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Pediatric Cardiac Tumors Treatment & Management

  • Author: Edwin Rodriguez-Cruz, MD; Chief Editor: P Syamasundar Rao, MD  more...
 
Updated: Jan 04, 2016
 

Medical Care

Most childhood cardiac tumors are benign, with no treatment necessary unless the tumor severely obstructs blood flow or causes intractable arrhythmias. Most only require close follow-up care.[10]

New data have addressed the use of serum creatine kinase-myocardial band (CK-MB) fraction levels in evaluating ventricular function. Greater ventricular dysfunction correlated with higher CK-MB levels.

Nonetheless, examine the patient extensively and expeditiously because finding a tumor is a stressful situation for both patient and family. By reaching a prompt and precise diagnosis, the physician can proceed with the best treatment approach.

The mode of treatment varies and cannot be easily simplified because the kind of tumor (benign vs malignant, infiltrative vs localized) dictates therapy. Location and extent of the tumor, as well as symptoms, are clinical variables that direct treatment. Observation is sufficient when the mass is small and does not interfere with vascular hemodynamics. However, the clinician must be aggressive when the tumor is causing hemodynamic problems. In occasions, electrical problems may also arise due to the location requiring resection and/or implantation of a pacemaker if atrioventricular block occurs.

Consultations

Consult a cardiologist, thoracic and cardiovascular surgeon, pathologist, radiologist, and hematologist/oncologist.

Transfer

Transfer to a facility with the necessary specialties and consultants.

Diet and activity

No dietary restrictions are needed. Occasionally, certain restrictions such as low-sodium diet for congestive heart failure (CHF) are required.

Advise no restrictions unless the patient's clinical condition merits otherwise.

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Surgical Care

Many advocate excision of the mass as soon as it is found; however, most childhood tumors are benign and do not require resection. Nevertheless, if a tumor is causing severe obstruction or intractable arrhythmias and has been rendered resectable, perform surgery as soon as possible.[11]

Excision and biopsy

If excision is required, perform it as extensively and completely as possible. Certain tumors recur even after complete excision. An extensive resection carries its own risks since, occasionally, removal of papillary muscles, valves, chordae tendineae, and conduction tissue are necessary.

Cardiac transplantation

Heart transplantation is an option for unresectable benign masses causing hemodynamic compromise. Transplantations have been performed with variable results for primary malignant cardiac tumors in children and adults without evidence of metastases.

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Contributor Information and Disclosures
Author

Edwin Rodriguez-Cruz, MD Director, Section of Cardiology, Department of Pediatrics, San Jorge Children’s Hospital, Puerto Rico; Private Practice in Interventional Pediatric Cardiology and Internal Medicine, Centro Pedíatrico y Cardiovascular

Edwin Rodriguez-Cruz, MD is a member of the following medical societies: American College of Cardiology, American Heart Association, American Society of Echocardiography, Society for Cardiovascular Angiography and Interventions, Society of Pediatric Echocardiography, American College of Physicians-American Society of Internal Medicine, American Medical Association, Puerto Rico Medical Association

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: St Jude's Medical Co.<br/>Received grant/research funds from NOVARTIS for investigator; Received consulting fee from St. Jude Medical Corp. for speaking and teaching.

Coauthor(s)

Robert D Ross, MD Director of Pediatric Cardiology Fellowship Program, Department of Pediatrics, Division of Pediatric Cardiology, Children's Hospital of Michigan; Professor of Pediatrics, Wayne State University School of Medicine

Robert D Ross, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Society of Pediatric Echocardiography

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Ameeta Martin, MD Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine

Ameeta Martin, MD is a member of the following medical societies: American College of Cardiology

Disclosure: Nothing to disclose.

Chief Editor

P Syamasundar Rao, MD Professor of Pediatrics and Medicine, Division of Cardiology, Emeritus Chief of Pediatric Cardiology, University of Texas Medical School at Houston and Children's Memorial Hermann Hospital

P Syamasundar Rao, MD is a member of the following medical societies: American Academy of Pediatrics, American Pediatric Society, American College of Cardiology, American Heart Association, Society for Cardiovascular Angiography and Interventions, Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

Jeffrey Allen Towbin, MD, MSc FAAP, FACC, FAHA, Professor, Departments of Pediatrics (Cardiology), Cardiovascular Sciences, and Molecular and Human Genetics, Baylor College of Medicine; Chief of Pediatric Cardiology, Foundation Chair in Pediatric Cardiac Research, Texas Children's Hospital

Jeffrey Allen Towbin, MD, MSc is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American College of Cardiology, American College of Sports Medicine, American Heart Association, American Medical Association, American Society of Human Genetics, New York Academy of Sciences, Society for Pediatric Research, Texas Medical Association, Texas Pediatric Society, Cardiac Electrophysiology Society

Disclosure: Nothing to disclose.

