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Everything you need to know about Computed Tomography (CT) & CT Scanning

Cardiac: Cardiac Tumors Imaging Pearls - Educational Tools | CT Scanning | CT Imaging | CT Scan Protocols - CTisus
Imaging Pearls ❯ Cardiac ❯ Cardiac Tumors

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  • “Tuberous sclerosis complex (TSC) is a relatively rare autosomal dominant neurocutaneous disorder secondary to mutations in the TSC1 or TSC2 tumor suppressor genes. Although manifestation of the classic triad of seizures, intellectual disability, and facial angiofibromas may facilitate timely diagnosis of TSC, the multisystem features that may indicate TSC in the absence of these manifestations remain highly variable. In addition, patients with TSC are at risk of developing multiple benign and malignant tumors in various organ systems, resulting in increased morbidity and mortality.”
    Tuberous Sclerosis: Current Update
    Wang MX et al.
    RadioGraphics 2021; 41:1992–2010
  • “Cardiac rhabdomyomas are considered a major feature of TSC. They manifest in up to 80% of TSC cases and are rarely seen in persons who do not have this disease. Therefore, TSC should be suspected if cardiac rhabdomyomas are identified. The majority (90%) of persons with TSC are found to have multiple rhabdomyomas after they present. At gross pathologic examination, cardiac rhabdomyomas are well-circumscribed nonencapsulated masses with diameters ranging from 2 to 20 mm. These benign tumors are characterized by irregular myocyte architecture, including “spider”cells, which are so named because of their unique cytoplasmic extensions.”
    Tuberous Sclerosis: Current Update
    Wang MX et al.
    RadioGraphics 2021; 41:1992–2010
  • Cardiac Tumors: Factoids
    - Primary tumors are rare and metastases to the heart are more common (20-40x more common)
    - 75% of primary cardiac tumors are benign (most commonly myxoma)
    - 25% of primary cardiac tumors are malignant (most commonly sarcomas)

  • Cardiac Masses on Cardiac CT: A Review 
    David Kassop  et al.
    Curr Cardiovasc Imaging Rep (2014) 7:9281 
  • Cardiac CT: Key Findings
    - size, 
    - quantity, 
    - location (cardiac chamber, pericardial involvement, extracardiac structures),
    - morphology (attach-ment, margin appearance, infiltration), 
    - tissue characteristics (calcification, fat attenuation, vascularity)
    - clinical correlation (known malignancy or infection, presence of a catheter, associated syndromes) 
  • Cardiac Myxoma: Facts
    - Myxoma is the most common primary cardiac tumor, account- ing for 25-50 % of cases.
    - typically affects middle-aged adults, 30-60 years of age, with a higher prevalence in women.
    - majority (60-75 %) of cases are located in the left atrium attached to the fossa ovalis by a thin stalk, though less common locations include the right atrium (15-20 %), inferior vena cava, ventricles, and the valve leaflets
  • Myxoma is the most common primary cardiac tumor, accounting for 25-50 % of cases. It typically affects middle-aged adults, 30-60 years of age, with a higher prevalence in women. A majority (60-75 %) of cases are located in the left atrium attached to the fossa ovalis by a thin stalk, though less common locations include the right atrium (15-20 %), inferior vena cava, ventricles, and the valve leaflets.  
    Cardiac Masses on Cardiac CT: A Review  
    David Kassop  et al.
    Curr Cardiovasc Imaging Rep (2014) 7:9281
  • “On cardiac CT, approximately two-thirds of myxomas are ovoid with a smooth or lobular shape, with the remainder villous in appearance. When visualized on non-contrast CT, they typically appear hypodense, consistent with blood, and may demonstrate calcifications more often in the right atrial location. On contrast-enhanced cardiac CT, myxomas appear as intracavitary filling defects with heterogeneous con- trast enhancement, though the intensity may be variable de- pending on their chronicity and whether necrosis or hemorrhage is present.”
    Cardiac Masses on Cardiac CT: A Review  
    David Kassop  et al.
    Curr Cardiovasc Imaging Rep (2014) 7:9281
  • Cardiac Lipoma
    - Lipoma is the second most common primary benign tumor and accounts for approximately 10 % of primary cardiac tumors 
    - Lipomas commonly occur in middle-aged and older adults. 
    - Lipomas are encapsulated, well-circumscribed tumors consisting of mature adipocytes that can occur any- where in the heart. Approximately 50 % arise in the epicardial or mid-myocardial layers, while the other half are subendocardial, where they create filling defects with a homogenous appearance of fat attenuation (density < -50 Hounsfield units [HU]). 
  • Papillary Fibroelastoma 
    - Papillary fibroelastoma is the third most common primary benign cardiac tumor with an incidence of up to 0.33 % in autopsy series 
    - Papillary fibroelastomas account for ap- proximately 75 % of all cardiac valvular tumors and affect men and women equally with a mean age of 60 years 
    - They are characterized by a collection of avascular fronds of dense connective tissue lined by endothelium and may arise from any endocardial surface, though the majority are found on the aortic and mitral valves. Most papillary fibroelastomas are solitary and small with an average diameter of 10 mm. 

