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Cardiac Cancer
Introduction
Cardiac tumors are uncommon and can be primary or metastatic. Primary cardiac tumors are rare, with an incidence ranging from 0.001% to 0.3% by autopsy. On the other hand, metastatic cardiac tumors were found in 2.3% and 18.3% of autopsies.[1][2] In addition to autopsy findings, contemporary imaging modalities, including echocardiography, cardiac magnetic resonance imaging (MRI), and computed tomography (CT), have significantly enhanced cardiac tumors' detection and anatomical delineation, many of which historically remained unrecognized until postmortem examination.[3]
The World Health Organization categorizes primary cardiac neoplasms into 4 principal groups: benign tumors, neoplasms of indeterminate biological potential, germ cell tumors, and malignant variants. Sometimes, the pericardial tumors are also included in the classification of cardiac tumors. This classification was proposed in 2015, then updated in 2021, without substantial change.[4][5]
Nearly 75% to 90% of primary cardiac tumors are benign.[6] Benign tumors include rhabdomyoma, cardiac myxoma, fibroma, lipoma, and papillary fibroelastoma, among others. Myxoma is the most common primary tumor of the heart, accounting for slightly more than half of all primary cardiac tumors.[7] Neoplasms of uncertain behavior encompass inflammatory myofibroblastic tumors and paragangliomas. Germ cell tumors, both cardiac and pericardial, comprise mature and immature teratomas and yolk sac tumors.
Malignant cardiac tumors are primarily sarcomas, eg, angiosarcoma, rhabdomyosarcoma, and undifferentiated pleomorphic sarcoma, along with primary cardiac lymphomas. The pericardial compartment may host similar malignant entities, including angiosarcoma, synovial sarcoma, malignant mesothelioma, and germ cell tumors.Malignant cardiac tumors are rare and account for 10% to 25% of all primary cardiac neoplasms.[8] Tumors that metastasize to the heart are much more common than primary cardiac tumors, forming approximately 95% of all cardiac tumors, but are rarely surgically excised.[9]
Etiology
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Etiology
Genetic Etiologies of Cardiac Cancers
Some cardiac tumors occur in the setting of genetic predispositions or are part of genetic syndromes; this may have implications for genetic counseling and therapeutic implications for targeted therapies.[10]
Beckwith-Wiedemann syndrome
Beckwith-Wiedemann syndrome (BWS) is an imprinting disorder (11p15 region) causing fetal overgrowth (macroglossia, organomegaly, hemihypertrophy). Infants with BWS have an increased incidence of cardiac tumors, most often benign hamartomas such as rhabdomyomas or fibrous hamartomas in the heart. These cardiac lesions can present in the neonatal period alongside other BWS features.[11]
Neurofibromatosis type 1
Neurofibromatosis type 1 (NF1) is an autosomal dominant syndrome (NF1 gene on 17q) known for nerve sheath tumors and characteristic skin findings. Rarely, patients with NF1 can develop intracardiac neurofibromas. Cardiac neurofibromas in a young patient should prompt evaluation for NF1 (eg, café-au-lait spots, peripheral neurofibromas).[12]
Birt-Hogg-Dubé syndrome
Birt-Hogg-Dubé syndrome (BHD) is an autosomal dominant syndrome (FLCN gene) typically featuring skin fibrofolliculomas, lung cysts, and renal tumors. There are rare reports of cardiac rhabdomyomas in BHD, suggesting that this phakomatosis, like tuberous sclerosis complex, can occasionally involve the heart. Cardiac tumors in BHD are not common, but their possibility underscores the spectrum of hamartomas in this syndrome.[13]
Cowden syndrome
Cowden syndrome (eg, phosphatase and tensin homolog [PTEN] hamartoma tumor syndrome) is an autosomal dominant condition due to PTEN mutations, characterized by multiple hamartomas and a risk of malignancies (eg, breast and thyroid). Cardiac lipomas have been noted in Cowden syndrome, consistent with the propensity for benign overgrowths. Thus, an incidental cardiac lipoma, especially if multifocal, might warrant consideration of an underlying PTEN mutation.[14]
Other cardiac tumor-associated syndromes
