Presentation on theme: "Cardiac tumors Dr Shreetal Rajan Nair"— Presentation transcript:
1Cardiac tumors Dr Shreetal Rajan Nair Senior Resident, Department of Cardiology,Calicut Medical College72 slides
2History 1559 : Columbus , a noted pathologist – first to report 1934 : first report of a primary sarcoma of the heart diagnosed in a living patient by Barnes1942 : surgical resection of intrapericardial teratoma by Beck1951 : The first successful excision of a cardiac tumor performed by Maurer1952 : Goldberg diagnosed intracavitary myxoma by angiocardiography1968 : Shattenberg used echo to diagnose cardiac tumors
4Incidence All age groups - 0.002% to 0.03% Pediatric - 0.027% to 0.08% Adults upto 0.02%By comparison, metastatic involvement is 20 times more common
5Incidence of benign tumors Shapiro LM. Cardiac tumours: diagnosis and management. Heart2001;85:218e22.Shapiro LM. Cardiac tumours: diagnosis and management. Heart 2001; 85:
6Tumors of the heart - Constitutional or systemic Embolic Cardiac Phenomena secondary to metastatic diseases.Specific signs and symptoms generally are determined by the location of the tumor, size, friability , mobility in the heart and depends least on the histopathology
7Tumors of the heart- clinical presentations Primary tumors most commonly involve the endocardium, followed by myocardium and then epicardiumMetastatic involvement of heart is reverse that of primary tumorsEndocardium- obstruction to flow of blood through heartMyocardium - rhythm abnormalitiesEpicardium – pericardial effusion, cardiac tamponade
8Approach to a cardiac tumor PUOIE/CONNECTIVE TISSUE DISEASESVALVULAR LESIONSCCFCONDUCTION ABNORMALITIESEMBOLISMSYNCOPEPERICARDIAL EFFUSION
10Diagnostic evaluation Confirm the diagnosislocate the lesion within the heartWhether benign or malignant
11Echocardiography Simple, noninvasive technique for initial evaluation. Images myocardium and the cardiac chambers Identify the presence of a mass and its mobility.Information about any obstruction to the circulation & whether the tumor could be a source of emboli
12Echocardiography - TEE vs TTE Transthoracic echocardiography – the initial diagnostic toolTransesophageal echocardiography (TEE)-more informative.This is due to the proximity of the esophagus to the heart, the lack of intervening lung and bone, and the ability to use high-frequency imaging transducers that afford superior spatial resolution
13CARDIAC MRI VS ULTRA FAST CT Both provide noninvasive, high resolution images of the heart, but MRI generally is preferred.cMRI -detailed anatomic imagesT1- and T2-weighted sequences help to identify the chemical microenvironment within a tumorCT -useful when calcification is present and MRI is not available or is contraindicated.
14CORONARY ANGIOGRAPHY Mapping the blood supply of tumors arising from the epicardial surfacesvital to the success of excising such tumors.Significant involvement of coronary arteries - resection and grafting of such arteries
15Role of Transvenous biopsy Limited data are available on the risks and benefitsconsidered reasonable if potential benefits outweigh risks and there is diagnostic dilemma
16PET SCAN Identifying cardiac involvement in patients with -metastatic tumors-atrial myxoma- lipomatous septal hypertrophy
17Benign tumors Around 75 percent of primary cardiac tumors are benign In adults, most common- myxomas (50%)Other common benign lesions-papillary fibroelastomas and lipomas.In children, the most common-rhabdomyomas and fibromas
18Myxomas Most common primary cardiac neoplasm. Histology- scattered cells within a mucopolysaccharide stroma which originate from multipotent mesenchymal cells capable of neural and endothelial differentiation .Hallmark histological feature is myxoma (lepidic) cellProduce VEGF-induction of angiogenesis and the early stages of tumor growth&IL 6 -responsible for the constitutional features.
