69. Cardiac Tumors- Indications and Guidelines

Vuong-Lam Pham MD, Alexander Schutz MD, Marc Moon MD
Baylor College of Medicine
August 2024

Abbreviations & Definitions

AV – atrioventricular
CPB – cardiopulmonary bypass
CVA – cerebral vascular accident
PFE – papillary fibroelastomas

Indications & Guidelines for Management of Benign Cardiac Neoplasms

Myxomas

Most primary tumors are benign (75%), and myxomas are the most common benign cardiac tumors, usually arising in the left atrium. Surgical resection is indicated for all myxomas due to the risk of embolization or obstruction. Anticoagulation is indicated during the initial discovery and evaluation period to decrease the risk of embolization. If a patient presents with CVA without hemorrhage due to embolization, the tumor should be resected approximately seven days afterward to prevent further embolization and allow stabilization of the brain before CPB.1 The tumor should be resected with 0.5 to 1 cm margins if possible while avoiding injury to nearby structures.2

Papillary Fibroelastomas (PFE)

PFEs typically arise on cardiac valves or adjacent endocardium, generally on the left side of the heart. Surgical excision is recommended for symptomatic PFEs, which include embolism presenting as CVA or coronary ostial obstruction manifesting as myocardial ischemia, infarction, or sudden death.3,4 For patients who present with acute stroke and intracerebral hemorrhage, it is advised to wait 4 weeks prior to resection. The indication for resection of asymptomatic tumors is less clear. If a tumor is mobile, >1 cm, or if the patient is undergoing other cardiac procedures, the tumor should be resected. If the tumor is <1 cm and non-mobile, it can be followed closely with echocardiography.2 Simple valve-sparing shave excision is usually adequate. If needed, repair should be considered first-line therapy over replacement.2

Lipomas

Symptomatic tumors should be resected. Recurrence risk is low; however, the goal should be to have negative margins. Smaller asymptomatic tumors or tumors encountered during concomitant cardiac operations may be resected if it can be reasonably done without additional operative risk. When cardiac lipomas exist in a non-encapsulated form in the interatrial septum, this is referred to as lipomatous hypertrophy of the interatrial septum. This is more common than cardiac lipomas and is common in elderly, obese, or female patients. Due to the difficulty of distinguishing it from other neoplasms, MRI is often indicated. The natural history of lipomatous hypertrophy is unknown, and indications for resection are unclear but is indicated if there are arrhythmias or conduction abnormalities.2

Rhabdomyomas

Rhabdomyomas are most frequently seen in children, occurring with equal frequency in both ventricles. Partial or complete spontaneous regression has been observed with small tumors in younger children. Early resection is indicated in symptomatic patients with signs of obstruction or risk of embolization. The tumor can often be enucleated.2 Rhabdomyomas may occur sporadically, but most patients have or will develop tuberous sclerosis.2 Early resection is recommended before the age of 1 year for patients without tuberous sclerosis.5 However, the prognosis is poor for patients with associated tuberous sclerosis.2

Fibromas

After myxomas, fibromas are the second most common benign cardiac tumors and are often found in children. Surgical excision is successful in some patients if it does not involve vital structures, is localized, and can be enucleated. Surgery appears to be optimal for symptomatic resectable tumors while resection for asymptomatic tumors is less clear.6 Partial resection is palliative, but patients can survive for many years.7 Extensive fibromas have been treated with transplantation.8

Pheochromocytomas

Intrathoracic pheochromocytomas are rare (<2% of all pheochromocytomas), and most are in the dome of the left atrium. Other locations can include the interatrial septum or anterior surface of the heart. Diagnosis is confirmed with elevated urinary catecholamines and can often be localized with a 131I-metaiodobenzylguanidine (MIBG) scan. Similar to the management of pheochromocytomas at other locations, management consists of pre-operative control of hyperadrenergic syndrome with α-blockade, intravenous hydration, and, if necessary, β-blockade.2 Complete resection is the goal and explantation of the heart to remove the atrial or ventricular wall or major coronary artery, followed by re-implantation have been described.9