  • Cardiac Masses on Cardiac CT: A Review 
    David Kassop  et al.
    Curr Cardiovasc Imaging Rep (2014) 7:9281 
  • Metastatic Tumors to the Heart
    - Malignant cardiac tumors are most often a result of metastatic disease arising by direct extension of adjacent organs or spread via hematogenous, lymphatic, or intracavitary routes 
    - The most common malignancies metastatic to the heart originate from the lung (35-40 %), followed by breast (10 %) and hematologic (10-20 %) carcinomas. Melanoma has the greatest propensity to metastasize to the heart, but it is often found late in the disease process
  • Metastatic Tumors to the Heart
    - The most frequent location of metastasis is the pericardium (65-70 %), followed by epicardium (25-35 %) and myocardium (30 %). Endocardial or intracavitary involvement is rarely observed (3.5 % of cases) [18•]. 
  • Cardiac Angiosarcoma
    - Angiosarcoma is the most common primary cardiac malignant tumor and is comprised of cells that develop multiple, irregular vascular channels. The primary site of origin is the right atrial free wall in 80 % of cases and less commonly the right ventricle or pericardium The tumor morphology typically consists of a large, multilobar mass with a heterogeneous composition that spreads along the epicardial surface and replaces the right atrial wall 
    - These tumors may also be localized to the pericardium and often invade adjacent cardiac structures leading to cardiomegaly and recurrent pericardial effusions 
  • Rhabdomyosarcoma: facts
    - Rhabdomyosarcoma is the second most common primary malignant tumor. It accounts for 4-7 % of cardiac sarcomas and remains the most common pediatric cardiac malignancy. 
    - It is a malignant tumor of striated muscle that always involves the myocardium. In contrast to angiosarcomas, rhabdomyosarcomas may arise from any location with no predilection for a specific cavity as 60 % of cases involve multiple sites of origin. Additionally, the tumors may invade the pericardial space with a characteristic nodular appearance .
  • Intracardiac Thrombus: facts
    Thrombus accounts for the most commonly encountered intra- cardiac mass. It can occur in any of the cardiac chambers, though it most often involves the left-sided structures. Thrombus formation can be caused by hypercoagulable states, systolic dysfunction with wall motion abnormalities, atrial fibrillation, or artificial devices. It typically appears as a hypodense, low-attenuation filling defect in a contrast pool within a cardiac chamber and may be differentiated from primary and secondary tumors by knowledge of predisposing risk factors, attachment location, shape, and lack of mobility. 
  • Left Atrial Appendage Thrombi
    Cardiac CT has very high sensitivity for excluding thrombus of the left atrial appendage but findings of low attenuation in the left atrial appendage (LAA) are not specific to thrombus as this often represents circulatory stasis, an incomplete mixing of contrast material and blood. This “pseudo” filling defect may mimic thrombus, especially in low-flow states. However, delayed imaging of the LAA may significant- ly improve the specificity to distinguish thrombus from circulatory stasis.
  • Left Ventricle Thrombus: facts
    Left ventricular thrombi are often located in an area of myocardial hypokinesis, dyskinesia or aneurysm formation. They are frequently crescent-shaped filling defects with broad based attachments. However, a pedunculated appearance has been observed and can mimic myxom. Chronic thrombi may develop spotty calcifications, though this feature has not been shown to significantly differentiate thrombus from myxoma. Thrombus within the left ventricle may be distinguished from myocardi- um by lower attenuation characteristics with a threshold of 65 HU providing a sensitivity and specificity of 94 % and 97 %, respectively. 
  • Valvular Vegatations
    Cardiac CT has demonstrated to be highly accurate in identifying valvular vegetations, with a sensitivity of 97 % and specificity of 88 % as compared to transesophageal echocardiography. On cardiac CT, vegetations appear as low- attenuation masses usually involving the valve leaflet free edge. As a result, vegetations can lead to valvular destruction and/or dysfunction and cardiac CT is partic- ularly helpful in identifying perivalvular complications, such as perivalvular abscess formation, valve perforation, aortic pseudoaneurysms or extracardiac manifestations, such as sep- tic emboli to the lung parenchyma. 
  • Pericardial Cyst: Facts
    Pericardial cysts are benign, congenital lesions that ac- count for 7 % of all mediastinal tumors [35]. Over 75 % are located within the cardiophrenic spaces, the majority of which have a right-sided predominance. This anatomic location helps to distinguish pericardial cysts from other similar findings to include bronchogenic cysts, thymic cysts, and cardiac teratomas. Additionally, with pericardial cysts, there is no connection with the pericardial space, unlike that of pericardial diverticulae. On cardiac CT, pericardial cysts are thin-walled structures that are sharply demarcated, lack septae, and have a homogenous appearance. They are non-enhancing lesions with intravenous contrast administration and have attenuation similar to water (-10 to 20 HU). 
    Cardiac Masses on Cardiac CT: A Review 
    David Kassop  et al.
    Curr Cardiovasc Imaging Rep (2014) 7:9281 
  • Cardiac Tumor: Clinical Presentation
    1.Systemic: constitutional (fever, arthralgias, weight loss, fatigue) and paraneoplastic syndromes (PCTs).
    2.Cardiac: mass effect interfering with myocardial function or blood flow, resultant arrhythmias, interference with heart valves causing regurgitation, or pericardial effusion with or without tamponade. Typical symptoms include dyspnea, chest discomfort, pre-syncope, or syncope.
    3. Embolic: pulmonary and/or systemic thromboembolic phenomenon from the tumor.
    Cardiac Tumors: JACC CardioOncology State-of-the-Art Review.” 
    Tyebally, Sara et al. 
    JACC. CardioOncology vol. 2,2 293-311. 16 Jun. 2020, 
  • Cardiac Myxomas: Facts
    - Cardiac myxomas are the most common PCT and are believed to be derived from mesenchymal cell precursors. 
    - They form intracavitary masses, which are most commonly found in the left atrium attached by a stalk to the fossa ovalis, but may be seen in the right atrium in children. Other anatomical origins include the atrial free wall and mitral valve leaflets; however, these are less likely. 
    - Association with Carney complex (a multiple neoplasia and lentiginosis syndrome) is well established in 7% of patients found to have a cardiac myxoma on presentation.
    - Mean age at diagnosis is 50 years, and approximately 70% occur in women.
  • Cardiac myxomas: Facts
    - On cardiac CT, myxomas often manifest as a low-attenuation intracavitary mass with a smooth or slightly villous surface. 
    - Calcification is seen in approximately 14% of patients and is more commonly associated in right-sided lesions. 
    - Arterial-phase contrast enhancement is usually not present , but heterogeneous enhancement is recognized on studies performed with a longer time delay. 
  • Cardiac Lipoma
    - Lipoma is the second most common primary benign cardiac neoplasm (8% to 12%) and most commonly occurs in middle-aged and older adults. 
    - Approximately 50% of lipomas originate from the subendocardial layer, and the other half arise from the subepicardial or myocardial layers and grow into the pericardial sac. 
    - They are typically asymptomatic but may cause arrhythmias or valvular dysfunction . Subepicardial lipomas can compress the coronary arteries, which leads to ischemic chest pain.
  • Cardiac Angiosarcoma: Facts
    - highly aggressive tumors consisting of irregularly shaped vascular channels lined by anaplastic epithelial cells with sizeable areas of necrosis and hemorrhage.
    - They preferentially affect men and have a peak incidence in the fourth decade of life. 
    - They are of right atrial origin in approximately 75% of cases and typically fill this chamber and then infiltrate into the pericardium, tricuspid valve, right ventricle, and right coronary artery . 
    - Metastases develop in 47% to 89% of patients, most commonly to the lungs but also to the bone, colon, and brain. Patients typically present with symptoms of right heart failure, shortness of breath due to hemopericardium, and palpitations secondary to supraventricular arrhythmias.
  • Primary Cardiac Lymphoma: Facts
    - Primary cardiac lymphomas are mainly aggressive B-cell lymphomas seen more commonly in immunocompromised individuals. These tumors have a predilection for the right side of the heart and can present with nonspecific symptoms, including constitutional symptoms.
    - Nearly all primary cardiac lymphomas are aggressive B-cell lymphomas, with an increasing incidence secondary to lymphoproliferative disorders related to Epstein-Barr virus in patients with AIDS and in patients who have received transplants. The mean age at diagnosis is 63 years of age. 
    - They most commonly involve the right side of the heart, particularly the right atrium, but any chamber can be involved. There are frequently multiple lesions.
  • Cardiac Metastases
    Cardiac metastases are 20 to 40 times more common than PCTs. Up to 12% of oncology patients have metastases to the heart or pericardium at autopsy, although most remain clinically silent. Melanomas have the greatest propensity for cardiac involvement, whereas carcinomas of the thorax, including breast, lung, and esophageal, are the most common carcinomas that metastasize to the heart. Malignant tumors use 1 of 4 routes to reach the heart , including hematogeneous, lymphatic, transvenous spread, or direct invasion. In addition, the metastatic route significantly determines the target tissue. For instance, masses that spread through the lymphatics often seed the pericardium or epicardium.

  • Pathology, imaging, and treatment of cardiac tumours.
    Maleszewski, J., Anavekar, N., Moynihan, T. et al.  
    Nat Rev Cardiol 14, 536–549 (2017).

