Definition

Cardiac tumors stand for a heterogeneous group, potentially involving any type of of the love structures.

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Secondary malignancies (metastatic) space the many frequent, with a 20 – 40 times higher incidence than main tumors. Main cardiac tumors have actually an incidence the 0.02% in autopsy series, seventy five percent that them gift benign<1>. The frequency and type of cardiac tumors varies amongst age groups and also are various in adults compared to children<2>.

Depending top top location and morphology, cardiac tumors can develop four types of clinical manifestations:

Systemic – constitutional (fever, arthralgias, weight loss, fatigue) and also paraneoplastic syndromes (primary cardiac tumors).Embolic – pulmonary and/or systemic by tumor/thrombo emboli.Cardiac – mass impact interfering v myocardial function, blood flow, electrical impulse formation and also conduction, pericardial liquid dynamics.Secondary to metastatic an illness of a main malignant cardiac tumor – manifestations relying on site that metastasis<3>.

Role the echocardiography (transthoracic and also transesophageal) in the work-up of cardiac tumors:

Diagnosis - usually first line imaging tool. Based upon location, appearance and extension and also correlated with the clinical findings deserve to discriminate in between primary - secondary and light - malignant.Treatment – along with complementary imaging modalities (CT, CMR, PET) offers information ~ above extension and resecability, and also plays a an important role in the follow up process.Newer methods like comparison echocardiography and real – time three dimensional echocardiography have the right to increase diagnostic accuracy by giving information ~ above tumor blood supply, composition and also relationship to nearby structures<4><5>.

Primary cardiac tumors

Benign Cardiac tumorsMyxoma

Figure 1.3D Transesophageal echocardiography mirroring a left atrial myxoma generally inserted in ~ the level the fosa ovalis and also protruding into the left ventricular inflow in diastole.

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Myxomas are the most usual benign cardiac tumors (50%) and usually affect middle-aged women<4>.Most instances are sporadic, through familial myxomas, additionally known together “syndrome myxoma” or “Carney complex”, being rare (10%). This syndrome is characterised by multiple, recurrent cardiac myxomas at younger age, connected with spotty skin pigmentation and endocrine overreactivity<2>.Clinical manifestations of myxomas consist of in a triad: constitution symptoms, embolization and also intracardiac obstruction.Location – left atrium (75%), best atrium (23%), seldom in the ventricles; usually associated to the atrial septum (fosa ovalis) by a small stalk; depiction of website and kind of tumor attachment is necessary when differentiating from thrombus<1>.They have actually variable functions on echocardiography – globular, smooth – surfaced or irregular, friable surface ar with multilobular appearance, 4 to 8 cm in size, heterogeneous echogenicity with locations of echolucency and also sometimes calcifications.Myxomas have actually variable mobility relying on size and also narrow/broad implantation stalk. Usually, they protrude into the ventricles v the mitral or tricuspid valves in diastole. Diastolic filling disability can be assessed by shade Doppler imaging and also further quantified by continuous Doppler map analysis, showing indicators of mitral/tricuspid stenosis<4>.Transesophageal echocardiography may be necessary to far better visualize the implantation site and also the potential extension into the pulmonary or caval veins<1>.Myxomas have actually a poor blood supply, through partial and lower than normal myocardium enhancement on contrast echocardiography<6>.After surgical procedure – echocardiography should document the complete tumor resection and should be performed together follow up, considering the risk of recurrence<4>.Lipoma and also lipomatous hypertrophy that atrial septum

Lipomasare the 2nd most common benign cardiac tumors (10-20%). They stand for encapsulated fatty accumulations, space variable in size and also usually asymptomatic. Sometimes they may reason symptoms concerned compression, arrhythmias and conduction abnormalities<2>.

Location – everywhere in the heart, most commonly in the left ventricle, appropriate atrium, and atrial septum. Lock arise most frequently in the subepicardium and also subendocardium, but intamyocardial lipomas have also been described. Subepicardial lipomas often prosper into the pericardial an are resulting in effusions and also subendocardial lipomas could protrude in the cardiac chambers, interfering through blood flow.On echocardiography castle present as well demarcated, homogeneous, large based masses (not diagnostic). Your appearance varies with location: in the pericardial room they arise indigenous the epicardium and are hypoechogenic, if intracavitary lipomas are hyperechogenic<1><2>.Cardiac CT and MRI room the ideal imaging devices for diagnosing cardiac lipomas together they determine fat with high specificity.Lipomas have actually the propensity to grow and periodic echocardiography must be performed. These tumors have to be operation excised when symptomatic<4>.