Cardiac rhabdomyomas correlate with tuberous sclerosis, an autosomal dominant disorder characterized by the development of hamartomas in multiple organs.[15] Cardiac fibromas may correlate with Gorlin syndrome, an autosomal dominant disorder characterized by developmental abnormalities and a predisposition to neoplasia.[16] Cardiac myxomas may be part of the Carney complex, an autosomal dominant syndrome.[17]
Histiocytoid cardiomyopathy sometimes occurs in families, but the exact genetic abnormalities are unknown.[18] The genomic profile of most cardiac undifferentiated sarcomas is like that of pulmonary artery intimal sarcomas with recurrent alterations in MDM2, PDGFRA, and CDKN2A genes.[19] Papillary fibroelastomas have been reported after cardiac instrumentation and thoracic irradiation.[20]
Familial predisposition to malignant tumors
Though most primary cardiac malignancies are sporadic, certain cancer predisposition syndromes include heart tumors in their spectrum. For example, Li-Fraumeni syndrome (germline TP53 mutation) markedly increases the risk of various sarcomas and has been reported in patients with cardiac angiosarcoma. Likewise, germline mutations in POT1 (a telomere maintenance gene) have been identified in families with cardiac angiosarcomas. These inherited mutations in tumor suppressor genes call for high suspicion and early surveillance if a cardiac malignancy occurs in a young patient or a family history with a high potential.[21]
Acquired Primary Cardiac Tumors
Acquired primary tumors have also been linked with variable degrees of certainty to different causative agents, including:
- Prior cardiac surgery or instrumentation: Iatrogenic trauma to the endocardium can lead to reactive tumor formation years later. Papillary fibroelastomas, which are benign papillomatous growths on valves or endocardial surfaces, have been reported to develop at sites of surgical intervention (eg, valve replacement or other cardiac procedures). In a series of cardiac surgery cases, roughly half of papillary fibroelastomas occurred on valve apparatus that had been surgically manipulated. This suggests that endothelial injury or foreign material (prosthetic valves, catheter trauma) can trigger the growth of these tumor-like lesions.[22]
- Radiation exposure: Therapeutic thoracic radiation (eg, mediastinal irradiation for lymphoma or breast cancer) is a known risk factor for later cardiac tumors. Papillary fibroelastomas have occurred within the field of prior chest irradiation. More ominously, radiation can induce malignant cardiac tumors, particularly sarcomas of endothelial origin. Cardiac angiosarcomas (the most common primary malignant heart tumor) have been linked to prior radiation exposure in some cases, analogous to radiation-associated angiosarcoma of the breast or scalp. Patients who received high-dose mediastinal radiation should be monitored for late complications, including rare cardiac neoplasms.[23]
- Viral infection and immunosuppression: Oncogenic viruses can contribute to cardiac tumor development, especially under conditions of immune suppression. Notably, Epstein–Barr virus (EBV) is associated with primary cardiac lymphoma in immunocompromised individuals. Primary cardiac lymphoma (typically a diffuse large B-cell lymphoma confined to the heart) occurs more often in individuals with acquired immunodeficiency syndrome (AIDS) or posttransplant immunosuppression, due to EBV-driven lymphoproliferation. Similarly, Kaposi sarcoma, a vascular tumor caused by human herpesvirus 8 (HHV-8), can involve the heart in those with AIDS or transplant recipients. These viral-related tumors underscore the importance of immune status: prolonged immunosuppression or HIV infection creates an environment where latent oncogenic viruses can give rise to neoplastic lesions in the heart.[24]
Epidemiology
Primary cardiac tumors are much rarer than secondary malignant lesions, occurring in about 0.001% to 0.3% of autopsies. Almost 75% to 90% of primary cardiac tumors excised surgically are benign. Cardiac myxomas have wide recognition as the most common primary benign cardiac neoplasm of adulthood, representing nearly 80% of benign tumors. Secondary malignant disease of the heart and pericardium is considerably more common than primary cardiac malignant disease; in some estimates, secondary malignant cardiac cancers are 30 to 1000 times more common.
