Childhood Cardiac Tumors Treatment (PDQ®): Treatment - Health Professional Information [NCI]

Incidence and Types of Childhood Cardiac Tumors

Cardiac tumors are rare in children, with an autopsy frequency of 0.001% to 0.30%.[1] In one report, the percentage of cardiac surgeries performed as a result of cardiac tumors was 0.093%.[2]

The distribution of cardiac tumors in the fetal and neonatal period is different from that in older patients, with two-thirds of teratomas occurring during this period of life.[3]

The most common primary tumors of the heart are benign and include the following:[4,5,6]

• Rhabdomyoma.
• Myxoma.
• Teratoma.
• Fibroma.

Other benign tumors include histiocytoid cardiomyopathy tumors, hemangiomas, and neurofibromas (i.e., tumors of the nerves that innervate the muscles).[3,4,7,8,9]

Primary malignant pediatric heart tumors are rare and include the following:[4,10,11,12]

• Malignant teratoma.
• Lymphoma.
• Various sarcomas, including rhabdomyosarcoma, angiosarcoma, undifferentiated pleomorphic sarcoma, leiomyosarcoma, chondrosarcoma, synovial sarcoma, and infantile fibrosarcoma.

Secondary tumors of the heart include metastatic spread of rhabdomyosarcoma, other sarcomas, melanoma, leukemia, thymoma, and carcinomas of various sites.[1,4]

References:

1. Butany J, Nair V, Naseemuddin A, et al.: Cardiac tumours: diagnosis and management. Lancet Oncol 6 (4): 219-28, 2005.
2. Bielefeld KJ, Moller JH: Cardiac tumors in infants and children: study of 120 operated patients. Pediatr Cardiol 34 (1): 125-8, 2013.
3. Isaacs H: Fetal and neonatal cardiac tumors. Pediatr Cardiol 25 (3): 252-73, 2004 May-Jun.
4. Burke A, Virmani R: Pediatric heart tumors. Cardiovasc Pathol 17 (4): 193-8, 2008 Jul-Aug.
5. Becker AE: Primary heart tumors in the pediatric age group: a review of salient pathologic features relevant for clinicians. Pediatr Cardiol 21 (4): 317-23, 2000 Jul-Aug.
6. Padalino MA, Vida VL, Boccuzzo G, et al.: Surgery for primary cardiac tumors in children: early and late results in a multicenter European Congenital Heart Surgeons Association study. Circulation 126 (1): 22-30, 2012.
7. Elderkin RA, Radford DJ: Primary cardiac tumours in a paediatric population. J Paediatr Child Health 38 (2): 173-7, 2002.
8. Uzun O, Wilson DG, Vujanic GM, et al.: Cardiac tumours in children. Orphanet J Rare Dis 2: 11, 2007.
9. Bruce CJ: Cardiac tumours: diagnosis and management. Heart 97 (2): 151-60, 2011.
10. Kogon B, Shehata B, Katzenstein H, et al.: Primary congenital infantile fibrosarcoma of the heart: the first confirmed case. Ann Thorac Surg 91 (4): 1276-80, 2011.
11. Wang JG, Li NN: Primary cardiac synovial sarcoma. Ann Thorac Surg 95 (6): 2202-9, 2013.
12. Ostrowski S, Marcinkiewicz A, Kośmider A, et al.: Sarcomas of the heart as a difficult interdisciplinary problem. Arch Med Sci 10 (1): 135-48, 2014.

Risk Factors

Multiple cardiac tumors noted in the fetal or neonatal period are highly associated with a diagnosis of tuberous sclerosis.[1,2]

A retrospective review of 94 patients with cardiac tumors detected by prenatal or neonatal echocardiography showed that 68% of the patients exhibited features of tuberous sclerosis.[3] In another study, 79% of patients (15 of 19) with rhabdomyomas discovered prenatally had tuberous sclerosis, while 96% of those diagnosed postnatally had tuberous sclerosis.

