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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
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
Cardiac consequences of malignancy and their treatment.
Lancaster LD, Ewy GA
Adv Intern Med 1984;30:275-93. PMID: 6397037

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 cardiac rhabdomyosarcoma in a mitral valve involved with rheumatic disease.
Izzat MB, Kara Tahhan N, Izzat AW, Chatty EM
Asian Cardiovasc Thorac Ann 2024 Mar;32(2-3):136-139. Epub 2024 Jan 8 doi: 10.1177/02184923231225991. PMID: 38190842
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
Cardiac rhabdomyosarcoma.
Katz JN, Cooper DL, Farber L, Panosian KJ
Conn Med 1988 Feb;52(2):72-5. PMID: 3345664
Cardiac rhabdomyosarcoma: case report with review of clinical and pathologic features.
Becker RC, Hobbs RE, Ratliff NB
Cleve Clin Q 1984 Spring;51(1):83-8. doi: 10.3949/ccjm.51.1.83. PMID: 6713681
Cardiac rhabdomyosarcoma presenting as hypereosinophilic syndrome.
Sullivan MJ, Wanger GP, Schonfeld SA, Bashore TM
Am J Cardiol 1983 Mar 1;51(5):909-10. doi: 10.1016/s0002-9149(83)80158-1. PMID: 6829455

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
Multi-modality imaging in the assessment of a metastatic cardiac rhabdomyosarcoma presenting with recurrent ventricular tachycardia.
Platts DG, Morsy M, Burstow D
Eur Heart J Cardiovasc Imaging 2014 Mar;15(3):306. Epub 2013 Oct 10 doi: 10.1093/ehjci/jet183. PMID: 24114574
Surgical treatment of primary cardiac rhabdomyosarcoma.
Lima Rde C, Mendes A, Bezerra E, Oliveira W
Rev Bras Cir Cardiovasc 2009 Apr-Jun;24(2):242-4. doi: 10.1590/s0102-76382009000200023. PMID: 19768306
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
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

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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