):458-62. doi: 10.1007/s11748-005-0086-7. PMID: 16164262
Total orthotopic heart transplantation for primary cardiac rhabdomyosarcoma: factors influencing long-term survival.
Grandmougin D, Fayad G, Decoene C, Pol A, Warembourg H
Ann Thorac Surg 2001 May;71(5):1438-41. doi: 10.1016/s0003-4975(01)02480-8. PMID: 11383779
Primary cardiac rhabdomyosarcoma: definition of a rare entity.
Hui KS, Green LK, Schmidt WA
Am J Cardiovasc Pathol 1988;2(1):19-29. PMID: 3207486

Diagnosis

Primary Biatrial Cardiac Rhabdomyosarcoma.
Uchida T, Kuroda Y, Sadahiro M
Braz J Cardiovasc Surg 2020 Jun 1;35(3):399-401. doi: 10.21470/1678-9741-2018-0414. PMID: 32549111Free PMC Article
Primary cardiac rhabdomyosarcoma successfully treated with eribulin: a case report.
Ota S, Shimonaga T, Yuki S, Kuraoka K, Ogawa T, Hirata T
Anticancer Drugs 2020 Mar;31(3):304-309. doi: 10.1097/CAD.0000000000000860. PMID: 31725047
A case of primary cardiac rhabdomyosarcoma with surgical removal and mitral valve repair.
Yilmaz M, Kehlibar T, Arslan IY, Yilmaz HY, Tarhan IA, Ozler A
Heart Surg Forum 2013 Jun;16(3):E164-6. doi: 10.1532/HSF98.20131001. PMID: 23803244
A triad: cardiac rhabdomyosarcoma, stroke and tamponade.
Ashraf T, Day TG, Marek J, Hughes M, Giardini A
Pediatr Cardiol 2013 Mar;34(3):771-3. Epub 2012 Oct 7 doi: 10.1007/s00246-012-0549-4. PMID: 23052680
Cardiac rhabdomyosarcoma: diagnosis by MR imaging.
Villacampa VM, Villarreal M, Ros LH, Alvarez R, Cózar M, Fuertes MI
Eur Radiol 1999;9(4):634-7. doi: 10.1007/s003300050723. PMID: 10354875

Therapy

Primary cardiac rhabdomyosarcoma successfully treated with eribulin: a case report.
Ota S, Shimonaga T, Yuki S, Kuraoka K, Ogawa T, Hirata T
Anticancer Drugs 2020 Mar;31(3):304-309. doi: 10.1097/CAD.0000000000000860. PMID: 31725047
Cardiac rhabdomyosarcoma in a renal transplant patient.
Rheeder P, Simson IW, Mentis H, Karussett VO
Transplantation 1995 Jul 27;60(2):204-5. PMID: 7624965
Magnetic resonance imaging of cardiac rhabdomyosarcoma. Quantifying the response to chemotherapy.
Szucs RA, Rehr RB, Yanovich S, Tatum JL
Cancer 1991 Apr 15;67(8):2066-70. doi: 10.1002/1097-0142(19910415)67:8<2066::aid-cncr2820670810>3.0.co;2-7. PMID: 2004325
Primary cardiac rhabdomyosarcoma exhibiting transient and pronounced regression with chemotherapy.
Satoh M, Horimoto M, Sakurai K, Funayama N, Igarashi K, Yamashiro K
Am Heart J 1990 Dec;120(6 Pt 1):1458-60. doi: 10.1016/0002-8703(90)90267-2. PMID: 2248196

Prognosis

Primary Biatrial Cardiac Rhabdomyosarcoma.
Uchida T, Kuroda Y, Sadahiro M
Braz J Cardiovasc Surg 2020 Jun 1;35(3):399-401. doi: 10.21470/1678-9741-2018-0414. PMID: 32549111Free PMC Article
A Mass Filling the Right Atrium: Primary Cardiac Rhabdomyosarcoma.
Kimura A, Tsuji M, Isogai T, Nagata K, Kato K, Hisagi M, Nonaka T, Ninomiya M, Kiriu T, Tanaka H, Tejima T
Intern Med 2018 Dec 15;57(24):3575-3580. Epub 2018 Aug 10 doi: 10.2169/internalmedicine.0657-17. PMID: 30101906Free PMC Article
A triad: cardiac rhabdomyosarcoma, stroke and tamponade.
Ashraf T, Day TG, Marek J, Hughes M, Giardini A
Pediatr Cardiol 2013 Mar;34(3):771-3. Epub 2012 Oct 7 doi: 10.1007/s00246-012-0549-4. PMID: 23052680
Primary cardiac rhabdomyosarcoma of the right atrium: case report.
Sokullu O, Sanioglu S, Deniz H, Ayoglu U, Ozgen A, Bilgen F
Heart Surg Forum 2008;11(2):E117-9. doi: 10.1532/HSF98.20071176. PMID: 18430653
Hypereosinophilia associated with cardiac rhabdomyosarcoma.
Lo Re V 3rd, Fox KR, Ferrari VA, Scott CH, Kossev PM, Kostman JR
Am J Hematol 2003 Sep;74(1):64-7. doi: 10.1002/ajh.10373. PMID: 12949893

Clinical prediction guides

Total orthotopic heart transplantation for primary cardiac rhabdomyosarcoma: factors influencing long-term survival.
Grandmougin D, Fayad G, Decoene C, Pol A, Warembourg H
Ann Thorac Surg 2001 May;71(5):1438-41. doi: 10.1016/s0003-4975(01)02480-8. PMID: 11383779
Cardiac rhabdomyosarcoma: a clinicopathologic and electron microscopy study.
Attanasio A, Romitelli S, Mauriello A, Palmieri G, Stefani A, Pierangeli L
G Ital Cardiol 1998 Apr;28(4):383-6. PMID: 9616853

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