Primary Cardiac Neoplasms

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Primary Cardiac Neoplasms

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The most common primary cardiac tumor is the atrial myxoma, which accounts for 40-50% of all these neoplasms. [1]  The remainder of the pathological spectrum includes benign and malignant cell types. Although the overall incidence of primary cardiac neoplasms is low (0.0001-0.5% in autopsy series), these cardiac tumors provide unique diagnostic and therapeutic challenges. [2, 3, 4, 5]

The clinical symptoms caused by cardiac tumors are generally secondary to their mass effect, local invasion, embolization, or constitutional symptoms. An intracardiac tumor mass may obstruct blood flow, compromise valve function, or induce neurological catastrophe secondary to tumor embolization. The location of the tumor determines the type of symptoms produced, which can include syncope, angina, dyspnea, edema, ascites, depression of pump function, cardiomyopathy, and pulmonary hypertension. Some tumors produce no symptoms and are found incidentally as a consequence of secondary symptoms such as stroke or evidence of peripheral embolization.

Most benign tumors can be resected completely with excellent outcomes. Consider heart transplantation in those instances in which the benign tumor is too large to resect. Resection is the treatment of choice for malignant cardiac tumors; however, long-term results are dismal, even with the addition of adjuvant therapy. [6]

Several types of tumors can arise in the heart, depending upon the tissues and structures involved. Primary neoplasms may arise from endocardium, valvular structures, primitive tissue rests, and the conduction system. Secondary or metastatic neoplasms arise from hematologic spread of the originating tissue. The physiologic derangements induced by the tumor are dependent upon the location of the mass and the tissue from which it arises.

United States

Incidence depends upon tumor cell type. Overall incidence of the disease is 0.0001-0.5% in autopsy series. Myxomas account for 40-50% of primary cardiac tumors in patients aged 30-60 years.

International

Rates of cardiac tumors in other countries parallel rates in the United States.

See the list below:

Myxomas: The mortality rate of patients after myxoma removal does not differ significantly from that of the general population.

Rhabdomyoma, fibroma, fibroelastoma, hemangioma, lipoma, teratoma, and hamartoma: These are benign tumors, and mortality rates in patients with these tumors do not differ significantly from that of the general population.

Malignant tumors: These tumors are generally sarcomatous in nature. In spite of resections (complete or incomplete), median survival duration ranges from 10-24 months.

Primary cardiac tumors appear to occur equally in all races, unless their presence is connected directly with underlying genetic disorders that demonstrate a race predilection. The frequencies of these tumors in males and females are the same.

Cardiac tumors can occur in all age groups, but the following variations are seen:

Cardiac myxoma, lipomatous septal hypertrophy, [7] paraganglioma, and sarcoma tumors occur primarily in adults.

Rhabdomyoma, fibroma, teratoma, hamartoma, and Purkinje tumors occur primarily in children. [8]

Papillary fibroelastoma, [9] hemangioma, and lipoma occur in all age groups.

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Wei K, Guo HW, Fan SY, Sun XG, Hu SS. Clinical features and surgical results of cardiac myxoma in Carney complex. J Card Surg. 2019 Jan. 34 (1):14-19. [Medline].

Mary C Mancini, MD, PhD, MMM Surgeon-in-Chief and Director of Cardiothoracic Surgery, Christus Highland

Mary C Mancini, MD, PhD, MMM is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Phi Beta Kappa, Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Benjamin Movsas, MD 

Benjamin Movsas, MD is a member of the following medical societies: American College of Radiology, American Radium Society, American Society for Radiation Oncology

Disclosure: Nothing to disclose.

Eric H Yang, MD Associate Professor of Medicine, Director of Cardiac Catherization Laboratory and Interventional Cardiology, Mayo Clinic Arizona

Eric H Yang, MD is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Nothing to disclose.

Clarence Sarkodee Adoo, MD, FACP Consulting Staff, Department of Bone Marrow Transplantation, City of Hope Samaritan BMT Program

Clarence Sarkodee Adoo, MD, FACP is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American Society of Hematology, American Society of Clinical Oncology

Disclosure: Nothing to disclose.

Primary Cardiac Neoplasms

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