  • Table 1. Benign cardiac neoplasms and recommendations for resection
Tumor Resection Recommendations
Myxomas Complete resection for all myxomas is the goal with 0.5-1 cm margins
Papillary Fibroelastomas Resection if symptomatic If asymptomatic, resection if >1 cm, mobile, or undergoing other cardiac procedures Usually valve-sparing shave excision is adequate. No defined margins, resect to healthy tissue
Lipomas Large tumors producing symptoms Small asymptomatic tumors during other cardiac procedures No defined margins, resect to healthy tissue
Rhabdomyomas Resection if symptomatic Resection in patients without tuberous sclerosis <1 year of age No defined margins, resect to healthy tissue
Fibromas Resection if localized and does not involve vital structures No defined margins Partial removal for extensive disease for palliation
Pheochromocytomas Complete resection is the goal after medical treatment No defined margins

Indication & Guidelines for Management of Malignant Primary Cardiac Neoplasms

Primary cardiac malignancies are rare and are most commonly sarcomas. All patients should undergo staging workup for extracardiac spread. Patients without metastasis are candidates for resection. Histology does not greatly affect treatment nor prognosis; thus, classification is based on anatomic location: right heart, left heart, and pulmonary artery sarcomas.10

Right Heart Sarcomas

Right heart sarcomas have a very poor prognosis, and surgical resection is the only treatment shown to increase survival. However, complete surgical resection is complicated by its bulky and infiltrative nature and high risk of metastasis. Patients may require neoadjuvant therapy once a definitive tissue diagnosis is obtained to shrink the tumor. The tricuspid valve, right coronary artery, and up to 30% of the right ventricular muscle mass may be resected and replaced or reconstructed to achieve complete resection. R0 resection followed by adjuvant chemotherapy has been shown to extend survival.1

Pulmonary Artery Sarcomas

Pulmonary artery sarcomas primarily originate at the level of the pulmonary valve which could require pulmonary root resection and homograft replacement. A large pulmonary valve homograft at least one size larger than the aortic valve is used. For sarcomas extending beyond the first bifurcation, tumor endarterectomy is reasonable. Tumors extending beyond this would require a pneumonectomy.2

Left Heart Sarcomas

Left heart sarcomas tend to be more solid, less infiltrative, and metastasize later. Obstruction quickly results in heart failure; thus, neoadjuvant therapy can rarely be utilized. Left heart sarcomas are often misdiagnosed as left atrial myxomas and are frequently identified after undergoing resection for what was initially thought to be a benign myxoma. Resection should be considered if the tumor is anatomically reachable without evidence of metastatic disease. However, due to the location, it is often difficult to obtain optimal exposure which would lead to inadequate resection. Cardiac explantation, ex vivo resection, and reconstruction with subsequent reimplantation have been described.11-12

Indication & Guidelines for Management of Malignant Secondary Cardiac Neoplasms

Secondary tumors are the most common type of cardiac neoplasm. Almost every type of malignant tumor has been observed to metastasize to the heart or pericardium. In decreasing frequency, sites of metastasis are the pericardium, epicardium, myocardium, and endocardium. Symptoms are infrequent but when they do occur, it is usually due to pericardial effusion. Palliative pericardial drainage offers symptomatic relief.2

Neuroendocrine tumor (NET)

NET rarely metastasize to the myocardium with midgut NETs being most associated with cardiac metastasis.13 Resection should be considered if the primary site is controlled. Valve replacement is indicated in carcinoid valve disease.14

Right Atrial Extension of Subdiaphragmatic Tumors

Renal cell carcinomas are the most common. Tumors confined to Gerota’s fascia with extension into the vena cava or right atrium without regional lymph node or distant metastasis have the best prognosis. Only distant metastasis is an absolute contraindication for surgery. Different approaches have been described for level III (tumor thrombi extending beyond the hepatic veins but below the diaphragm) and level IV (tumor thrombi extending above the diaphragm) tumor extensions. This can be accomplished with or without sternotomy, CPB, and/or hypothermic circulatory arrest and is institution-dependent.1,2