Acknowledgements

Rosa M Cintrón-Maldonado, MD Clinical Instructor of Pediatrics, Department of Pediatrics, San Juan Bautista Medical Center

Rosa M Cintrón-Maldonado, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

References
  1. Tao TY, Yahyavi-Firouz-Abadi N, Singh GK, Bhalla S. Pediatric cardiac tumors: clinical and imaging features. Radiographics. 2014 Jul-Aug. 34 (4):1031-46. [Medline].

  2. Kutluk T, Demir HA, Buyukpamukcu M, et al. Cardiac rhabdomyomas in childhood: six cases from a single institution. Turk J Pediatr. 2013 Jan-Feb. 55(1):69-73. [Medline].

  3. Nield LE, Mendelson M, Ahmad N, Manlhiot C, Jaeggi ET, McCrindle BW. Clinical Review of Obstructive Primary Cardiac Tumors in Childhood. Congenit Heart Dis. 2013 Aug 21. [Medline].

  4. Lam KY, Dickens P, Chan AC. Tumors of the heart. A 20-year experience with a review of 12,485 consecutive autopsies. Arch Pathol Lab Med. 1993 Oct. 117(10):1027-31. [Medline].

  5. Miyake CY, Del Nido PJ, Alexander ME, Cecchin F, Berul CI, Triedman JK, et al. Cardiac tumors and associated arrhythmias in pediatric patients, with observations on surgical therapy for ventricular tachycardia. J Am Coll Cardiol. 2011 Oct 25. 58(18):1903-9. [Medline].

  6. Salpea P, Stratakis CA. Carney complex and McCune Albright syndrome: An overview of clinical manifestations and human molecular genetics. Mol Cell Endocrinol. 2013 Sep 5. [Medline].

  7. Kono T, Koide N, Hama Y, et al. Expression of vascular endothelial growth factor and angiogenesis in cardiac myxoma: a study of fifteen patients. J Thorac Cardiovasc Surg. 2000 Jan. 119(1):101-7. [Medline].

  8. Beroukhim RS, Prakash A, Buechel ER, Cava JR, Dorfman AL, Festa P, et al. Characterization of cardiac tumors in children by cardiovascular magnetic resonance imaging: a multicenter experience. J Am Coll Cardiol. 2011 Aug 30. 58(10):1044-54. [Medline].

  9. Grant FD, Treves ST. Nuclear medicine and molecular imaging of the pediatric chest: current practical imaging assessment. Radiol Clin North Am. 2011 Sep. 49(5):1025-51. [Medline].

  10. Amelia A, Mohd Nizam MB. Perinatal management of cardiac tumors: a case series. Med J Malaysia. 2013 Aug. 68(4):374-5. [Medline].

  11. Linnemeier L, Benneyworth BD, Turrentine M, Rodefeld M, Brown J. Pediatric cardiac tumors: a 45-year, single-institution review. World J Pediatr Congenit Heart Surg. 2015 Apr. 6 (2):215-9. [Medline].

  12. Butany J, Nair V, Naseemuddin A, et al. Cardiac tumours: diagnosis and management. Lancet Oncol. 2005 Apr. 6(4):219-28. [Medline].

  13. Dhillon G, Rodriguez-Cruz E, Kathawala M, Alqassem N. Primary cardiac myofibroblastic sarcoma, case report and review of diagnosis and treatment of cardiac tumors. Bol Asoc Med P R. 1998 Jul-Dec. 90(7-12):130-3. [Medline].

  14. Farooki ZQ, Ross RD, Paridon SM, et al. Spontaneous regression of cardiac rhabdomyoma. Am J Cardiol. 1991 Apr 15. 67(9):897-9. [Medline].

  15. Gaumann A, Strubel G, Bode-Lesniewska B, Schmidtmann I, Kriegsmann J, Kirkpatrick CJ. The role of tumor vascularisation in benign and malignant cardiovascular neoplasms: a comparison of cardiac myxoma and sarcomas of the pulmonary artery. Oncol Rep. 2008 Aug. 20(2):309-18. [Medline].

  16. Le Guyader A, Laskar M. Removal of left ventricular tumors by a transaortic transvalvular approach with the help of thoracoscopic instruments. J Thorac Cardiovasc Surg. 2008 Aug. 136(2):537-8. [Medline].

