Primary cardiac tumors
Updates to Article Attributes
Primary cardiac tumours are uncommon and comprise only a small minority of all tumours that involve the heart: most are mediastinal or lung tumours that extend through the pericardium and into the heart, or metastases 1.
Epidemiology
Primary cardiac tumours have an estimated autopsy prevalence of 0.001-0.03% 2.
Clinical Presentation
The most common presenting symptoms include shortness and breath and chest discomfort, however many cases are discovered incidentally. On physical exam a characteristic auscultatory "plop" in late diastole is classically associated with left atrial myxomas of the polypoid variety 11. Other reported symptoms may include:
-
constitutional symptoms
- anorexia, fatigue, fever
- typically associated with myxomas
-
focal neurologic deficits
- secondary to cardioembolic stroke/TIA 12
-
arrhythmogenic syncope, obstructive shock
- rhabdomyomas and fibromas are associated with a predilection for malignant dysrhythmias
- the former may cause a structural inflow or outflow obstruction, potentially a substrate for obstructive shock10
Pathology
Primary cardiac tumours can then be divided into:
-
benign cardiac tumours: 60-75% 1,4
-
cardiac myxoma
:- most common in adults, accounting for over half of benign primary cardiac tumours
-
cardiac lipoma
(-
incidence ≈10% 4, second most common in adults 6
)
-
incidence ≈10% 4, second most common in adults 6
- cardiac rhabdomyoma: most common in children
- papillary fibroelastoma
- cardiac fibroma
- cardiac haemangioma
- cardiac paraganglioma
- pericardial teratoma (can rapidly grow despite being benign) 7,8
-
cardiac myxoma
-
malignant cardiac tumours
-
sarcomas account for 25% of allcardiac tumours 2,6
-
cardiac angiosarcoma
:- most common malignant primary cardiac tumour 3,9
- undifferentiated pleomorphic sarcoma of the heart
- cardiac leiomyosarcoma
- cardiac spindle cell sarcoma
- cardiac fibrosarcoma
- cardiac liposarcoma
- primary cardiac osteosarcoma: 3-9% of primary cardiac malignant tumours 9
-
cardiac angiosarcoma
- malignant fibrous histiocytoma of heart
- cardiac haemangiopericytoma
- primary cardiac lymphoma
- pericardial mesothelioma
-
sarcomas account for 25% of allcardiac tumours 2,6
Radiographic features
Echocardiography
Intracardiac masses are often discovered and initially evaluated with transthoracic echocardiography (TTE) which allows delineation of the anatomic location, degree of mobility, dimensions, and any associated perturbation of cardiac structure (e.g. disruption of valvular integrity leading to regurgitation, or occlusion of the valvular orifice leading to stenosis) and function. Pericardial extension can also be delineated. Transthoracic evaluation is limited both by inherent anatomic barriers to visualisation of several pertinent structures such as the great vessels, and may be further limited by patient specific factors, such as prior surgeries or dressings, chest wall abnormalities, body habitus, and hyperinflated lungs.
Transoesophageal echocardiography (TEE) is particularly utile for evaluation of masses which may be related to a valvular apparatus and is superior for imaging structures within the atria. It is particularly useful for delineating the origin/attachment site of masses (commonly difficult with a transthoracic approach).
3-dimensional echocardiography (3DE) is often sought for its ability to detail the anatomical relationships of masses, provide better definition of mass shape and dimensions, and additional details subtle structural elements within the mass which would not otherwise be apparent e.g. subtle internal trabeculation.
Sonographic contrast agents may also provide additional diagnostic yield, allowing one to assess the presence of vascularity within a mass, and the relative abundance or dearth of perfusion. Typical features associated with benign tumours include qualitatively small amounts of microbubbles circulating throughout the mass (i.e. lacking the hypervascularity associated with malignancy), appearing to have a lesser degree of enhancement to the adjacent myocardium. Identification of intra-cavitary masses may also be enhanced with their presence indicated by a filling defect within the relevant cardiac chamber 14.