In a random autopsy series, the frequency of metastatic involvement was 0.4%; in patients with confirmed cancer, cardiac involvement can be as high as 20%. Spread to the heart is generally via direct tumor extension, venous/lymphatic spread, or arterial metastasis. The most common underlying malignant diseases with secondary cardiac involvement are carcinoma of the lung, breast, esophagus, stomach, kidneys, melanoma, lymphoma, and leukemia.[2]
Histopathology
Primary Benign Cardiac Tumors
Myxoma
Myxomas are the most common benign cardiac tumors in adults. They most frequently occur in the left atrium (approximately 80%), followed by the right atrium and, less commonly, the ventricles. They typically originate near the interatrial septum, adjacent to the fossa ovalis membrane. The nonfamilial form occurs slightly more often in women and usually presents in the sixth decade of life.
While most myxomas are sporadic, familial cases have been reported, particularly associated with Carney Complex (CNC). CNC is a rare autosomal dominant disorder characterized by pigmented skin lesions, multiple neoplasms, and endocrine abnormalities. This cardiac tumor is most often linked to inactivating mutations in the PRKAR1A gene, which encodes a regulatory subunit of protein kinase A, disrupting cyclic adenosine monophosphate signaling. Dermatologic findings commonly include lentigines and blue nevi, especially on the face, neck, and torso. Endocrine manifestations are key features and may involve hormone-secreting tumors, eg, growth hormone-producing pituitary adenomas, Sertoli cell tumors, and primary pigmented nodular adrenocortical disease, which often leads to adrenocorticotropic hormone-independent Cushing syndrome.
Macroscopically, myxomas are categorized into 2 gross subtypes: solid and villous. Solid myxomas can be globular or elongated with smooth, shiny, sometimes undulant surfaces, while villous myxomas have irregular, friable, papillary surfaces.[25] Histologically, the hallmark feature is the myxoma (or lepidic) cell—a cytologically bland cell with eosinophilic cytoplasm and oval to round nuclei. These stellate, ovoid, or spindle-shaped cells are embedded in a mucopolysaccharide-rich myxoid matrix. Thick-walled blood vessels are often found near the tumor stalk or base. Due to their fragile structure, myxomas are prone to embolization, which can lead to severe clinical complications.[26]
Lipomas
Lipomas are the second most frequent type of primary cardiac mass, accounting for approximately 0.5% of all cardiac tumors. They are most commonly found in the sub-endocardium and sub-epicardium of the left ventricle, right atrium, or interatrial septum.[27] Macroscopically, cardiac lipomas appear as yellow, soft, smooth, well-circumscribed, and encapsulated solitary masses. Microscopically, they are composed of mature white adipose tissue enclosed by a fibrous capsule, often with fibrous septa, and the fat cells exhibit minimal to no atypia. While most lipomas are asymptomatic, their location can lead to clinical manifestations such as arrhythmias, conduction system disturbances, or heart failure symptoms.
Papillary fibroelastoma
Papillary fibroelastoma, or endocardial papilloma, is the most frequent tumor of the cardiac valves, accounting for approximately 75% of valvular tumors, and most commonly arises on the aortic valve, followed by the mitral valve. These tumors are derived from endocardial cells and are predominantly found in the left-sided cardiac chambers (95%). Papillary fibroelastomas are more common in men than women and are typically diagnosed in the sixth to seventh decade of life.
Macroscopically, they appear round, whitish, and soft, and when placed in solution, their delicate fronds unfurl to resemble a sea anemone. The tumors typically range from 2 to 50 mm in size and are usually attached to the endocardium by a single stalk.[4][28] Microscopically, they feature narrow, avascular papillary fronds with complex branching patterns, and their collagen and elastic fiber arrangement resembles the tendinous cords of the atrioventricular valves. Clinically, embolization is common, and affected valves may exhibit insufficiency or obstruction. Typical presentations include heart failure symptoms and the appearance of a new murmur on physical examination.
Rhabdomyomas
Rhabdomyomas are the most common cardiac tumors in children and often occur before the first year of life. Over 86% of these tumors have been reported in association with tuberous sclerosis.[29] Multiple rhabdomyomas can occur at once, most commonly affecting the ventricles. Macroscopically, they appear as well-defined, unencapsulated, whitish or grayish nodular masses ranging in size from millimeters to several centimeters.
