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Cardiac rhabdomyomas

DOI: https://doi.org/10.29296/25877305-2021-09-09
Issue: 
9
Year: 
2021

Professor V. Delyagin(1, 2), MD; E. Balina(3), Candidate of Medical Scences (1)Dmitry Rogachev
National Medical Research Center of Pediatric Hematology, Oncology, and Immunology, Ministry of Health of
Russia, Moscow (2)Scientific and educational biomedical cluster «Translational Medicine» Peoples’ Friendship
University of Russia, Moscow (3)Republican Children’s Clinical Hospital, Syktyvkar

Despite the rarity of cardiac rhabdomyomas (CR) and the absence of metastasis, the outcomes of the neoplasm can be fatal. Objective. To describe the clinical and instrumental picture of CR and to formulate recommendations for the diagnosis and management of patients. Subjects and methods. Seventeen CR patients (8 boys and 9 girls) whose median age was 2 months (0.1-360 months) were examined. The diagnosis was based on the results of a clinical and instrumental observation and on those of surgical treatment with the histological verification of the type of a distant neoplasm (n=2), postmortem (n=1) and molecular genetic studies (n=1). Results. The disease manifested in early childhood; it was detected during a family examination in a mother in one case. The clinical picture showed neurocutaneous syndrome, seizures (n=5), marfanoid habitus, renal angiolipomas and cysts (n=3), hamartomas of the optic disc (n=3), and paracortical foci of demyelination (n=2). Tuberous sclerosis (TS) was diagnosed in 11 patients; a purposeful examination for CR revealed a heart tumor in 7 of the children with TS. The common cardiac symptoms were circulatory insufficiency, cardiac arrhythmia, and conduction disorders; the progressive growth of the tumor was accompanied by impaired coronary blood flow. Echocardiography (EchoCG) is the leading diagnostic method. Single small rhabdomyoma nodules are rare, mainly multiple and widespread neoplasms, were recorded. Conclusion. CR is often accompanied by TS; there may be family cases. Multiple and widespread tumors, rhythm and myocardial contraction disturbances are unfavorable prognostic factors. EchoCG is a determining diagnostic and follow-up technique.

Keywords: 
oncology
heart
tumors
rhabdomyoma
clinical presentations
genetics
diagnosis
management



References: 
  1. Isaacs H. Fetal and neonatal cardiac tumors. Pediatr Cardiol. 2004; 25 (3): 252–73. DOI: 10.1007/s00246-003-0590-4
  2. Ren D., Fuller N., Gilbert S., Zhang Y. Cardiac tumors: clinical perspective and therapeutic considerations. Curr Drug Targets. 2017; 18 (15): 1805–9. DOI: 10.2174/1389450117666160703162111
  3. Uzun O., Wislon D., Vujanic G. Cardiac tumors in children. Orphanet J Rare Dis. 2007; 2: 11. DOI: 10.1186/1750-1172-2-11
  4. Grebenc M., Rosado de Christenson M., Burke A. et al. Primary cardiac and pericardial neoplasms: radiologic-pathologic correlation. Radiographics. 2000; 20 (4): 1073–103. DOI: 10.1148/radiographics.20.4.g00jl081073
  5. Mueller D. Benign Cardiac Tumors. Updated: Aug 28, 2020. URL: https://emedicine.medscape.com/article/161239
  6. Harding C., Pagon R. Incidence of tuberous sclerosis in patients with cardiac rhabdomyoma. Am J Med Genet. 1990; 37 (4): 443–6. DOI: 10.1002/ajmg.1320370402
  7. Varma R., Williams Sh. Neurology. In: Zitalli B., McIntire S., Nowalk A. (Eds.) Zitalli and Davis’ Atlas of Pediatric Physical Diagnosis. 7th Ed. 2018; p. 567.
  8. Burke A., Virmani R. Pediatric heart tumors. Cardiovasc Pathol. 2008; 17 (4): 193–8. DOI: 10.1016/j.carpath.2007.08.008
  9. Bosi G., Lintermans J., Pellegrino P. et al. The natural history of cardiac rhabdomyoma with and without tuberous sclerosis. Acta Paediatr. 1995; 85 (8): 928–31. DOI: 10.1111/j.1651-2227.1996.tb14188.x
  10. Flood T. Cardiac Rhabdomyoma Pathology. Updated: Dec. 09, 2015. URL: https://emedicine.medscape.com/article/1612571-overview#showall
  11. Muñiz J.-C. Cardiac Tumors. In: Wernowsky G., Anderson R., Kumar R. Mussato K., Redington A., Tweddel J., Tretter J. (Eds.) Anderson’s Pediatric Cardiology. 4th Ed. 2020; p. 963–67.e3.
  12. Aw F., Goyer I., Raboisson M.-J. et al. Accelerated cardisc Rhabdomyoma regression with Everolimus in Infants with Tuberous Sclerosis Complex. Pediatr Cardiol. 2017; 38 (2): 394–400. DOI: 10.1007/s00246-016-1528-y
  13. Yin L., He D., Shen H., et al. Surgical treatment of cardiac tumors: a 5-year experience from a single cardiac center. J Thorac Dis. 2016; 8 (5): 911–9. DOI: 10.21037/jtd.2016.03.87