MRI
While of limited utility in structures subject to a high degree of motion (such as masses associated with a valve) cardiac magnetic resonance imaging (CMR) allows both qualitative and quantitative evaluation of the mass in question as well as cardiac structure, function, and adjacent structures 15. Features salient to assess on CMR include:
- location, shape/size, involvement of adjacent anatomical structures 14
- absence or presence (and degree/pattern of) late gadolinium enhancement
CT
As metastases are far more common than primary tumours, computed tomography (CT) of the chest, abdomen, and pelvis may be considered to identify a primary source of malignancy, with common culprits including:
-
haematologic malignancy
- lymphoma, leukaemia
- haematogenous seeding from melanoma may also occur
- breast cancer
- lung cancer
- oesophageal cancer
- renal cell carcinoma
CTCA may also be implemented to evaluate masses though to involve coronary vessels and allows identification of extra-cardiac extension of a tumour into adjacent structures. Contrast enhancement allows insight into the vascular nature of the mass, and fat/calcifications are visualised well with CT 15.
See also
-<p><strong>Primary cardiac tumours</strong> are uncommon and comprise only a small minority of all tumours that involve the <a href="/articles/heart">heart</a>: most are mediastinal or <a href="/articles/lung-cancer-3">lung tumours</a> that extend through the pericardium and into the heart, or metastases <sup>1</sup>.</p><h4>Epidemiology</h4><p>Primary cardiac tumours have an estimated autopsy prevalence of 0.001-0.03% <sup>2</sup>. </p><h4>Pathology</h4><p>Primary cardiac tumours can then be divided into:</p><ul>- +<p><strong>Primary cardiac tumours</strong> are uncommon and comprise only a small minority of all tumours that involve the <a href="/articles/heart">heart</a>: most are mediastinal or <a href="/articles/lung-cancer-3">lung tumours</a> that extend through the pericardium and into the heart, or metastases <sup>1</sup>.</p><h4>Epidemiology</h4><p>Primary cardiac tumours have an estimated autopsy prevalence of 0.001-0.03% <sup>2</sup>. </p><h4>Clinical Presentation</h4><p>The most common presenting symptoms include shortness and breath and chest discomfort, however many cases are discovered incidentally. On physical exam a characteristic auscultatory "plop" in late diastole is classically associated with left atrial myxomas of the polypoid variety <sup>11</sup>. Other reported symptoms may include:</p><ul>
- +<li>constitutional symptoms<ul>
- +<li>anorexia, fatigue, fever</li>
- +<li>typically associated with myxomas</li>
- +</ul>
- +</li>
- +<li>focal neurologic deficits<ul><li>secondary to cardioembolic stroke/TIA <sup>12</sup>
- +</li></ul>
- +</li>
- +<li>arrhythmogenic syncope, obstructive shock<ul>
- +<li>rhabdomyomas and fibromas are associated with a predilection for malignant dysrhythmias</li>
- +<li>the former may cause a structural inflow or outflow obstruction, potentially a substrate for obstructive <a href="/articles/shock">shock</a> <sup>10</sup>
- +</li>
- +</ul>
- +</li>
- +</ul><h4>Pathology</h4><p>Primary cardiac tumours can then be divided into:</p><ul>
-<a href="/articles/cardiac-myxoma">cardiac myxoma</a>: most common in adults</li>- +<a href="/articles/cardiac-myxoma">cardiac myxoma</a><ul><li>
- +<a href="/articles/cardiac-myxoma"></a>most common in adults, accounting for over half of benign primary cardiac tumours</li></ul>
- +</li>
-<a href="/articles/cardiac-lipoma">cardiac lipoma </a>(≈10% <sup>4</sup>, second most common in adults <sup>6</sup>)</li>- +<a href="/articles/cardiac-lipoma">cardiac lipoma </a><ul><li>
- +<a href="/articles/cardiac-lipoma"></a>incidence ≈10% <sup>4</sup>, second most common in adults <sup>6</sup>
- +</li></ul>