Microscopically, they are composed of well-demarcated nodules of enlarged, vacuolated cells with clear cytoplasm due to glycogen deposits, often exhibiting a characteristic "spider cell" appearance. Clinical symptoms result from obstruction of the cardiac chambers or disturbances in the conduction system, such as heart block or ventricular tachyarrhythmias.[30] Most rhabdomyomas regress spontaneously after birth, and surgical resection is rarely necessary, except in cases of significant obstruction or arrhythmia.
Fibromas
Fibromas are the second most frequent cardiac tumor found in childhood and show a slight male predominance. These intramural tumors primarily occur in the left ventricular septum. Macroscopically, cardiac fibromas are well-circumscribed, firm, solitary white masses that resemble fibromatosis or uterine leiomyomas, with a typically whorled appearance on cut section. Microscopically, they consist of monomorphic fibroblasts with little or no atypia, and the tumor margins may infiltrate the surrounding cardiac muscle.[31] Clinically, symptoms align with those of heart failure and can include an elevated risk of ventricular tachyarrhythmias and sudden cardiac death.
Primary Malignant Cardiac Tumors
Cardiac sarcomas
Angiosarcoma is the most common primary malignant cardiac tumor, with its incidence increasing notably in the fourth decade of life. This condition most frequently arises in the right atrial chamber. Macroscopically, angiosarcomas appear as dark brown or black hemorrhagic masses with infiltrating borders. Microscopically, they infiltrate the cardiac muscle without distinct margins and are characterized by irregular, anastomosing, and sinusoidal vascular channels lined with pleomorphic cells that exhibit frequent mitotic figures.[32] The prognosis for patients with angiosarcoma is generally poor.
Secondary cardiac tumors
Secondary cardiac tumors, or metastatic involvement of the heart, occur more frequently and may occur through direct invasion from the mediastinum or via hematogenous spread.[33] Clinical suspicion for cardiac metastasis or tumor extension should be raised in patients presenting with new symptoms suggestive of heart failure or valvular disease. One of the most common findings in such cases is pericardial effusion. Histologically, the nature of the cardiac mass in secondary tumors is defined by the characteristics of the underlying malignancy responsible for the cardiac involvement.
History and Physical
The clinical symptoms of different cardiac tumors are often nonspecific and insidious, which can delay diagnosis and surgical management. The symptoms may relate to processes in the heart, eg, valvular dysfunction, pericardial effusion with tamponade, intracardiac blood flow obstruction, arrhythmia, and congestive heart failure. An increasing proportion of cardiac tumors induce no symptoms and become obvious only as an incidental finding.[34] The clinical manifestations depend on the tumor's size, anatomical location, growth, and invasiveness.[34]
Evaluation
Multiple imaging modalities can be useful in determining mass characteristics and origin. Ultimately, due to the complexity of histological evaluation of cardiac masses, most of these tumors, unless removed, cannot be diagnosed with certainty. The following imaging techniques are primarily used to determine cardiac masses:
Echocardiogram
Echocardiogram (ECG) is a crucial imaging technique that will likely aid in diagnosing the cardiac tumor. Sensitivity and specificity are as high as 90% and can be performed easily at the bedside. If additional detail is necessary, a transesophageal echocardiogram can help further assess the cardiac tumor's characteristics and location. This modality can determine the tumor's size, mobility, extension, and relation with neighboring structures; it can also determine dynamics related to the mass, such as obstructions or valvular insufficiency.
A transesophageal echocardiogram can also increase the visualization of small tumors that are unclear in the transthoracic echocardiogram. Contrast echocardiography is also useful in distinguishing between cardiac tumors and cardiac thrombi. A 3-dimensional echocardiography is an additional feature to determine the tumor's dimensions, shape, and structural involvement (see Image. Transesophageal Echocardiography).[35][36]
Cardiac Magnetic Resonance Imaging
Cardiac magnetic resonance (CMR) is a highly accurate tool for characterizing cardiac masses, offering detailed insights into their size, location, mobility, and effects on surrounding structures. Beyond its superior resolution compared to echocardiography, CMR uniquely provides tissue characterization, distinguishes cystic or edematous tissue through water content quantification, and assesses vascularity via first-pass perfusion.[37]
Late gadolinium enhancement helps detect extracellular matrix, with its absence suggesting thrombus. In differentiating benign from malignant lesions, CMR has shown up to 79% accuracy in small cohorts. Fat suppression sequences further aid in identifying fatty tumors such as lipomas or liposarcomas. For instance, a myxoma may present with low T1 signal and minimal contrast uptake, reflecting low vascularity and matrix content—features indicative of a benign lesion.[38]
Computed Tomography Scan
This imaging study is useful for assessing cardiac mass and adjacent structures, and can determine the nature of the mass by evaluating the x-ray attenuation.[39] The calcification of the mass is also visible in this imaging study, and it can also assess small masses.