  17. Lopez M, Pinto A, Moreno V, Diaz M, Gonzalez Baron A. Primary cardiac osteosarcoma. Clin Transl Oncol. 2008 Aug. 10(8):515-6. [Medline].

  18. Ludomirsky A. Cardiac tumors. Bricker JT, Fisher DJ, eds. The Science and Practice of Pediatric Cardiology. 9th ed. Williams & Wilkins; 1998. Vol 2: 1885-93.

  19. Martinez Quesada M, Trujillo Berraquero F, Almendro Delia M, et al. [Cardiac hamartoma. Case report and literature review]. Rev Esp Cardiol. 2005 Apr. 58(4):450-2. [Medline].

  20. Marx GR. Cardiac Tumors. Emmanouilides GC, Gutgesell HP, Riemenschneider TA, Allen HD, eds. Moss and Adams Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult. 5th ed. Baltimore, MD: Williams & Wilkins; 1995. Vol 2: 1773-86.

  21. McAllister HA, Fenoglio JJ Jr. Tumors of the cardiovascular system: atlas of tumor pathology, second series. Washington, DC: Armed Forces Institute of Pathology. 1978.

  22. Padalino MA, Basso C, Milanesi O, et al. Surgically treated primary cardiac tumors in early infancy and childhood. J Thorac Cardiovasc Surg. 2005 Jun. 129(6):1358-63. [Medline].

  23. Piazza N, Chughtai T, Toledano K, et al. Primary cardiac tumours: eighteen years of surgical experience on 21 patients. Can J Cardiol. 2004 Dec. 20(14):1443-8. [Medline].

  24. Rodriguez-Cruz E, Cintron-Maldonado RM, Bercu BA. Primary cardiac osteogenic sarcoma treated with heart transplantation. Bol Asoc Med PR. 1999. 91 (7-12):98-99. [Medline].

  25. Rodriguez-Cruz E, Cintron-Maldonado RM, Forbes TJ. Treatment of primary cardiac malignancies with orthotopic heart transplantation. Bol Asoc Med P R. 2000 Apr-Aug. 92(4-8):65-71. [Medline].

  26. Takach TJ, Reul GJ, Ott DA, Cooley DA. Primary cardiac tumors in infants and children: immediate and long-term operative results. Ann Thorac Surg. 1996 Aug. 62(2):559-64. [Medline].

  27. Tatli S, Lipton MJ. CT for intracardiac thrombi and tumors. Int J Cardiovasc Imaging. 2005 Feb. 21(1):115-31. [Medline].

  28. Tazelaar HD, Locke TJ, McGregor CG. Pathology of surgically excised primary cardiac tumors. Mayo Clin Proc. 1992 Oct. 67(10):957-65. [Medline].

  29. Wu XM, Hu CZ, Li N, et al. [Relationship of serum level of creatine kinase of MB type to cardiac function of patients with advanced tumors and its prognostic value]. Ai Zheng. 2005 Apr. 24(4):506-8.abs. [Medline].

 
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Echocardiographic parasternal long-axis view showing a large mass within the left ventricular cavity invading or connected to the anterior mitral valve leaflet. AO=Aorta, RV=Right ventricle, LA=Left atrium, Arrow=Left ventricular mass.
Echocardiographic subcostal view of a patient with a large mass within the left ventricular cavity invading or connected to the anterior mitral valve leaflet. Mass within the left ventricle fills most of the ventricular cavity. LA=Left atrium, RA=Right atrium, RV=Right ventricle, Arrow=Left ventricular mass.
Echocardiographic parasternal long-axis view demonstrating a rounded mass in the area of the right ventricular outflow tract. Mass was not causing any outflow obstruction. RV=Right ventricle, LV= Left ventricle, AO=Aorta, Arrow=Right ventricular mass.
Echocardiographic apical 5 chamber view of a 1 month old patient with several ventricular tumors filling the ventricular cavities in both sides. The patient was having seizures and diagnosed to have tuberous sclerosis. The tumor associated with this disease is rhabdomyoma (see text). RA= Right Atrium; Ao= Aorta; LA= Left Atrium; RV= Right Atrium; LV= Left Ventricle
Follow-up echocardiographic 4 chamber view from the same patient as shown in the image above. This echocardiogram was done 8 months after the previous view. Note that the tumors have changed in size and some of them have regressed totally, especially in the right ventricle. The arrows show the remaining areas of tumors seen in both ventricles.
 
 
 
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