  • Pathology, imaging, and treatment of cardiac tumours.
    Maleszewski, J., Anavekar, N., Moynihan, T. et al.  
    Nat Rev Cardiol 14, 536–549 (2017).
  • “Primary cardiac tumours are very rare with a prevalence rate ranging from 0.001 to 0.03% in autopsy series. Most primary cardiac tumours are benign, such as myxoma, lipoma, papillary fibroelastoma and rhabdomyoma. Angiosarcomas and myxofibrosarcomas are the most common sarcomas of the heart. However, a primary cardiac chondroblastic osteosarcoma in the heart is a unique case, due to the fact that this type is considered to be a member of the extraskeletal osteosarcoma (ESOS) family.”
    Extraskeletal chondroblastic osteosarcoma in the left atrium  
    Özge Korkmaza et al.
    Interactive CardioVascular and Thoracic Surgery 19 (2014) 1077–1079
  • “ESOS is an uncommon malignant neoplasm, which can produce osteoid, bone or chondroid matrix, located in the soft tissue without connection to the skeleton. The most common location is the lower extremity, especially the thigh, followed by the upper extremity and the retroperitoneum.”
    Extraskeletal chondroblastic osteosarcoma in the left atrium  
    Özge Korkmaza et al.
    Interactive CardioVascular and Thoracic Surgery 19 (2014) 1077–1079
  • “The current study reported a primary cardiac chondroblastic osteosarcoma, which is a subtype of ESOS and in which the heart is an uncommon localization. Unfortunately, this tumour has aggressive behaviour and poor prognosis.”
    Extraskeletal chondroblastic osteosarcoma in the left atrium  
    Özge Korkmaza et al.
    Interactive CardioVascular and Thoracic Surgery 19 (2014) 1077–1079
  • “Extraskeletal osteosarcomas are rare malignancies that account for about 1% of all soft tissue sarcomas. Only a few large series of these tumours have been reported. These tumours are usually located in the lower and upper extremities, the thigh, or the buttocks (45–62%).4 Because of its rarity and the absence of a specific biomarker, extraskeletal osteosarcoma is easily mistaken for myositis ossificans or a benign tumour.The heart and pericardium are unusual sites for primary tumours, the prevalence rate in autopsy studies being only 0.001–0.3%; metastatic tumours are far more common.”
    Primary extraskeletal chondroblastic osteosarcoma of the pericardium: a case report and literature review,  
    Qianqian Wang et al.  
    European Heart Journal - Case Reports, Volume 4, Issue 4, August 2020, Pages 1–7
  • “Primary cardiac neoplasms occur at an incidence of 30 per 100,000 people per year. Approximately 80% of primary cardiac tumors are benign. The World Health Organization has classified neoplasms of the heart into either benign tumors and tumorlike lesions such as myxoma, malignant lesions such as angiosarcoma, and pericardial tumors such as solitary fibrous tumors. In patients older than 16 years, the most common primary cardiac neoplasms are myxomas, lipomatous tumors, and papillary fibroelastomas. In patients younger than 16 years, the most common tumors are rhabdomyomas, teratomas, fibromas, and myxomas.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • "The most common presenting symptom of a cardiac tumor is dyspnea, but the manifestations of a neoplasm will depend on the location of the lesion and size. Even benign cardiac tumors may cause obstruction of blood flow, decreased cardiac output, arrhythmia, or heart failure, which can be fatal. In addition, systemic manifestations such as fatigue, anorexia, and fever may also be seen.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • "Cardiac myxoma is the most common benign primary cardiac neoplasm, accounting for up to 80% of all cases,5 but only represent 10% of benign primary cardiac tumors in children. Approximately 3% to 10% of cardiac myxomas are associated with Carney complex,6 an autosomal dominant disorder characterized by pigmented lesions of the skin and mucosae, cardiac myxomas, cutaneous tumors, and multiple other endocrine and nonendocrine neoplasms.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • "Although they can be located in any chamber, most myxomas are located within the left atrium and originate at the interatrial septum. These lesions may have an irregular border, a pedunculated morphology, and be mobile. The size of the tumor is related to the degree of mobility and the potential that the lesion can obstruct the atrio- ventricular valve.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • "Although computed tomography (CT) is not the preferred method to characterize the tumor, typical findings on a contrast-enhanced CT include a spherical or ovoid mass that is lower in attenuation than surrounding myocardium.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • “Rhabdomyoma is the most common primary cardiac tumor of infancy and childhood representing 60% of pediatric primary cardiac neoplasms.These lesions are most often diagnosed during the first year of life or prenatally. Cardiac rhabdomyomas are associated with tuberous sclerosis in 30% to 50% of cases but also occur sporadically and rarely in association with congenital heart disease. Tuberous sclerosis is char- acterized by cortical tubers and subependymal nodules within the brain, multiple retinal hamarto- mas, adenoma sebaceum of the skin, and periungual fibromas.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • “Rhabdomyomas are most commonly located within the ventricles attached to the myocardium but are less commonly located in the atrioventricular groove. They are multiple in 60% of cases, typically in the setting of tuberous sclerosis. On contrast-enhanced CT, they are most often hypodense masses with little contrast enhancement.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • "Cardiac angiosarcomas are the most common pri- mary cardiac malignancy in adults with specific differentiation, accounting for 40% of cardiac sar- comas. Patients are usually younger than 65 years, peaking in the fourth to fifth decade, with a slight male preponderance of 1.3:1.  Cardiac angiosarcomas occur almost exclusively in the right atrium near the atrioventricular sulcus (80%–90% of cases). Symptoms generally result from obstruction, tumor emboli, or local invasion into the myocardium and atrial free wall.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • "These tumors are prone to local and distant metastases with the lung being the most common site. Additional areas reported include the liver, mediastinal lymph nodes, bone, adrenal glands, and spleen. Patients undergoing surgical resection interestingly demonstrate a propensity for brain metastases, thought to be due to intravascular dissemination at tumor resection and manipulation.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • "Cardiac angiosarcomas occur in the right atrium 80% to 90% of the time. At surgical resection, the mass typically projects into the cardiac chambers with permeative growth into the myocardium and local invasion of the pericardium, vena cava, tricuspid valve, and even the coronary arteries. Gross pathology reveals a large lobulated mass that is dark red and brown in color, reflecting its hemorrhagic and necrotic components. If pericardial invasion is present, a thickened rind of gray- black tissue is observed that is inseparable from the remainder of the tumor.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • "Most of the primary malignant cardiac tumors are sarcomas, accounting for greater than 90% of  cases. Undifferentiated high-grade pleomorphic sarcoma (UHGPS), rhabdomyosarcoma (RS), os- teosarcoma (OS), and leiomyosarcoma (LS) are discussed further. The most common of these tumors is UHGPS, previously known as malignant fibrous histiocytoma. UHGPS has a slight female predominance with a mean age of 47 years with a wide age range. RS, on the other hand, is the most common primary cardiac malignancy of childhood.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • "Primary cardiac lymphoma (PCL) is a rare extranodal lymphoma accounting for 1% to 1.5% of all primary cardiac tumors, with the majority involving the right heart. PCLs are aggressive and are usually of the non-Hodgkin type. A typical patient is an immunocompetent male adult (2:1 male to fe- male ratio) in his 6th to 7th decade of life. The mean age is 60 years with a range of 12 to 86 years.44 Certain subtypes occur more commonly in the immunocompromised. Post- transplant lymphoproliferative disorder, a B-cell proliferation related to Epstein-Barr virus infection, may develop mostly in lung and cardiac transplant recipients. Primary effusion lymphoma (PEL) uniquely affects patients with human immunodeficiency virus, which is associated with human herpesvirus-8 (HHV-8)/Kaposi sarcoma– associated herpesvirus.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • “PCL commonly presents as a homogenously low- attenuating, hypoenhancing mass on contrast- enhanced CT, involving the right atrium or right ventricle in 92% of cases. Necrosis and involvement of the cardiac valves are atypical and, if pre- sent, should invoke an alternative diagnosis such as angiosarcoma. Extension along the epicardial surface with encasement of the coronary arteries, aortic root, and great vessels is classic. There is often pericardial thickening and massive pericardial effusion, which can occasionally be the only imaging manifestation, particularly in the PEL subtype.