Left Heart Catheterization with Coronary Angiogram
Left cardiac catheterization can help determine the blood supply and the adjacent structures to tumors.[40] The technique can also help determine the tumor's invasion of the epicardial vessels, and with ventriculography, clinicians can determine the tumor's extension into the ventricular chamber.
Biopsy and Histological Evaluation
In vivo, intracardial biopsy is a risky procedure mostly done at high-volume centers with experienced surgeons. Right-sided tumors are more likely to be biopsied to determine the histology of the tumor.[41] Left-sided tumor biopsy has a higher risk of perforation and systemic embolization during the procedure. Imaging-guided biopsy increases a biopsy's success rate and provides a good tissue specimen for determining histological origin.[42] Cytology from the pericardial fluid can also be used to determine the underlying nature of tumors. Ultimately, surgical resection of the tumor with histological evaluation and fluoroscopy is used to determine the type of tumor.
Treatment / Management
Primary Benign Cardiac Tumors
Primary benign cardiac tumors are typically managed based on symptomatology and risk of complications. Recommended treatment strategies include:
- Myxoma: Surgical removal of the myxoma is necessary due to the high risk of embolization secondary to the friable structure, with tumor-free margins needed to prevent recurrence.[43]
- Lipomas: Lipomas generally do not need intervention unless symptomatic or causing arrhythmias.
- Papillary fibroelastoma: Left-sided papillary fibroelastomas should undergo surgical resection in patients with large mobile masses or with evidence of embolization.
- Rhabdomyoma: Rhabdomyomas often regress spontaneously, with surgery reserved for cases causing obstruction or arrhythmias. Fibromas should be resected entirely when feasible.
- Fibroma: Complete surgical resection, when feasible, is recommended.[44]
Primary Cardiac Sarcomas
Most sarcomas have an overall poor prognosis; some study results have found an increase in survival rate with complete surgical resection of the sarcoma compared with patients without surgical management. Surgical removal after diagnosis is the recommendation.[45]
Secondary Cardiac Tumors
Treatment of the underlying malignancy helps with cardiac invasion, but cardiac invasion or metastasis is very frequently a poor prognostic factor. Pericardial effusion is managed via pericardial drainage, and if reaccumulation occurs, clinicians can perform a pericardial window.
Differential Diagnosis
Cardiac masses may be an overlapping finding with several differential diagnoses, such as thrombus or vegetation. Therefore, the clinical scenario is vital to determine the underlying etiology of the cardiac mass. Intracardiac or prosthetic devices in the infection setting increase the suspicion that a cardiac mass is a vegetation. Heart failure symptoms in patients with underlying malignancy can also be related to cardiotoxicity from chemotherapy, and this requires evaluation before determining possible metastasis or invasion of the primary tumor. Infectious diseases can also produce intracardial and pericardial masses or effusion; microbiological assessment is necessary.
Treatment Planning
When evaluating the feasibility of surgically removing a primary malignant cardiac tumor, several critical factors must be taken into account; these include the tumor’s histopathological subtype, its size and anatomical location, the depth of myocardial infiltration, and its proximity to vital structures, eg, the cardiac valves and the fibrous skeleton of the heart. Equally important is determining whether the lesion can be completely excised with negative margins and confirming the absence of distant metastatic disease. Beyond tumor-specific characteristics, the patient’s general health is pivotal in guiding the surgical decision. Age, baseline functional and mental status, comorbidities, and overall frailty must be carefully weighed to ensure the benefits of a major cardiac intervention outweigh the risks.