Carney complex is a risk factor for developing myxomas. Carney complex is a rare syndrome characterized by lentigines, cardiac myxomas or other myxoid fibromas, and endocrine abnormalities.[4,5,6] A variant of the PRKAR1A gene is noted in more than 90% of Carney complex cases.[4,7]

References:

1. Isaacs H: Fetal and neonatal cardiac tumors. Pediatr Cardiol 25 (3): 252-73, 2004 May-Jun.
2. Kocabaş A, Ekici F, Cetin Iİ, et al.: Cardiac rhabdomyomas associated with tuberous sclerosis complex in 11 children: presentation to outcome. Pediatr Hematol Oncol 30 (2): 71-9, 2013.
3. Tworetzky W, McElhinney DB, Margossian R, et al.: Association between cardiac tumors and tuberous sclerosis in the fetus and neonate. Am J Cardiol 92 (4): 487-9, 2003.
4. Boikos SA, Stratakis CA: Carney complex: the first 20 years. Curr Opin Oncol 19 (1): 24-9, 2007.
5. Carney JA, Young WF: Primary pigmented nodular adrenocortical disease and its associated conditions. Endocrinologist 2: 6-21, 1992.
6. Stratakis CA, Kirschner LS, Carney JA: Clinical and molecular features of the Carney complex: diagnostic criteria and recommendations for patient evaluation. J Clin Endocrinol Metab 86 (9): 4041-6, 2001.
7. Boikos SA, Stratakis CA: Carney complex: pathology and molecular genetics. Neuroendocrinology 83 (3-4): 189-99, 2006.

Clinical Presentation and Diagnostic Evaluation

Patients may be asymptomatic and present with sudden death,[1][Level of evidence C1] but about two-thirds of patients have symptoms that may include the following:

• Abnormalities of heart rhythm.
• Enlargement of the heart.
• Fluid in the pericardial sac.
• Congestive heart failure.
• Syncope.
• Stroke.
• Respiratory distress.[2]

Cardiac magnetic resonance imaging using specific sequences can lead to an accurate diagnosis in most patients with cardiac tumors.[3,4] However, a histological diagnosis should remain the standard in cases where the diagnosis cannot be established by noninvasive methods or if the possibility of malignancy has been considered.

References:

1. Neri M, Di Donato S, Maglietta R, et al.: Sudden death as presenting symptom caused by cardiac primary multicentric left ventricle rhabdomyoma, in an 11-month-old baby. An immunohistochemical study. Diagn Pathol 7: 169, 2012.
2. Padalino MA, Vida VL, Boccuzzo G, et al.: Surgery for primary cardiac tumors in children: early and late results in a multicenter European Congenital Heart Surgeons Association study. Circulation 126 (1): 22-30, 2012.
3. Beroukhim RS, Ghelani S, Ashwath R, et al.: Accuracy of Cardiac Magnetic Resonance Imaging in Diagnosing Pediatric Cardiac Masses: A Multicenter Study. JACC Cardiovasc Imaging 15 (8): 1391-1405, 2022.
4. Beroukhim RS, Prakash A, Buechel ER, et al.: Characterization of cardiac tumors in children by cardiovascular magnetic resonance imaging: a multicenter experience. J Am Coll Cardiol 58 (10): 1044-54, 2011.

Treatment of Childhood Cardiac Tumors

Successful treatment may require surgery, debulking for progressive symptoms, cardiac transplant, and chemotherapy that is appropriate for the type of cancer that is present.[1,2,3]; [4][Level of evidence C1] In one series, 95% of patients were free from cardiac tumor recurrence at 10 years.[5]

Treatment options for childhood cardiac tumors, according to tumor type or resectability, are as follows:

Rhabdomyoma

Most rhabdomyomas, whether diagnosed prenatally or postnatally, will spontaneously regress.[6] However, although some lesions can regress spontaneously, certain practitioners may recommend prophylactic resection to prevent mass-related complications.[5,7,8]; [9][Level of evidence C2]

Treatment with the mammalian target of rapamycin (mTOR) inhibitors everolimus or sirolimus has been reported to be associated with a decrease in the size of rhabdomyomas in patients with tuberous sclerosis.[8,10,11,12,13]

Sarcoma

Patients with cardiac sarcomas have a poor outcome and can be treated with multimodal therapy. The use of preoperative chemotherapy and/or radiation therapy may be of value in reducing tumor volume before surgery.