20MyxomasTypically pedunculated and gelatinous in consistency; smooth, villous or friable.About 35 % of are friable or villous, these tend to present with emboliMyxoma triad – constitutional features,emboli and valvular obstructionCalcification , necrosis and cystic changes seenMostly (90%) solitary
21MyxomasThe cardiovascular manifestations depend upon the anatomic location of the tumor.Mostly seen in females40-60 years of ageCharacteristic tumor plop may be heard80% -originate in the left atrium,most of the remainder is found in the right atriumconstitutional symptoms (eg, weight loss, fever) and laboratory abnormalities
23Myxoma - ASSOCIATIONS Characteristics Syndromic associations Younger than 40 yearsBiatrial ,ventricular , valvesall first degree relatives should be screenedRecurrent – 30%No gender predilectionLAMB (lentigines, atrial myxomas,mucocutaneous myxomas, and blue nevi),NAME (nevi, atrial myxomas, myxoid neurofibroma, and ephelidesCarney syndrome (atrial, cutaneous and mammary myxomas, lentigines, blue nevi, endocrine disorders and testicular tumours)
24Myxoma Carney syndrome Histological features Mutations in : PRKAR1A (protein kinase A regulatory subunit 1A )MYH8 - myosin isoformPASVimentinS100 and NSE- POSITIVITY
25TreatmentPrompt resection-due to the risk of embolization or cardiovascular complications, including sudden deathResult of surgical resection is good ( <5% mortality)Cardiac autotransplantation
26LipomasLipomas and fibrolipomas are characterized by a predominance of benign fatty cells.About half of these tumors occur in the subendocardial region with the remainder evenly divided between the myocardial and subepicardial regionsThey may also occur on valves
27LipomasSymptoms are generally related to local tissue encroachment (arrhythmias, conduction block, sudden death)valvular tumors can cause insufficiency and symptoms of heart failureThe diagnosis can be made with echocardiography and the distinctive fat pattern (increased T1 weighted signals) on MRI.Because of the symptoms they cause and their progressive growth, myocardial lipomas require resection.
29Lipomatous hypertrophy of the interatrial septum Lipomatous hypertrophy of the interatrial septum is an exaggerated growth of normal fat existing within the septum and is not a true tumor.developmental disorder caused by expansion of adipose tissue trapped in the interatrial septum during embryogenesisThe septal hypertrophy may be as much as 2 – 3 cm in thicknessseen in older patients and in those who are obese
30Lipomatous hypertrophy of the interatrial septum this disorder is associated with the presence of coronary artery disease in proportion to the degree of atrial septal thicknessLipomatous hypertrophy of the interatrial septum is indistinguishable from lipoma except that the former occurs in the atrial septum with a typical distribution (generally sparing the fossa ovalis).In the absence of symptoms of atrial arrhythmias, heart block or rare vena caval obstruction, they do not require resection
31Pericardial lipomasPericardial lipomas are typically an incidental finding and clinically insignificant.Rarely, a pericardial lipoma can assume gigantic proportions and its appearance on a chest radiograph may be mistaken for a huge pericardial effusion or massive cardiomegalyBenign pericardial lipomas can infiltrate the myocardium. If the ventricular septum is invaded, communication between the pericardial space and the right ventricular cavity may result.
32Papillary fibroelastomas third most common primary cardiac tumor in adultsTheir appearance is compared to pom-pom or sea anemones
33Clinical featuresSymptoms -caused by embolization of the tumor/thrombus.most common clinical presentation- stroke /TIA.Commonly attached to the aortic valve30%- incidentally diagnosed by echocardiography, at cardiac surgery, or at autopsy
34TREATMENTSURGERY –1. patients who have had embolic events or complications directly related to tumor mobility (eg, coronary ostial occlusion)2.Those with highly mobile or large (≥1 cm) tumorsRecurrence not reported
36Rhabdomyomas develop almost exclusively in children before the age of one year; most common tumor in children80 to 90 percent are associated with tuberous sclerosisfound in the ventricles or on the atrioventricular valves.Most rhabdomyomas regress spontaneouslyresection required only if child is symptomaticSymptoms are caused by obstruction of blood flow through the heart or consist of rhythm disturbancesPresent with features of preexcitation on the ECG
38Rhabdomyositis A rare form of cardiomyopathy in infants Tumor nodules are not grossly apparentMicroscopically, the cardiac muscle fibers and conduction system are diffusely involved with rhabdomyomatous histologic changesRecurrent atrial tachycardia and sudden death from intractable ventricular tachycardia
39Why rhabdomyomas regress? Characterised by high expression of ubiquitin which starts expressing from 32 week onwardsAbsent mitotic activityThis ubiquitin is responsible for the degradation of rhabdomyoma cells and hence tumor regressionCharacteristic spider cells are formed in the process.