Ongoing Trials

“The Safety and Efficacy Evaluation of Echocardiography-guided Percutaneous Radiofrequency Ablation for Cardiac Tumors: a Single-arm, Prospective, Single-center Clinical Study”, ClinicalTrials.gov ID: NCT06830512

The above study is aimed at evaluating echocardiography-guided percutaneous radiofrequency ablation (Liwen procedure) that is frequently utilized in obstructive hypertrophic cardiomyopathy for cardiac tumors in patients with clinical symptoms who cannot tolerate surgery or if the tumor cannot be completely excised by surgery.

“Characteristics of Cardiac Tumor and the Risk Stratification of Subsequent Adverse Events (UNIQUE)”, ClinicalTrials.gov ID: NCT06100328

The above study aims to construct a retrospective and prospective long-term follow-up cohort and management of cardiac tumors through a multi-center collaborative network. This study is expected to assess disease characteristics, progression patterns, clinical features, natural course, and long-term prognosis of cardiac tumors.

Expert Commentary

Cardiac tumors, while rare, will most likely be encountered in some form by most cardiothoracic surgeons in their careers. There is limited data on the management of malignant tumors, given the limited number of cases that prevent large cohort studies. Given the overall dismal prognosis of many of these tumors, a multimodal approach is often warranted and should be considered by a multidisciplinary team. Appropriate imaging and identification of the tumor type is key to successful intervention. In terms of technical challenges, most of the more common tumors, like PFEs or myxomas, can be readily resected with sound anatomic knowledge using the surgical approach with which the operative surgeon is most comfortable. The risk of embolic or obstructive events is most notable to keep in mind at the time of resection, and a no-touch technique of the heart prior to aortic cross-clamping may help to mitigate these risks to prevent dislodgement of any acquired thrombus burden.

Sources

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  3. Shing M, Rubenson DS. (2001). Embolic stroke and cardiac papillary fibroelastoma. Clinical cardiology, 24(4), 346–347.
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  6. Burke AP, Rosado-de-Christenson M, Templeton PA, Virmani R. (1994). Cardiac fibroma: clinicopathologic correlates and surgical treatment. The Journal of thoracic and cardiovascular surgery, 108(5), 862–870.
  7. Yamaguchi M, Hosokawa Y, Ohashi H, Imai M, Oshima Y, Minamiji K. Cardiac fibroma. Long-term fate after excision. J Thorac Cardiovasc Surg. 1992;103(1):140-145.
  8. Valente M, Cocco P, Thiene G, Casula R, Poletti A, Milanesi O, Fasoli G, Livi U. (1993). Cardiac fibroma and heart transplantation. The Journal of thoracic and cardiovascular surgery, 106(6), 1208–1212.
  9. Cooley DA, Reardon MJ, Frazier OH, Angelini P. Human cardiac explantation and autotransplantation: application in a patient with a large cardiac pheochromocytoma. Tex Heart Inst J. 1985;12(2):171-176.
  10. Blackmon SH, Patel A, Reardon MJ. Management of primary cardiac sarcomas. Expert Rev Cardiovasc Ther. 2008;6(9):1217-1222.
  11. Reardon MJ, DeFelice CA, Sheinbaum R, et al. Cardiac autotransplant for surgical treatment of a malignant neoplasm. Ann Thorac Surg. 1999;67(6):1793-5.
  12. Reardon MJ, Walkes JC, Defelice CA, et al. Cardiac autotransplantation for surgical resection of a primary malignant left ventricular tumor. Tex Heart Inst J. 2006;33(4):495-7.
  13. Jann H, Wertenbruch T, Pape U, et al. A matter of the heart: myocardial metastases in neuroendocrine tumors. Horm Metab Res. 2010;42(13):967-76.
  14. Bhattacharyya S, Davar J, Dreyfus G, et al. Carcinoid heart disease. Circulation. 2007;116(24):2860-5.
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