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • "When confronted with a cardiac mass, the most important imaging considerations are the location of the tumor, the possibility of metastatic disease, and the clinical presentation. When considering the differential diagnosis for primary malignant cardiac masses, location is usually the most helpful feature. Myxomas tend to be left sided, and their overall frequency skews left-sided heart masses as more frequently benign. Cardiac lymphoma and angiosarcoma, the most common primary cardiac malignancies are predominantly right sided. Necrosis, surface enhancement (“sun-ray” appearance), and valvular involvement favor angiosarcoma, whereas homogeneity and vascular encasement favor lymphoma. Other cardiac sarcomas tend to be left sided, specifically arising from the posterior wall of the left atrium.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • “Primary cardiac neoplasms occur at an incidence of 30 per 100,000 people per year. Approximately 80% of primary cardiac tumors are benign. The World Health Organization has classified neoplasms of the heart into either benign tumors and tumorlike lesions such as myxoma, malignant lesions such as angiosarcoma, and pericardial tumors such as solitary fibrous tumors. In patients older than 16 years, the most common primary cardiac neoplasms are myxomas, lipomatous tumors, and papillary fibroelastomas. In patients younger than 16 years, the most common tumors are rhabdomyomas, teratomas, fibromas, and myxomas.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • "The most common presenting symptom of a cardiac tumor is dyspnea, but the manifestations of a neoplasm will depend on the location of the lesion and size. Even benign cardiac tumors may cause obstruction of blood flow, decreased cardiac output, arrhythmia, or heart failure, which can be fatal. In addition, systemic manifestations such as fatigue, anorexia, and fever may also be seen.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • "Cardiac myxoma is the most common benign primary cardiac neoplasm, accounting for up to 80% of all cases,5 but only represent 10% of benign primary cardiac tumors in children. Approximately 3% to 10% of cardiac myxomas are associated with Carney complex,6 an autosomal dominant disorder characterized by pigmented lesions of the skin and mucosae, cardiac myxomas, cutaneous tumors, and multiple other endocrine and nonendocrine neoplasms.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • "Although they can be located in any chamber, most myxomas are located within the left atrium and originate at the interatrial septum. These lesions may have an irregular border, a pedunculated morphology, and be mobile. The size of the tumor is related to the degree of mobility and the potential that the lesion can obstruct the atrio- ventricular valve.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • "Although computed tomography (CT) is not the preferred method to characterize the tumor, typical findings on a contrast-enhanced CT include a spherical or ovoid mass that is lower in attenuation than surrounding myocardium.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • “Rhabdomyoma is the most common primary cardiac tumor of infancy and childhood representing 60% of pediatric primary cardiac neoplasms.These lesions are most often diagnosed during the first year of life or prenatally. Cardiac rhabdomyomas are associated with tuberous sclerosis in 30% to 50% of cases but also occur sporadically and rarely in association with congenital heart disease. Tuberous sclerosis is char- acterized by cortical tubers and subependymal nodules within the brain, multiple retinal hamarto- mas, adenoma sebaceum of the skin, and periungual fibromas.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • “Rhabdomyomas are most commonly located within the ventricles attached to the myocardium but are less commonly located in the atrioventricular groove. They are multiple in 60% of cases, typically in the setting of tuberous sclerosis. On contrast-enhanced CT, they are most often hypodense masses with little contrast enhancement.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • "Cardiac angiosarcomas are the most common pri- mary cardiac malignancy in adults with specific differentiation, accounting for 40% of cardiac sar- comas. Patients are usually younger than 65 years, peaking in the fourth to fifth decade, with a slight male preponderance of 1.3:1.  Cardiac angiosarcomas occur almost exclusively in the right atrium near the atrioventricular sulcus (80%–90% of cases). Symptoms generally result from obstruction, tumor emboli, or local invasion into the myocardium and atrial free wall.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • "These tumors are prone to local and distant metastases with the lung being the most common site. Additional areas reported include the liver, mediastinal lymph nodes, bone, adrenal glands, and spleen. Patients undergoing surgical resection interestingly demonstrate a propensity for brain metastases, thought to be due to intravascular dissemination at tumor resection and manipulation.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • "Cardiac angiosarcomas occur in the right atrium 80% to 90% of the time. At surgical resection, the mass typically projects into the cardiac chambers with permeative growth into the myocardium and local invasion of the pericardium, vena cava, tricuspid valve, and even the coronary arteries. Gross pathology reveals a large lobulated mass that is dark red and brown in color, reflecting its hemorrhagic and necrotic components. If pericardial invasion is present, a thickened rind of gray- black tissue is observed that is inseparable from the remainder of the tumor.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • "Most of the primary malignant cardiac tumors are sarcomas, accounting for greater than 90% of  cases. Undifferentiated high-grade pleomorphic sarcoma (UHGPS), rhabdomyosarcoma (RS), os- teosarcoma (OS), and leiomyosarcoma (LS) are discussed further. The most common of these tumors is UHGPS, previously known as malignant fibrous histiocytoma. UHGPS has a slight female predominance with a mean age of 47 years with a wide age range. RS, on the other hand, is the most common primary cardiac malignancy of childhood.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • "Primary cardiac lymphoma (PCL) is a rare extranodal lymphoma accounting for 1% to 1.5% of all primary cardiac tumors, with the majority involving the right heart. PCLs are aggressive and are usually of the non-Hodgkin type. A typical patient is an immunocompetent male adult (2:1 male to fe- male ratio) in his 6th to 7th decade of life. The mean age is 60 years with a range of 12 to 86 years.44 Certain subtypes occur more commonly in the immunocompromised. Post- transplant lymphoproliferative disorder, a B-cell proliferation related to Epstein-Barr virus infection, may develop mostly in lung and cardiac transplant recipients. Primary effusion lymphoma (PEL) uniquely affects patients with human immunodeficiency virus, which is associated with human herpesvirus-8 (HHV-8)/Kaposi sarcoma– associated herpesvirus.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • “PCL commonly presents as a homogenously low- attenuating, hypoenhancing mass on contrast- enhanced CT, involving the right atrium or right ventricle in 92% of cases. Necrosis and involvement of the cardiac valves are atypical and, if pre- sent, should invoke an alternative diagnosis such as angiosarcoma. Extension along the epicardial surface with encasement of the coronary arteries, aortic root, and great vessels is classic. There is often pericardial thickening and massive pericardial effusion, which can occasionally be the only imaging manifestation, particularly in the PEL subtype.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • "When confronted with a cardiac mass, the most important imaging considerations are the location of the tumor, the possibility of metastatic disease, and the clinical presentation. When considering the differential diagnosis for primary malignant cardiac masses, location is usually the most helpful feature. Myxomas tend to be left sided, and their overall frequency skews left-sided heart masses as more frequently benign. Cardiac lymphoma and angiosarcoma, the most common primary cardiac malignancies are predominantly right sided. Necrosis, surface enhancement (“sun-ray” appearance), and valvular involvement favor angiosarcoma, whereas homogeneity and vascular encasement favor lymphoma. Other cardiac sarcomas tend to be left sided, specifically arising from the posterior wall of the left atrium.”
    Cardiac Neoplasms Radiologic-Pathologic Correlation  
    John P. Lichtenberger III et al.
    Radiol Clin N Am 59 (2021) 231–242 
  • “Secondary cardiac tumors (metastases) are 30–40 times more common than primary tumors and can spread to the heart by hematogenous dissemination, lymphatic spread, venous extension, or direct invasion. Melanoma and thoracic neoplasms such as lung, breast, and esophageal carcinomas are the most frequently implicated primaries.”
    The Assessment of Cardiac Masses by Cardiac CT and CMR Including Pre-op 3D Reconstruction and Planning
    Stephen Liddy et al.
    Current Cardiology Reports (2019) 21:103
  • Benign and malignant masses by location, with characteristic imaging features