41Fibromas The second most common pediatric cardiac tumor but can also occur in adultsFibromas usually arise in the ventricular muscle and may become quite large.They arise approximately five times more frequently in the left ventricle than the right ventricleFibromas do not regress spontaneously.
42FibromasHeart failure is the most common symptom, due to obstruction of blood flow or interference with valvular function.Myocardial dysfunction and conduction disturbances also occur.Echocardiography, supplemented with CT scans or MRI confirms the diagnosis.Symptomatic tumors should be resected. Complete resection of very large tumors may require cardiac transplantation.
44Teratomas Teratomas arise within the pericardium Although these tumors are generally benignHigh risk for developing hydrops fetalis in utero because of mechanical effectsCause cardiac tamponade .
45TeratomasTreatment therefore requires either fetal tumor excision, or caesarean section and immediate operation on the newbornBecause teratomas usually have a single blood supply and are not invasive, properly timed tumor surgery is straightforward and successful
46Purkinje cell tumors/hamartomas Consist of small, flat sheets of cells most frequently located in the left ventricleThey are undetectable by echocardiographic or radiologic techniques.These are usually tumors of young children and present with incessant ventricular tachycardiaECG demonstrate a bundle branch pattern (right bundle branch block when the tumor is in the left ventricle).Electrophysiologic studies can localize the tumors, facilitating surgical excision.
47Paragangliomasneuroendocrine tumors that can be either benign or malignant and can be hormonally active or inactive.Hormonally active tumors primarily produce norepinephrine and may cause systemic symptoms (eg, headache, sweating, tachycardia, hypertension)In hormonally inactive , symptoms are due to cardiac compression or tamponadethe hormonally inactive tumors are more frequent in the pericardium
48Paragangliomas Paragangliomas can be localized with echo extremely vascularCoronary angiography is required to plan the operative resection
49ParagangliomasBoth benign and malignant paragangliomas occurring within the pericardium parasitize the cardiac blood supply and are very difficult to exciseAll intrapericardial paragangliomas require resectionCardiopulmonary bypass and even circulatory arrest may be required because of the high degree of vascularity, or to moderate the extreme hypertension possible from tumor manipulation or hormonally active tumorsIf complete resection is not possible, cardiac transplantation may be required
50 PRIMARY MALIGNANT TUMORS Malignant tumors constitute approximately 15 percent of primary cardiac tumorsSarcomas are the most commonVirtually all types of sarcomas have been reported in the heartPrognosis depends onMitotic activity, extent of tumor necrosis and cellular differentiation.
51Malignant cardiac tumors As with benign lesions, the clinical presentation is largely determined by the location of the tumor, rather than its histopathology.The diagnostic approach relies upon echocardiography, MRI, and CT to define the presence of a tumor and its anatomic relationship to normal structuresIncreased mitoses
53AngiosarcomasAngiosarcomas are composed of malignant cells that form vascular channels.Angiosarcomas arise predominantly in the right atrium
54RhabdomyosarcomasRhabdomyosarcomas constitute as many as 20 percent of all primary cardiac sarcomasThese tumors are most commonly found in adults, although they have also been described in children.Multiple sites of myocardial involvement are common, and there is no predominant localization within any area of the heart.
55FibrosarcomasFibrosarcomas and malignant fibrous histiocytomas are white fleshy ("fish flesh") tumors that are composed of spindle cells, and may have extensive areas of necrosis and hemorrhageThese tumors tend to extensively infiltrate the myocardium
56LeimyosarcomasLeimyosarcomas are spindle-celled, high-grade tumors that arise more frequently in the left atriumThese sarcomas have both a high rate of local recurrence and systemic spread.