- +</li>
-<a href="/articles/cardiac-angiosarcoma">cardiac angiosarcoma</a>: most common malignant primary cardiac tumour <sup>3,9</sup>- +<a href="/articles/cardiac-angiosarcoma">cardiac angiosarcoma</a><ul><li>
- +<a href="/articles/cardiac-angiosarcoma"></a>most common malignant primary cardiac tumour <sup>3,9</sup>
- +</li></ul>
-<li><a title="Cardiac undifferentiated pleomorphic sarcoma" href="/articles/cardiac-undifferentiated-pleomorphic-sarcoma">undifferentiated pleomorphic sarcoma of the heart</a></li>- +<li><a href="/articles/cardiac-undifferentiated-pleomorphic-sarcoma">undifferentiated pleomorphic sarcoma of the heart</a></li>
- +</ul><h4>Radiographic features</h4><h5>Echocardiography</h5><p>Intracardiac masses are often discovered and initially evaluated with <a href="/articles/transthoracic-echocardiography">transthoracic echocardiography</a> (TTE) which allows delineation of the anatomic location, degree of mobility, dimensions, and any associated perturbation of cardiac structure (e.g. disruption of valvular integrity leading to <a href="/articles/mitral-valve-regurgitation">regurgitation</a>, or occlusion of the valvular orifice leading to <a href="/articles/mitral-valve-stenosis-1">stenosis</a>) and function. Pericardial extension can also be delineated. Transthoracic evaluation is limited both by inherent anatomic barriers to visualisation of several pertinent structures such as the great vessels, and may be further limited by patient specific factors, such as prior surgeries or dressings, chest wall abnormalities, body habitus, and hyperinflated lungs.</p><p><a href="/articles/transesophageal-echocardiography">Transoesophageal echocardiography</a> (TEE) is particularly utile for evaluation of masses which may be related to a valvular apparatus and is superior for imaging structures within the atria. It is particularly useful for delineating the origin/attachment site of masses (commonly difficult with a transthoracic approach).</p><p><a href="/articles/3d-ultrasound">3-dimensional echocardiography</a> (3DE) is often sought for its ability to detail the anatomical relationships of masses, provide better definition of mass shape and dimensions, and additional details subtle structural elements within the mass which would not otherwise be apparent e.g. subtle internal trabeculation. </p><p>Sonographic <a href="/articles/contrast-medium">contrast agents</a> may also provide additional diagnostic yield, allowing one to assess the presence of vascularity within a mass, and the relative abundance or dearth of perfusion. Typical features associated with benign tumours include qualitatively small amounts of microbubbles circulating throughout the mass (i.e. lacking the hypervascularity associated with malignancy), appearing to have a lesser degree of enhancement to the adjacent myocardium. Identification of intra-cavitary masses may also be enhanced with their presence indicated by a filling defect within the relevant cardiac chamber <sup>14</sup>. </p><h5>MRI</h5><p>While of limited utility in structures subject to a high degree of motion (such as masses associated with a valve) <a href="/articles/cardiac-mri">cardiac magnetic resonance imaging</a> (CMR) allows both qualitative and quantitative evaluation of the mass in question as well as cardiac structure, function, and adjacent structures <sup>15</sup>. Features salient to assess on CMR include:</p><ul>
- +<li>location, shape/size, involvement of adjacent anatomical structures <sup>14</sup>
- +</li>
- +<li>absence or presence (and degree/pattern of) late <a href="/articles/gadolinium-1">gadolinium</a> enhancement</li>
- +</ul><h5>CT</h5><p>As metastases are far more common than primary tumours, computed tomography (CT) of the chest, abdomen, and pelvis may be considered to identify a primary source of malignancy, with common culprits including:</p><ul>