Other Tumor Types

Complete surgical excision of other lesions offers the best chance for cure. Postoperative complications are seen in about one-third of patients, and postoperative mortality rates are less than 10%.[5,7,14]

Unresectable Tumor

Radiation therapy is a rare treatment option for patients with unresectable disease. Radiation therapy is used to prevent progression because it is unlikely to produce full disease resolution.[15,16,17,18]

References:

1. Michler RE, Goldstein DJ: Treatment of cardiac tumors by orthotopic cardiac transplantation. Semin Oncol 24 (5): 534-9, 1997.
2. Stiller B, Hetzer R, Meyer R, et al.: Primary cardiac tumours: when is surgery necessary? Eur J Cardiothorac Surg 20 (5): 1002-6, 2001.
3. Günther T, Schreiber C, Noebauer C, et al.: Treatment strategies for pediatric patients with primary cardiac and pericardial tumors: a 30-year review. Pediatr Cardiol 29 (6): 1071-6, 2008.
4. Wu KH, Mo XM, Liu YL: Clinical analysis and surgical results of cardiac myxoma in pediatric patients. J Surg Oncol 99 (1): 48-50, 2009.
5. Padalino MA, Vida VL, Boccuzzo G, et al.: Surgery for primary cardiac tumors in children: early and late results in a multicenter European Congenital Heart Surgeons Association study. Circulation 126 (1): 22-30, 2012.
6. Bader RS, Chitayat D, Kelly E, et al.: Fetal rhabdomyoma: prenatal diagnosis, clinical outcome, and incidence of associated tuberous sclerosis complex. J Pediatr 143 (5): 620-4, 2003.
7. Bielefeld KJ, Moller JH: Cardiac tumors in infants and children: study of 120 operated patients. Pediatr Cardiol 34 (1): 125-8, 2013.
8. Kocabaş A, Ekici F, Cetin Iİ, et al.: Cardiac rhabdomyomas associated with tuberous sclerosis complex in 11 children: presentation to outcome. Pediatr Hematol Oncol 30 (2): 71-9, 2013.
9. Kutluk T, Demir HA, Büyükpamukçu M, et al.: Cardiac rhabdomyomas in childhood: six cases from a single institution. Turk J Pediatr 55 (1): 69-73, 2013 Jan-Feb.
10. Choudhry S, Nguyen HH, Anwar S: Rapid resolution of cardiac rhabdomyomas following everolimus therapy. BMJ Case Rep 2015: , 2015.
11. Barnes BT, Procaccini D, Crino J, et al.: Maternal Sirolimus Therapy for Fetal Cardiac Rhabdomyomas. N Engl J Med 378 (19): 1844-1845, 2018.
12. Nir-David Y, Brosilow S, Khoury A: Rapid response of a cardiac rhabdomyoma causing severe right ventricular outflow obstruction to Sirolimus in an infant with negative genetics for Tuberous sclerosis. Cardiol Young 31 (2): 312-314, 2021.
13. Chen XQ, Wang YY, Zhang MN, et al.: Sirolimus Can Increase the Disappearance Rate of Cardiac Rhabdomyomas Associated with Tuberous Sclerosis: A Prospective Cohort and Self-Controlled Case Series Study. J Pediatr 233: 150-155.e4, 2021.
14. Beroukhim RS, Geva T, Del Nido P, et al.: Risk Factors for Left Ventricular Dysfunction Following Surgical Management of Cardiac Fibroma. Circ Cardiovasc Imaging 14 (2): e011748, 2021.
15. Movsas B, Teruya-Feldstein J, Smith J, et al.: Primary cardiac sarcoma: a novel treatment approach. Chest 114 (2): 648-52, 1998.
16. Simpson L, Kumar SK, Okuno SH, et al.: Malignant primary cardiac tumors: review of a single institution experience. Cancer 112 (11): 2440-6, 2008.
17. Mery GM, Reardon MJ, Haas J, et al.: A combined modality approach to recurrent cardiac sarcoma resulting in a prolonged remission: a case report. Chest 123 (5): 1766-8, 2003.
18. Zerkowski HR, Hofmann HS, Gybels I, et al.: Primary sarcoma of pulmonary artery and valve: multimodality treatment by chemotherapy and homograft replacement. J Thorac Cardiovasc Surg 112 (4): 1122-4, 1996.