  • “The typical appearance is of a polypoid intracavitary mass within the left atrium, attached to the fossa ovalis by a stalk. On non-contrast-enhanced CT, myxomas appear as smooth, lobulated, hypodense masses. Calcification may be present but is more commonly seen when located in the right atrium. On contrast- enhanced CT, myxomas appear as intracavitary filling defects. Necrosis or hemorrhage may give rise to a het- erogenous enhancement pattern following the administration of contrast. Myxomas may be detected incidentally at CT and can often diagnosed with confidence without the need for CMR due to their typical appearance. The high spatial resolution of CT enables precise location of the size and insertion of the stalk.”
    The Assessment of Cardiac Masses by Cardiac CT and CMR Including Pre-op 3D Reconstruction and Planning
    Stephen Liddy et al.
    Current Cardiology Reports (2019) 21:103
  • Fibromas are the second most common primary cardiac neoplasm of childhood presenting at a mean age of 11 years . Like rhabdomyomas, they also arise in an intramural location, typically within the left ventricle or interventricular septum. However, in contrast to rhabdomyomas, fibromas are usually solitary. Histologically, they are composed of neoplas- tic fibroblasts interspersed with large amounts of collagen. Associations include familial adenomatous polyposis, Gardner syndrome, and several developmental anomalies including cleft lip and cleft palate.
    The Assessment of Cardiac Masses by Cardiac CT and CMR Including Pre-op 3D Reconstruction and Planning
    Stephen Liddy et al.
    Current Cardiology Reports (2019) 21:103
  • “Cardiac hemangiomas, also known as cardiac venous malformations, are rare lesions accounting for 5–10% of primary cardiac tumors. They consist of endothelial-lined channels and may be divided into capillary, cavernous, or arteriovenous subtypes. They are usually solitary lesions and are typically found in the ventricles, although they may arise in any cardiac chamber.”
    The Assessment of Cardiac Masses by Cardiac CT and CMR Including Pre-op 3D Reconstruction and Planning
    Stephen Liddy et al.
    Current Cardiology Reports (2019) 21:103
  • ”Rhabdomyosarcomas represent 4–7% of cardiac sarcomas and are the most common cardiac malignancy in infants and children. Unlike undifferentiated sarcomas and angiosarcomas, rhabdomyosarcomas demonstrate no predi- lection for any specific chamber. Similar to rhabdomyomas, multiple sites of origin are not uncommon . On CT, rhabdomyosarcomas appear as large infiltrative masses that may surround central areas of necrosis. On CMR, they appear isointense to myocardium on T1-weighted images and hyper intense on T2-weighted images. Enhancement is typically homogenous, although central areas of necrosis may give rise to central areas hypoenhancement.”
    The Assessment of Cardiac Masses by Cardiac CT and CMR Including Pre-op 3D Reconstruction and Planning
    Stephen Liddy et al.
    Current Cardiology Reports (2019) 21:103
  • “Primary cardiac lymphoma usually arises in the right side of the heart, most commonly in the right atrium, and often infiltrates the pericardium resulting in a pericardial effusion. Extensive nodular infiltration of the myocardium is also often present at the time of diagnosis. Epicardial extension along the surface of the heart and encasement of the aortic root and coronary arteries have been described.”
    The Assessment of Cardiac Masses by Cardiac CT and CMR Including Pre-op 3D Reconstruction and Planning
    Stephen Liddy et al.
    Current Cardiology Reports (2019) 21:103
  • “Pericardial metastases may present as pericardial thickening, nodularity, or a pericardial effusion. Malignant pericardial effusions are typically exudative and often hemorrhagic, and thus appear denser on CT and more hyperintense on T1- weighted CMR images compared with simple transudative effusions.”
    The Assessment of Cardiac Masses by Cardiac CT and CMR Including Pre-op 3D Reconstruction and Planning
    Stephen Liddy et al.
    Current Cardiology Reports (2019) 21:103
  • “Four routes of spread are described: hematogenous dissemination, lymphatic spread, venous extension and direct invasion. Hematogenous dissemination usually results in myocardial deposits and is typically seen with melanoma, lymphoma, or leukemia. Lymphatic spread is usually the result of retrograde propagation through the mediastinal lymphatics and results in cancerous cells implanting on the epicardium or pericardium. Thoracic malignancies, in particular lung and breast carcinomas, account for the majority of such cases.”
    The Assessment of Cardiac Masses by Cardiac CT and CMR Including Pre-op 3D Reconstruction and Planning
    Stephen Liddy et al.
    Current Cardiology Reports (2019) 21:103
  • Four routes of spread are described: hematogenous dissem- ination, lymphatic spread, venous extension and direct invasion. Hematogenous dissemination usually results in myocardial deposits and is typically seen with melanoma, lymphoma, or leukemia. Lymphatic spread is usually the result of retro- grade propagation through the mediastinal lymphatics and results in cancerous cells implanting on the epicardium or pericardium. Thoracic malignancies, in particular lung and breast carcinomas, account for the majority of such cases. Venous extension describes tumors propagating along the veins leading to the heart. Infradiaphragmatic tumors may ascend along the IVC and involve the right atrium. Renal cell carcinoma is the typical example, but hepatocellular and adrenocortical carcinoma may also spread via this route. Lastly, direct extension is usually seen in thoracic malignan- cies due to their proximity to the heart and is most commonly the result of lung, breast or esophageal carcinoma or, less commonly, pleural mesothelioma.
    The Assessment of Cardiac Masses by Cardiac CT and CMR Including Pre-op 3D Reconstruction and Planning
    Stephen Liddy et al.
    Current Cardiology Reports (2019) 21:103
  • “Venous extension describes tumors propagating along the veins leading to the heart. Infradiaphragmatic tumors may ascend along the IVC and involve the right atrium. Renal cell carcinoma is the typical example, but hepatocellular and adrenocortical carcinoma may also spread via this route. Lastly, direct extension is usually seen in thoracic malignancies due to their proximity to the heart and is most commonly the result of lung, breast or esophageal carcinoma or, less commonly, pleural mesothelioma.”
    The Assessment of Cardiac Masses by Cardiac CT and CMR Including Pre-op 3D Reconstruction and Planning
    Stephen Liddy et al.
    Current Cardiology Reports (2019) 21:103
  • “Pericardial cysts are benign, congenital, fluid-filled lesions, most commonly located in the right cardiophrenic angle. Their typical location helps distinguish them from other cystic mediastinal abnormalities such as bronchogenic, thy- mic, and foregut duplication cysts. They are almost always asymptomatic but rare cases have been reported of large cysts causing hemodynamic compromise, or hemorrhage into a cyst leading it to exert mass effect. On CT, pericardial cysts appear as thin-walled, fluid- density structures which do not communicate with the peri- cardial space (unlike pericardial diverticulae) and do not enhance post-contrast.”
    The Assessment of Cardiac Masses by Cardiac CT and CMR Including Pre-op 3D Reconstruction and Planning
    Stephen Liddy et al.
    Current Cardiology Reports (2019) 21:103
  • “Advances in multidetector CT hardware and more sophisticated post-processing algorithms have made 3D reconstructions a useful adjunct in the assessment of a cardiac mass [82]. Both maximum intensity projection (MIP) and volume rendering (VR) can be used for reconstructing CT datasets and can assist in displaying information that can be difficult to appreciate on two-dimensional (2D) images.”
    The Assessment of Cardiac Masses by Cardiac CT and CMR Including Pre-op 3D Reconstruction and Planning
    Stephen Liddy et al.
    Current Cardiology Reports (2019) 21:103
  • “MIP images are reconstructed by selecting the highest at- tenuation voxels in a given volume and displaying them as a single 2D image. MIP images are optimized for evaluating vascular anatomy and can be of value in assessing the course and locations of the coronary arteries and their relationship to the mass. Volume rendering employs a more complex algorithm and allows for better visualization of soft tissues, bony structures, and vasculature in a single image. In VR, each voxel is assigned a color and transparency based on its attenuation and the information is displayed as a single image which can be manipulated to better understand the anatomic and spatial relationships between the margins of the mass and the surrounding structures.”
    The Assessment of Cardiac Masses by Cardiac CT and CMR Including Pre-op 3D Reconstruction and Planning
    Stephen Liddy et al.
    Current Cardiology Reports (2019) 21:103
  • “Any tumor with metastatic potential can spread to the heart and can involve any of its structures, the pericardium, or the proximal great vessels. Among all patients with cardiac metastasis, the most common primary tumors are lung cancer, breast cancer, and hematologic malignancies such as non-Hodgkin lymphoma. This is largely influenced by the prevalence of these cancers in the population and, in the case of lung cancer, proximity to the heart.”