Lipomatous hypertrophy that the atrial septumis a benign cardiac mass result from fat infiltration that the atrial septum. Its described incidence ~ above echocardiographic research studies is 8% and, typically, is associated with enhancing age and also obesity.

On echocardiography, the lipomatous hypertrophy the the atrial septum has actually a characteristic appearance with massive, hyperechogenic, thickening that the atrial septum the spares the fossa ovalis, acquisition a “dumbbell” or “hourglass” shape.Differentiation between this light lesion and also other cardiac tumors including the atrial septum, an especially malignant, is an essential as it might avoid unnecessary surgery<7>.Papillary fibroelastoma

Figure 2.Transesophageal echocardiography showing a solitary mitral valve fibroelastoma.

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Papillary fibroelastomas space the most regular tumors including the love valves. They account for about 8% of primary benign cardiac tumors and also are typically encountered in enlarge ages. Because of their embolic potential, papillary fibroelastomas (particularly left-sided) should be thought about for resection also if asymptomatic<3>.Location – aortic and mitral valves, less often, tricuspid and also pulmonary valves or mural endocardium. Different from vegetations, papillary fibroelastomas are located on the downstream side of the valve and also rarely reason valvular dysfunction<4>.Echocardiographic appearance – tiny (Due come the require for differential diagnosis with vegetations, thrombi, valvular calcifications or Lamb’l excrescences, transesophageal echocardiography is regularly required<1>.

Figure 3.3D Transesophageal echocardiography showing two fibroelastomas entailing the aortic (arrow) and mitral (asterix) valves. Keep in mind the unusually huge mitral fibroelastoma originating in the posterior leaflet and also extending right into the subvalvular apparatus.

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RhabdomyomaRhabdomyomas space the most common primary cardiac tumors in infants and also young children, around three thirds emerging in patients less than 1 year old. Over there is a solid association in between cardiac rhabdomyomas and tuberous sclerosis (80%). In an ext than half of the patients they regress in size and number ~ infancy. The existence of rhabdomyomas in adulthood is uncommon<3>.Rhabdomyomas most frequently occur in the right and left ventricular myocardium. ~ above echocardiography they appear as multiple, homogeneous and also hyperechogenic tumors located intramurally or pedunculated, protruding right into the ventricular cavity, and ranging from couple of mm to few cm in size.Depending on your location and also size they might induce arrhythmias or blood circulation obstruction. Symptomatic rhabdomyomas be affected by each other a negative prognosis and also surgery is necessary, even though complete resecability is rarely completed due to your deep intramyocardial location<1>.FibromaFibromas space the 2nd most usual primary cardiac tumors in children. In adult they account for around 3% of every benign cardiac tumors.Fibromas space invariably located in the ventricular myocardium, usually in the left ventricular complimentary wall or interventricular septum. They might mimic hypertrophic cardiomyopathy.Fibromas appear as well circumscribed, very echogenic, solitary tumors (different native rhabdomyomas), often prolonging into the ventricular cavity. Typically, their size varieties from 1 come 10 cm and have central calcifications<2>.In 70% the cases, fibromas reason symptoms through impeding intracavitary blood flow or causing ventricular arrhythmias. Offered the raised risk because that fatal arrhythmias and also sudden death, associated with this kind of tumors, surgical resection is recommended nevertheless symptoms<1>.HemangiomaHemangiomas room benign vascular tumors audit for 2% of major cardiac tumors. Castle can occur at any age and also are occasionally associated with similar tumors in the gastrointestinal tract or skin. Typically, on coronary angiography they produce a “vascular blush”.Hemangiomas can happen in any cardiac chamber, however most often are located in the ventricles, cultivation intramurally or in the direction of the ventricular cavity. Similarly to fibromas, they have the right to mimic hypertrophic cardiomyopathy.Hemangiomas show up as hyperechogenic, fine demarcated masses, varying from 1 come 8 cm in size. In 30% of instances multiple tumors are described.In symptomatic patients radical surgical resection is indicated. Periodic echocardiography is recommended, considering the potential for tumor development or recurrence after surgery<8>.Pericardial cystPericardial cysts room the most typical pericardial tumors. They existing as small, rounded, echolucent masses, most often adjacent to the best atrium. Usually, pericardial cysts are asymptomatic and have great prognosis, however they have the right to grow and also compress the appropriate cardiac chambers or the neighboring mediastinal structures<4>.Malignant tumorsMalignant main cardiac tumors room rare and typically have actually rapid expansion, invading several cardiac frameworks (myocardium, cardiac chambers and also pericardium).When a cardiac tumor with functions of malignancy ~ above echocardiography is depicted, added imaging modalities (CT, CMR, PET) are required to characterize that is intra- and also extracardiac extension.Sarcomas

Figure 4.3D Transgastric check out of the appropriate chambers reflecting a giant sarcoma that obliterates the ideal ventricular inflow and infiltrates the tricuspid valve. Keep in mind the presence of hypoechogenic locations within the fixed (asterix) compatible with intratumoral necrosis. RA, ideal atrium; RVOT, best ventricular outflow tract.