Treatment Options Under Clinical Evaluation for Childhood Cardiac Tumors

Information about National Cancer Institute (NCI)–supported clinical trials can be found on the NCI website. For information about clinical trials sponsored by other organizations, see the ClinicalTrials.gov website.

Special Considerations for the Treatment of Children With Cancer

Cancer in children and adolescents is rare, although the overall incidence has been slowly increasing since 1975.[1] Referral to medical centers with multidisciplinary teams of cancer specialists experienced in treating cancers that occur in childhood and adolescence should be considered. This multidisciplinary team approach incorporates the skills of the following health care professionals and others to ensure that children receive treatment, supportive care, and rehabilitation that will achieve optimal survival and quality of life:

• Primary care physicians.
• Pediatric surgeons.
• Radiation oncologists.
• Pediatric medical oncologists/hematologists.
• Rehabilitation specialists.
• Pediatric nurse specialists.
• Social workers.
• Child-life professionals.
• Psychologists.

For information about supportive care for children and adolescents with cancer, see the summaries on Supportive and Palliative Care.

The American Academy of Pediatrics has outlined guidelines for pediatric cancer centers and their role in the treatment of pediatric patients with cancer.[2] At these pediatric cancer centers, clinical trials are available for most types of cancer that occur in children and adolescents, and the opportunity to participate is offered to most patients and their families. Clinical trials for children and adolescents diagnosed with cancer are generally designed to compare potentially better therapy with current standard therapy. Most of the progress made in identifying curative therapy for childhood cancers has been achieved through clinical trials. Information about ongoing clinical trials is available from the NCI website.

Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2020, childhood cancer mortality decreased by more than 50%.[3,4,5] Childhood and adolescent cancer survivors require close monitoring because side effects of cancer therapy may persist or develop months or years after treatment. For information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors, see Late Effects of Treatment for Childhood Cancer.

Childhood cancer is a rare disease, with about 15,000 cases diagnosed annually in the United States in individuals younger than 20 years.[6] The U.S. Rare Diseases Act of 2002 defines a rare disease as one that affects populations smaller than 200,000 people. Therefore, all pediatric cancers are considered rare.

The designation of a rare tumor is not uniform among pediatric and adult groups. In adults, rare cancers are defined as those with an annual incidence of fewer than six cases per 100,000 people. They account for up to 24% of all cancers diagnosed in the European Union and about 20% of all cancers diagnosed in the United States.[7,8] Also, the designation of a pediatric rare tumor is not uniform among international groups, as follows:

• A consensus effort between the European Union Joint Action on Rare Cancers and the European Cooperative Study Group for Rare Pediatric Cancers estimated that 11% of all cancers in patients younger than 20 years could be categorized as very rare. This consensus group defined very rare cancers as those with annual incidences of fewer than 2 cases per 1 million people. However, three additional histologies (thyroid carcinoma, melanoma, and testicular cancer) with incidences of more than 2 cases per 1 million people were also included in the very rare group because there is a lack of knowledge and expertise in the management of these tumors.[9]
• The Children's Oncology Group (COG) defines rare pediatric cancers as those listed in the International Classification of Childhood Cancer subgroup XI, which includes thyroid cancers, melanomas and nonmelanoma skin cancers, and multiple types of carcinomas (e.g., adrenocortical carcinomas, nasopharyngeal carcinomas, and most adult-type carcinomas such as breast cancers, colorectal cancers, etc.).[10] These diagnoses account for about 5% of the cancers diagnosed in children aged 0 to 14 years and about 27% of the cancers diagnosed in adolescents aged 15 to 19 years.[4]

  Most cancers in subgroup XI are either melanomas or thyroid cancers, with other cancer types accounting for only 2% of the cancers in children aged 0 to 14 years and 9.3% of the cancers in adolescents aged 15 to 19 years.