    
Metastasis to the Heart:A Radiologic Approach to Diagnosis With Pathologic Correlation 
Lichtenberger JP et al.
AJR 2016; 207:764–772 

  • “When the incidence of cardiac metastasis for a specific primary tumor is considered, those neoplasms with a high rate of metastasis to the heart are melanoma, malignant pleural mesothelioma, and mediastinal malignancies such as thymoma.” 


    Metastasis to the Heart: A Radiologic Approach to Diagnosis With Pathologic Correlation 
Lichtenberger JP et al.
AJR 2016; 207:764–772 

  • “Most patients with metastatic disease to the heart and pericardium are asymptomatic. When present, symptoms depend on the size and location of the metastasis. The most common symptoms attributable to cardiac metastasis are nonspecific and mimic other cardiac diseases such as myocardial ischemia, heart failure, or cardiac injury related to chemotherapy or radiation therapy. These symptoms include dyspnea, chest pain, palpitations, lower extremity edema, and new-onset atrial fibrillation.”

    
Metastasis to the Heart: A Radiologic Approach to Diagnosis With Pathologic Correlation 
Lichtenberger JP et al.
AJR 2016; 207:764–772 

  • “Direct invasion of the heart is usually attributable to aggressive thoracic malignancies such as lung cancer, lymphoma, invasive thymoma, and malignant pleural mesothelioma. By definition, the parietal pericardium is the first site involved by direct invasion. Neoplasms may invade through both layers of the pericardium and well into the myocardium. Tumors invading the atrium may be resected for palliation, but direct invasion is a poor prognostic finding and recurrence is common when the endocardium has been seeded.” 


    Metastasis to the Heart: A Radiologic Approach to Diagnosis With Pathologic Correlation 
Lichtenberger JP et al.
AJR 2016; 207:764–772 

  • “The most specific indicator of cardiac metastasis is an intracardiac or pericardial mass. Most cardiac metastases involve the right heart, and the atria are most frequently involved in patients who undergo imaging evaluation or autopsy. The apparent predilection for right heart and atria involvement may reflect the transvenous route of spread of common infradiaphragmatic tumors and the selection bias of patients who survive to undergo imaging.” 


    Metastasis to the Heart: A Radiologic Approach to Diagnosis With Pathologic Correlation 
Lichtenberger JP et al.
AJR 2016; 207:764–772
  • “Primary malignancies of the heart are rare and include sarcomas, such as angiosarcoma and rhabdomyosarcoma, and primary cardiac 
lymphoma. Similar to cardiac metastasis, they have been reported most frequently in the right heart and atria. The characteristic appearance of the most common primary cardiac malig- nancy—angiosarcoma—is an enhancing right atrial mass centered on the anterior atrioven- tricular groove.” 


    Metastasis to the Heart: A Radiologic Approach to Diagnosis With Pathologic Correlation 
Lichtenberger JP et al.
AJR 2016; 207:764–772
  • “Extracardiac findings should also be con- sidered when determining the significance of cardiac findings suspicious for metastasis. Many of these findings are nonspecific and common in patients with an underlying ma- lignancy, but they may support a unifying di- agnosis of cardiac metastasis. Pleural effu- sions and mediastinal lymphadenopathy are present in 50% and 80% of patients with doc- umented cardiac metastasis, respectively.” 


    Metastasis to the Heart: A Radiologic Approach to Diagnosis With Pathologic Correlation 
Lichtenberger JP et al.
AJR 2016; 207:764–772
  • Metastasis to the heart is not as infrequent as one might suspect. Although primary cardiac tumors are rare (generally between 0.01% and 0.1% on postmortem analysis), the frequency of secondary metastatic tumors to the pericardium, myocardium, great vessels, or coronary arteries is between 0.7% and 3.5% at autopsy in the general population and up to 9.1% in patients with known malignancies. Moreover, the risk of cardiac metastasis rises with metastatic disease burden; 14.2% of patients with multiple distant metastases were found to have cardiac involvement. The incidence of cardiac metastases has increased over the last 30 years, perhaps attributable to increased life expectancy in oncologic patients benefitting from advances in cancer diagnosis and management.

    Tumors Metastatic to the Heart Goldberg AT et al Circulation. 2013; 128: 1790-1794
  • “Primary lung cancer represents 36% to 39% of cardiac metastases, followed by breast cancer (10%–12%) and hematologic malignancies (10%–21%).These numbers reflect the high prevalence of these tumors in the general population and their aggressive nature; in contrast, prostate cancer, although more prevalent in men than any of the above tumors, rarely metastasizes to the heart. Pleural mesothelioma and melanoma have an unusual proclivity to involve the heart, with estimates of 28% to 56% of patients with metastatic melanoma having some cardiac involvement.”