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Sarcomas account because that 95% of primary malignant cardiac tumors. Lock occur in between the 3rd and fifth years of life, same in both genders. Sarcomas space characteristically an extremely aggressive, have a nonspecific clinical presentation and also median survive is much less than 1 year<1>.Based on qualities on echocardiography, it’s not feasible to differentiate in between histological subtypes of sarcomas.There are no conclusive data sustaining that histological subtype is prognostic for therapy outcome. Success of finish resection is the only proven aspect positively affecting outcome<2>.

Angiosarcoma

Angiosarcomas room the most typical histological subtype, typically affecting middle-aged men. This tumors have a predilection because that the appropriate chambers, particularly for the appropriate atrium.Echocardiography shows, a large, large based mass close to the worse vena cava, prolonging intracavitary and also into the pericardium, sometimes invading the caval veins or tricuspid valve<2><5>.

Rhabdomyosarcoma

Rhabdomyosarcomas stand for the most regular cardiac malignancy in infants and children.Rhabdomyosarcomas may take place in any type of cardiac chamber and also in 60% of instances are multiple. Likewise to angiosarcomas, this tumors prosper rapidly and are invasive, often extending to the pericardium prior to diagnosis<3>.

Other cardiac sarcomas

Other nonvascular sarcomas, consisting of fibrosarcoma, liposarcoma, leiomyosarcoma, osteosarcoma, myxosarcoma, synovial sarcomas and undifferentiated sarcoma occur incredibly rare as major malignancies that the heart. They normally arise in the left atrium and likewise to angio- and rhabdomyosarcomas, are very invasive and aggressive.LymphomaPrimary cardiac lymphomas are very rare, representing 5% of major cardiac malignancies. They have increase ubiquity in immunocompromised patients.They are defined as non-Hodgkin’s lymphoma located initially only in the heart or pericardium. Cardiac joining in lymphomas is usually second and occurs by dissemination.Primary cardiac lymphomas involve an ext often the best side of the heart and usually associate through pericardial effusion. Prognosis is negative with much less than 1 month survival without treatment and up to 5 years after radio- and also chemotherapy<1><5>.Pericardial mesotheliomaPrimary malignant pericardial mesothelioma is extremely rare and unlike pleural and peritoneal mesotheliomas has no identify correlation with exposure to asbestos.Echocardiographic findings space pericardial effusion, pericardial thickening or constrictive physiology. Invasion of other cardiac frameworks is rarely seen.It tote a negative prognosis with tiny benefit indigenous radio and also chemo-therapy.

Secondary cardiac tumors

Extension to the heart is excellent by: haematogenous, lymphatic, direct or venous spread.Except for melanoma (very rare) that metastasis preferentially to the heart, cardiac metastasis are found in lung, breast, ovarian, kidney, leukemia, lymphoma, esophageal cancer patient in widespread dissemination stages.Echocardiography should be performed in all patients with a history of malignancy and cardiac symptoms.Pelvic leiomyoma (benign), renal cell carcinoma, hepatoma and uterine leiomyosarcoma (malignant) can prolong to the ideal cavities v inferior vena cava. This is best visualised in the subcostal view.Cardiac metastases room predominantly situated in the pericardium, with pericardial effusion being the most frequent finding.Chemotherapy and tumor resection may alleviate symptoms and prolong survival<1><4>.

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Further reading

Pepi M, Evangelista A, Nihoyannopoulos p et al. On instead of of the europe Association of Echocardiography referrals for echocardiography usage in the diagnosis and also management that cardiac resources of embolism 2010;11:461-476.Galiuto L, Badano L, Fox K, Sicari R, Zamorano JL. The EAE Textbook of Echocardiography. 1st edition. Oxford, Oxford college Press;2011Armstrong WF, Ryan T. Feigenbaum"s Echocardiography. 7th edition. Philadelphia, PA:Lippincott Williams and Wilkins;2009.Bogaert J, Dymarkovski S, Taylor A, Muthurangu V. Clinical Cardiac MRI. Second edition. Berlin, Springer;2012.