In cases where malignancy is confirmed—typically through endomyocardial biopsy—and imaging demonstrates the tumor is amenable to complete excision, surgical resection should be considered, especially if a margin of at least 1 cm can be achieved without compromising essential cardiac function. When radical resection is feasible, it may not only provide superior palliation compared to medical management alone but can also lead to improved survival outcomes. This aligns with the oncologic surgical principle of residual tumor (R) classification, where an R0 resection (no residual tumor) is associated with significantly better prognosis than R1 (microscopic residual) or R2 (macroscopic residual) resections.[46]
Prognosis
The prognosis of the different cardiac tumors depends on their nature and mechanical disturbances (obstruction/insufficiency). Primary benign cardiac tumors have the best prognosis unless multiple embolizations occur, especially when systemic, because this can cause numerous strokes with different neurological involvement and physical limitations. A similar situation occurs when the tumor causes mechanical abnormalities in the conduction system, inducing arrhythmias that can be life-threatening or valvular disease-causing syncope or heart failure symptoms.[43]
Malignant tumors, especially primary tumors, have the worst overall prognosis among all cardiac tumors. Survival after the first year of diagnosis is low, especially in the absence of surgical excision.[47] Secondary cardiac tumors have a poor prognosis, but mainly because the primary tumor is likely to be advanced, with various areas of metastasis; a mechanical compromise also occurs in this scenario.
Complications
Complications due to cardiac tumors develop secondary to mechanical factors. Embolization causing strokes is a frequent complication that can present as a stroke, and later, while performing a stroke assessment, a clinician can find the cardiac mass. Valvular disease, eg, obstruction or insufficiency, is common when the mass is adjacent to the leaflets. Syncope can occur when there is a blood flow obstruction due to the mass, causing hypoperfusion in the brain and the coronary arteries and predisposing to tachyarrhythmias.
The presence of masses next to the conduction system in the epicardium and myocardium can generate electrical abnormalities, eg, atrioventricular block, complete heart block, and ventricular tachyarrhythmias. Pericardial involvement and pericardial effusion can cause a hemodynamic disturbance with hypotension, shortness of breath, and even cardiac tamponade.
Deterrence and Patient Education
Patients diagnosed with cardiac masses need extensive evaluation to determine the etiology and prevent decompensation. Cardiac masses can be abnormal tissue limited to the heart tissue and, after removal, can prevent mechanical complications.
Enhancing Healthcare Team Outcomes
Mangaing cardiac cancers demands a highly coordinated, interprofessional approach to ensure timely diagnosis, reduce complications, and improve patient outcomes. Given the wide differential diagnosis associated with cardiac masses, healthcare professionals must work collaboratively to ensure accurate identification and appropriate intervention. Cardiologists often initiate the diagnostic process through imaging and clinical assessment, while cardiac surgeons evaluate the feasibility and timing of resection. Electrophysiologists contribute expertise when tumors interfere with the conduction system, and internists help manage comorbidities that may complicate treatment. Intensivists oversee those who are critically ill, providing life-sustaining therapies and coordinating with the broader care team during acute decompensations. Advanced clinicians assist in diagnostic evaluations, ongoing monitoring, and patient education—bridging communication across specialties.
Cardiac nurses are essential in providing direct patient care, monitoring for hemodynamic changes, and alerting the team to early signs of deterioration. Pharmacists ensure safe and effective medication use, particularly in managing anticoagulation, chemotherapy, or supportive therapies. Optimal patient-centered care hinges on clear, timely interprofessional communication and a shared understanding of the patient’s condition, goals, and treatment plan. Care coordination among team members is vital to streamline diagnostics, facilitate surgical planning, and manage postoperative care. Through collaborative rounds, electronic health record updates, and shared decision-making, the team enhances safety, reduces delays, and supports the emotional and informational needs of patients and families. This cohesive strategy ultimately improves clinical outcomes, enhances patient satisfaction, and strengthens team performance.
Media
(Click Video to Play)
Transesophageal Echocardiography. A left atrial myxoma identified by transesophageal echocardiography (4-chamber view).
G.steph.rocket, Public Domain, via Wikimedia Commons
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