    Tumors Metastatic to the Heart Goldberg AT et al Circulation. 2013; 128: 1790-1794
  • “The clinical manifestations of cardiac metastases are nonspecific and depend on their location and tumor burden. Most cardiac metastases are clinically silent and are diagnosed only postmortem. When cardiac metastases manifest clinically, they can be difficult to distinguish from other causes of cardiovascular disease, with the most common symptoms and signs including dyspnea, palpitations, atrial flutter or fibrillation, lower-extremity edema, and chest pain. Cardiac metastasis may also present with dramatic, life-threatening manifestations, including cardiac tamponade from pericardial involvement.”

    Tumors Metastatic to the Heart Goldberg AT et al Circulation. 2013; 128: 1790-1794
  • “Epicardial involvement (25%–34%) and myocardial involvement (29%–32%) represent the second and third most common sites of cardiac metastasis. Depending on their location, epicardial or myocardial metastases may result in a variety of life-threatening complications. Disruption of the cardiac conduction system by cardiac metastases can lead to lethal arrhythmias, including atrial fibrillation with rapid ventricular response, complete atrioventricular block, or ventricular fibrillation.”

    Tumors Metastatic to the Heart Goldberg AT et al Circulation. 2013; 128: 1790-1794
  • Papillary Fibroelastoma: Facts
    • most common benign neoplasms of the cardiac valvular structures
    • 2nd most common type of cardiac tumor, surpassed only by myxomas, which arise 6 times more often
    • Surgical resection should be offered to all patients who have symptoms and to asymptomatic patients who have pedunculated lesions or tumors larger than 1 cm in diameter.
  • “Cardiac papillary fibroelastomas are classified as primary benign endocardial tumours arising from the normal component of the endocardium like fibrous tissue, elastic fibers or smooth muscle cells. Characteristically they have a short pedicle and multiple papillary fronds similar to a sea anemone . They often (85%) originate from the valvular endocardium. The aortic valve (29%), mitral valve (25%), tricuspid valve (17%) and pulmonary valves (13%) are involved in that order.”


    Papillary fibroelastoma of the aortic valve - a case report and literature review
Jha NK et al.
Journal of Cardiothoracic Surgery 2010, 5:84
  • “In addition, non-valvular origin was observed in approximately 16% cases that included left and right ventricular septal and mural endocardial surfaces, atrial endocardium, papillary muscles, chordae tendinae or intima of the right coronary ostium.”


    Papillary fibroelastoma of the aortic valve - a case report and literature review
Jha NK et al.
Journal of Cardiothoracic Surgery 2010, 5:84
  • “Despite the benign nature of this tumour, it carries very high risk of embolic complications including neurological deficit. The fragile nature and frond-like papillary tissues of the tumour itself is prone to thromboembolism”
Papillary fibroelastoma of the aortic valve - a case report and literature review
Jha NK et al.
Journal of Cardiothoracic Surgery 2010, 5:84
  • “Clinical presentation of PFE's varies from asymptomatic to severe thromboembolic complications, myocardial ischemia, infarction and stroke. However, pulmonary embolism, congestive heart failure, near-syncope, ventricular fibrillation and sudden death have also been reported . Embolisation may occur from fragments of the fronds of the tumour or from a thrombus that frequently forms on the tumour 'nidus' due to platelet or fibrin aggregates.”

    Papillary fibroelastoma of the aortic valve - a case report and literature review
Jha NK et al.
Journal of Cardiothoracic Surgery 2010, 5:84
  • Approach to Differential Diagnosis of Cardiac Masses
    - Is there a cardiac mass-
    - Anatomic location-
    - Intracavitary mass
    - Mass extension along great vessels
    - Pericardial mass
    - Valvular mass
    - Specific tissue characteristics-
    - Relevant clinical history-
  • Is there a cardiac mass-
    - True cardiac mass:
    - Well defined filling defect within cardiac chamber
    - Defect persists on delayed or repeat imaging
    - Flow artifacts:
    - Coronal reconstruction may depict admixing of non-opacified blood from IVC
    - Resolves on delayed or repeat imaging
  • Intracavitary Mass Differential Diagnosis
    - Thrombus
    - Primary benign neoplasms
    - Myxoma, lipoma, rhabdomyoma, fibroma
    - Primary malignant neoplasms
    - Angiosarcoma, other sarcomas, lymphoma
    - Metastatic neoplasms
  • Thrombus
    - Most common cardiac mass
    - Hypodense filling defects within cardiac chambers
    - Absence of delayed enhancement
    - Differentiates thrombus from myxoma
    - Predisposing risk factors:
    - Myocardial infarction with scar or aneurysm
    - Arrhythmia
    - Indwelling catheter
    - Valvular disease
    - Hypercoagulable state
  • Myxoma
    - Most common benign tumor of the heart, accounting for ~50% of primary cardiac tumors
    - Distribution: 80% left atrium, 10-15% right atrium, rare cases in ventricles
    - Mean age at diagnosis 50, F > M
    - Clinical presentation:
    - Asymptomatic
    - Mitral valve obstruction, systemic embolization
    - Constitutional symptoms (inflammatory mediators)
    - Association with Carney complex
  • Myxoma
    - Typically originate from region of the fossa ovalis
    - Polypoid mass with lobulated margins
    - May contain focal calcifications, hemorrhage, necrosis, or cyst formation
  • Primary Malignant Cardiac Neoplasms
    - Rare entities, representing 25% of all primary cardiac tumors
    - Angiosarcoma is most common primary malignant neoplasm
    - Also includes other sarcomas with different mesenchymal differentiation
    - CT features suggestive of malignant neoplasm:
    - Large irregular hypodense intracavitary lesion
    - Infiltration along myocardium, pericardium, valves, and great vessels
  • Angiosarcoma
    - Usually middle aged, M > F
    - Usually arise from right atrium or pericardium
    - May also arise from the pulmonary artery
    - Clinical presentation:
    - Valvular obstruction
    - Right sided heart failure
    - Pericardial tamponade with hemorrhagic fluid
    - Metastatic disease 66-89% involving lungs, liver, cerebellum, skin, and bone
  • Intracavitary Cardiac Metastases
    - Hematogenous metastases
    - Via coronary arteries
    - Implantation of cancer fragments carried through the vena cava
    - Most common primary neoplasms: Melanoma and sarcomas
    - Imaging features:
    - Can involve any cardiac chamber
    - Multiple well-defined masses or diffuse myocardial infiltration
    - Usually accompanied by evidence of hematogenous metastases to other organs
  • “ The objectives of this article are to review the CT appearance of cardiac masses and the role of volumetric MDCT with 3D rendering to define characteristic features and provide a practical approach to formulating a differential diagnosis.”
    Multidetector CT of the Heart: Spectrum of Benign and Malignant Cardiac Tumors
    Chu LC, Johnson PT, Halushka MK, Fishman EK
    Emerg Radiol (2012) 19;415-428
  • “ Cardiovascular complications in patients with HIV infection include myocardial, endocardial, pericardial and vascular diseases. The complications are caused by the HIV infection itself, opportunistic infections, HIV related tumors and the side effects of highly active antiretroviral therapy.”
    HIV-Related Cardiac Complications: CT and MRI Findings
    Nakazono T et al.
    AJR 2012; 198:364-369
  • Cardiac Complications in HIV Patients
    - Dilated cardiomyopathy
    - Myocarditis
    - Coronary artery disease
    - Endocarditis
    - Cardiac tumors
    - Pulmonary arterial hypertension
    - Vasculitis
  • Cardiac Tumors in HIV Patients
    1. Kaposi’s sarcoma
    - Most common tumor
    - Usually involves the epicardium and the pericardium
    2. Lymphoma (usually B-cell lymphomas)
    - Second most common tumor
    - Usually involve the right atrium
    - Pericardial effusion and pericardial involvement are common
  • “ MDCT is a useful alternative to MRO in the evaluation of cardiac and paracardiac masses, particularly in those patients with contraindications to MRI. MDCT has high spatial and temporal resolutions and is sensitive for the presence of calcification and fat.”
    Computed Tomography of Cardiac and Pericardiac Masses
    Rajiah P et al.
    J Cardiovasc Computed Tomogr (2011) 5, 16-29
  • Primary Cardiac Neoplasms: Facts
    - Prevalence of 0.002%-0.3% at autopsy and 0.15% in echocardiography series
    - Metastases are 20-40 times as common as primary cardiac neoplasms
    - In an AFIP series of primary tumors and cysts of the heart and pericardium, 60% were benign tumors, 23% were malignant tumors and 17% were cysts
  • Cardiac Masses: Benign
    - Myxoma
    - Lipoma
    - Papillary fibroelastoma
    - Hemangioma
    - Paraganglioma
    - Rhabdomyoma
    - Fibroma
    - Teratoma
  • Cardiac Masses: Malignant
    - Metastases
    - Lymphoma
    - Sarcoma (spindle cell, angiosarcoma, undifferentiated sarcoma, liposarcoma, MFH)
    - Malignant teratoma
    - Mesothelioma
  • Cardiac Masses: Nontumoral
    - Lipomatous hypertrophy of the interatrial septum
    - Thrombus
    - Pericardial cyst
    - Bronchognic cyst
    - Caseous mairal annuar calcification
    - Hiatal hernia