These rare cancers are extremely challenging to study because of the low number of patients with any individual diagnosis, the predominance of rare cancers in the adolescent population, and the lack of clinical trials for adolescents with rare cancers.

References:

1. Smith MA, Seibel NL, Altekruse SF, et al.: Outcomes for children and adolescents with cancer: challenges for the twenty-first century. J Clin Oncol 28 (15): 2625-34, 2010.
2. American Academy of Pediatrics: Standards for pediatric cancer centers. Pediatrics 134 (2): 410-4, 2014. Also available online. Last accessed December 15, 2023.
3. Smith MA, Altekruse SF, Adamson PC, et al.: Declining childhood and adolescent cancer mortality. Cancer 120 (16): 2497-506, 2014.
4. National Cancer Institute: NCCR*Explorer: An interactive website for NCCR cancer statistics. Bethesda, MD: National Cancer Institute. Available online. Last accessed December 15, 2023.
5. Surveillance Research Program, National Cancer Institute: SEER*Explorer: An interactive website for SEER cancer statistics. Bethesda, MD: National Cancer Institute. Available online. Last accessed March 6, 2024.
6. Ward E, DeSantis C, Robbins A, et al.: Childhood and adolescent cancer statistics, 2014. CA Cancer J Clin 64 (2): 83-103, 2014 Mar-Apr.
7. Gatta G, Capocaccia R, Botta L, et al.: Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet-a population-based study. Lancet Oncol 18 (8): 1022-1039, 2017.
8. DeSantis CE, Kramer JL, Jemal A: The burden of rare cancers in the United States. CA Cancer J Clin 67 (4): 261-272, 2017.
9. Ferrari A, Brecht IB, Gatta G, et al.: Defining and listing very rare cancers of paediatric age: consensus of the Joint Action on Rare Cancers in cooperation with the European Cooperative Study Group for Pediatric Rare Tumors. Eur J Cancer 110: 120-126, 2019.
10. Pappo AS, Krailo M, Chen Z, et al.: Infrequent tumor initiative of the Children's Oncology Group: initial lessons learned and their impact on future plans. J Clin Oncol 28 (33): 5011-6, 2010.

Latest Updates to This Summary (04 / 26 / 2024)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Editorial changes were made to this summary.

This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® Cancer Information for Health Professionals pages.

About This PDQ Summary

Purpose of This Summary

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of childhood cardiac tumors. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.

Reviewers and Updates

This summary is reviewed regularly and updated as necessary by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).

Board members review recently published articles each month to determine whether an article should:

• be discussed at a meeting,
• be cited with text, or
• replace or update an existing article that is already cited.

Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.

The lead reviewers for Childhood Cardiac Tumors Treatment are:

• Denise Adams, MD (Children's Hospital Boston)
• Karen J. Marcus, MD, FACR (Dana-Farber Cancer Institute/Boston Children's Hospital)
• William H. Meyer, MD
• Paul A. Meyers, MD (Memorial Sloan-Kettering Cancer Center)
• Thomas A. Olson, MD (Aflac Cancer and Blood Disorders Center of Children's Healthcare of Atlanta - Egleston Campus)
• Alberto S. Pappo, MD (St. Jude Children's Research Hospital)
• Arthur Kim Ritchey, MD (Children's Hospital of Pittsburgh of UPMC)
• Carlos Rodriguez-Galindo, MD (St. Jude Children's Research Hospital)
• Stephen J. Shochat, MD (St. Jude Children's Research Hospital)

Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.

Levels of Evidence

Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Pediatric Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.

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The preferred citation for this PDQ summary is:

PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Cardiac Tumors Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/cardiac/hp-child-cardiac-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 31593384]

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Last Revised: 2024-04-26