57Mesothelioma cardiac mesothelioma arise from the pericardium, where they are usually malignantproduce tamponade and constrictionThese tumors will be seen with the usual diagnostic toolsPericardiocentesis may yield a cytologic diagnosis
58 Mesotheliomamesotheliomas also arise as benign tumors of the atrioventricular node where they may produce heart blockDiagnosis of the AV nodal tumors causing heart block can be confirmed with echocardiographyResection is the treatment of choice for mesothelioma, but the prognosis with malignant pericardial mesotheliomas is very poorNo much role for radiation and/or chemotherapy.
59Treatment and prognosis In general, sarcomas proliferate rapidly, and cause death through widespread infiltration of the myocardium, obstruction of blood flow through the heart, and/or distant metastases.Although complete resection is the treatment of choice, most patients develop recurrent disease and die of their malignancy even if their tumor can be completely resected
60Treatment and prognosis The median survival is typically 6 to 12 months although long-term survival has been reported with complete resection.Patients with low-grade sarcomas may have a better prognosisAdjuvant chemotherapy and radiation therapy have been used infrequently either after resection to improve results or in metastatic involvement.Rhabdomyosarcomas may have a better outcome with chemotherapy
61Treatment and prognosis The poor results with surgical resection have led to occasional attempts to treat patients with cardiac transplantation, if extracardiac disease is not presentcardiac autotransplantation is another novel approach.In these cases, the heart is excised, the tumor is resected ex vivo, and the heart is reconstructed before being reimplanted. The advantage of this procedure is the increased ease with which major resection and reconstruction can be performed, while at the same time avoiding the need for antirejection treatment
62Lymphomas and other tumors Primary lymphomas arise in the myocardium – usually of diffuse B cell typeSeen in immunocompetent as well as immunosuppressedHIVPTCL – Post Transplant Cardiac Lymphoma – associated with EBVVery aggressive tumorsChemotherapy main stay of therapy
63SECONDARY CARDIAC TUMORS Cardiac involvement may arise from hematogenous metastases, direct invasion from the mediastinum, or tumor growth into the vena cava and extension into the right atriumMalignant melanomas are particularly likely to metastasize to the heart. Other solid tumors commonly associated with cardiac involvement include lung cancer, breast cancer, soft tissue sarcomas, renal carcinoma, esophageal cancer, hepatocellular carcinoma, and thyroid cancer.There is also a high prevalence of secondary cardiac involvement with leukemia and lymphoma
64Secondary cardiac tumors Cardiac or pericardial metastasis should be considered whenever a patient with known malignancy develops cardiovascular symptoms, particularly if this occurs in conjunction with cardiomegaly, a new or changing heart murmur, electrocardiographic conduction delay, or arrhythmia.Emboli thought to originate in the heart should also raise the possibility of cardiac involvement with tumor.Cardiac metastases rarely may be the first manifestation of malignant disease
65Secondary cardiac tumors The specific symptoms will reflect the site of cardiac involvement, in a manner analogous to primary cardiac tumorsThe diagnostic evaluation is the same as that for primary cardiac tumors and relies upon echocardiography, MRI, and CT to ascertain the extent of cardiac involvement.Resection of cardiac metastases has been used to provide symptom palliation and prolong life
68Imaging features of cardiac features – in a nut shell
69Summary Cardiac tumours are rare. A cardiac mass most likely represents a thrombus or vegetation.Most cardiac tumours are secondary, ie, originate from a primary tumour elsewhere.Most primary cardiac tumours are benignIn addition to obtaining a careful history, a non- invasive diagnosis of cardiac tumours can usually be made by taking the following factors into account:Histology based likelihood.Tumour location.Age at presentation.Imaging characteristics
70Summary Rhabdomyomas usually decrease in size with age. The most common cardiac tumour involving valves is a papillary fibroelastoma.The most common primary malignant tumour is a sarcoma.Angiosarcomas are the most common histologic subtype of sarcoma and are usually found in the right atriumThe most common benign cardiac tumour is a myxoma.Myxomas are generally found in the left atrium arising from a stalk attached to the fossa ovalis membrane.The most common primary cardiac tumours in children are rhabdomyomas and fibromas, both of which are benign.