  • Cardiac Sarcoma: Facts
    -Most common primary cardiac malignancy
    -More common in woman
    -Age is usually 20-50 years of age
    -75% originate in the right atrium
    -Multiple lesions occur in 60% of patients
    -Angiosarcoma is the most common type and rhabdomyosarcoma the second most common type
    -Rhabdomyosarcoma is the most common primary cardiac tumor in children
  • Cardiac Lymphoma: Facts
    -More common in adults
    -More common in HIV patients
    -Usually highly aggressive non-Hodgkin B-cell lymphoma
    -May involve right atrium, pericardium, AV groove
    -Single or multipla masses may be seen
  • Tumor Extension and the Source
    -Direct extension from adjacent tumors- lung, breast, esophageal or other mediastinal tumors
    -Hematogenous spread-lung cancer
    -Transvenous spread-renal, hepatic, lung, adrenal tumors
    -Lymphatic spread-lymphoma, breast cancer, lung cancer
  • Malignant Cardiac Tumors: Facts
    -Cardiac metastases are 20-40 times more common than primary tumors
    -Sites of origin include melanoma, thyroid, lung, sarcoma. Esophagus, kidney and breast malgnancies as well as lymphoma and leukemia
    -Tumors may extend via
    -Direct extension from adjacent tumors
    -Hematogenous spread
    -Transvenous spread
    -Lymphatic spread
  • Fibroelastoma: CT Findings
    -Small homogeneous mass attached to a cardiac valve
    -Differential diagnosis includes vegatation, thrombus or myxoma
    -Most patients are asymptomatic while others may have angina or embolic phenomena
    -Surgical removal is recommended in symptomatic patients and patients with mobile left sided tumors
  • Fibroelastoma: Facts
    -10% of cardiac tumors
    -75% of all valvular neoplasms
    -Occur in middle aged and older adults
    -No sexual preference
    -Arise from valvular endothelium in 90% of cases, most commonly off aortic leaflets
    -Most are solitary in 10-20 mm range but can grow to 70 mm
  • Cardiac Myxoma: Facts
    -Most common benign cardiac neoplasm
    -Comprises 25-50% of all primary cardiac neoplasms
    -Patient age is 30-60 years old
    -Equal frequency in men and woman
    -Most common in left atrium (60-75%), near fossa ovalis
  • Nontumoral Masses:
    -Lipomatous hypertrophy of interatrial septum
    -Thrombus
    -Pericardial cyst
    -Bronchogenic cyst
    -Caseous mitral annular calcification
    -Hiatal hernia
  • Cardiac Tumors: Malignant
    -Metastases
    -Lymphoma
    -Sarcoma
    -Undifferentiated sarcoma
    -Spindle cell sarcoma
    -Angiosarcoma
    -rhabdomyosarcoma
    -Malignant teratoma
    -Mesothelioma
  • Cardiac Tumors: Benign
    -Myxoma
    -Lipoma
    -Papillary fibroelastoma
    -Hemangioma
    -Paraganglioma
    -Rhabdomyoma
    -Fibroma
    -Teratoma
    -Neurofibroma
  • “ MDCT is a useful alternative to MRI in the evaluation of cardiac and paracardiac masses, particularly in those patients with contraindications to MRI. MDCT has high spatial and temporal resolutions and is sensitive for the presence of calcification and fat. MDCT can be used to define the extent of the tumor, distinguish benign from aggressive neoplasms, and assess the effect of masses on cardiac function.”
    Computed tomography of cardiac and pericardiac masses
    Rajiah P et al
    J Cardiovascular Comput Tomogr (2011) 5, 16-29
  • Pericardial Cyst: Facts
    - More common in right cardiophrenic space (77%)
    - Congenital in origin
    - Always asymptomatic
    - Attenuation usually 0-20 HU
    - No enhancement on contrast enhanced studies
  • Lesions of the Cardiophrenic Space: Differential Diagnosis
    - Prominent cardiac fat pad
    - Diaphragmatic hernia (Morgagni hernia)
    - Pericardial fat necrosis
    - Pericardial cysts
    - Thymic tumors
    - Lymphoma
    - Adenopathy
  • Cardiac Lymphoma: Facts

    "Patients with chronic thromboembolic pulmonary hypertension may be asymptomatic for several years before their presentation with symptoms such as recurrent acute or progressive exertional dyspnea, chronic nonproductive cough, atypical chest pain, tachycardia, syncope, and cor pulmonale."

    CT Diagnosis of Chronic Pulmonary Thromboembolism
    Castaner E et al.
    RadioGraphics 2009; 29:31-53

  • Cardiac Lymphoma: Facts
    - Usually related to extensive systemic involvement
    - Primary cardiac lymphoma is rare
    - Primary cardiac lymphoma more common in immunosuppresed patients and favors the right side of the heart
  • Cardiac Angiosarcoma: Facts
    - Most common primary malignant tumor of the heart
    - Arises most commonly in the right atrial free wall
    - Most other primary cardiac tumors arise from the left atrium
© 1999-2022 Elliot K. Fishman, MD, FACR. All rights reserved.