- +<li>haematologic malignancy<ul>
- +<li>lymphoma, leukaemia</li>
- +<li>haematogenous seeding from <a href="/articles/metastatic-melanoma">melanoma</a> may also occur</li>
- +</ul>
- +</li>
- +<li>breast cancer</li>
- +<li>lung cancer</li>
- +<li>oesophageal cancer</li>
- +<li><a href="/articles/renal-cell-carcinoma-1">renal cell carcinoma</a></li>
- +</ul><p><a href="/articles/cardiac-ct-prospective-high-pitch-acquisition">CTCA</a> may also be implemented to evaluate masses though to involve coronary vessels and allows identification of extra-cardiac extension of a tumour into adjacent structures. Contrast enhancement allows insight into the vascular nature of the mass, and fat/calcifications are visualised well with CT <sup>15</sup>.</p><h4>See also</h4><ul>
- +<li><a href="/articles/heart">cardiac anatomy</a></li>
- +<li><a href="/articles/echocardiography">echocardiography</a></li>
- +<li><a href="/articles/cardiac-mri">cardiac MRI</a></li>
- +<li><a href="/articles/cardiac-ct-1">coronary CT angiography</a></li>
- +<li>
- +<a href="/articles/positron-emission-tomography">positron emission tomography</a> (PET)</li>
- +<li><a href="/articles/secondary-malignant-cardiac-tumour">cardiac metastases</a></li>
- +<li><a href="/articles/neoplastic-pericardial-disease">pericardial effusion</a></li>
References changed:
- 10. Suehiro S, Matsuda M, Hirata T et al. Primary Cardiac Rhabdomyosarcoma Developed After Receiving Radiotherapy for Left Breast Cancer 18 Years Prior. J Cardiol Cases. 2017;15(6):181-3. <a href="https://doi.org/10.1016/j.jccase.2017.01.003">doi:10.1016/j.jccase.2017.01.003</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/30279774">Pubmed</a>
- 11. Buksa M & Haracić A. Late Diastolic Tumor "Plop" in an Asymptomatic Case of Right Atrial Myxoma. Med Arh. 1999;53(2):77-9. - <a href="https://www.ncbi.nlm.nih.gov/pubmed/10386041">Pubmed</a>
- 12. Bhattacharyya S, Khattar R, Senior R. Characterisation of Intra-Cardiac Masses by Myocardial Contrast Echocardiography. Int J Cardiol. 2013;163(1):e11-3. <a href="https://doi.org/10.1016/j.ijcard.2012.06.098">doi:10.1016/j.ijcard.2012.06.098</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/22805540">Pubmed</a>
- 13. Tyebally S, Chen D, Bhattacharyya S et al. Cardiac Tumors. JACC: CardioOncology. 2020;2(2):293-311. <a href="https://doi.org/10.1016/j.jaccao.2020.05.009">doi:10.1016/j.jaccao.2020.05.009</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/34396236">Pubmed</a>
- 14. Ragland M & Tak T. The Role of Echocardiography in Diagnosing Space-Occupying Lesions of the Heart. Clin Med Res. 2006;4(1):22-32. <a href="https://doi.org/10.3121/cmr.4.1.22">doi:10.3121/cmr.4.1.22</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/16595790">Pubmed</a>
- 15. Motwani M, Kidambi A, Herzog B, Uddin A, Greenwood J, Plein S. MR Imaging of Cardiac Tumors and Masses: A Review of Methods and Clinical Applications. Radiology. 2013;268(1):26-43. <a href="https://doi.org/10.1148/radiol.13121239">doi:10.1148/radiol.13121239</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/23793590">Pubmed</a>
- 15. Motwani M, Kidambi A, Herzog B, Uddin A, Greenwood J, Plein S. MR Imaging of Cardiac Tumors and Masses: A Review of Methods and Clinical Applications. Radiology. 2013;268(1):26-43. <a href="https://doi.org/10.1148/radiol.13121239">doi:10.1148/radiol.13121239</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/23793590">Pubmed</a>
- https://doi.org/10.1148/radiol.13121239
Tags changed:
- echo
- cardiac
- ultrasound
- cancer
- oncology
Image 7 CT (C+ arterial phase) ( create )
Image 8 CT (C+ portal venous phase) ( create )
Image 9 MRI (Triple IR DB LAX_SAXs_RV2Ch_4Ch) ( create )
Unable to process the form. Check for errors and try again.