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Cardiac tumors Dr Shreetal Rajan Nair

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1 Cardiac tumors Dr Shreetal Rajan Nair
Senior Resident, Department of Cardiology, Calicut Medical College 72 slides

2 History 1559 : Columbus , a noted pathologist – first to report
1934 : first report of a primary sarcoma of the heart diagnosed in a living patient by Barnes 1942 : surgical resection of intrapericardial teratoma by Beck 1951 : The first successful excision of a cardiac tumor performed by Maurer 1952 : Goldberg diagnosed intracavitary myxoma by angiocardiography 1968 : Shattenberg used echo to diagnose cardiac tumors

3 Classification secondary Breast lungs
Primary benign (75%) malignant (25%) Myxoma – 50% Rhabdomyoma – 20% Fibroma Papillary fibroelastoma Lipoma Angiosarcoma Rhabdomyosarcoma Leiomyosarcoma Lymphomas

4 Incidence 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

5 Incidence of benign tumors
Shapiro LM. Cardiac tumours: diagnosis and management. Heart 2001;85:218e22. Shapiro LM. Cardiac tumours: diagnosis and management. Heart 2001; 85:

6 Tumors 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

7 Tumors of the heart- clinical presentations
Primary tumors most commonly involve the endocardium, followed by myocardium and then epicardium Metastatic involvement of heart is reverse that of primary tumors Endocardium- obstruction to flow of blood through heart Myocardium - rhythm abnormalities Epicardium – pericardial effusion, cardiac tamponade

8 Approach to a cardiac tumor


10 Diagnostic evaluation
Confirm the diagnosis locate the lesion within the heart Whether benign or malignant

11 Echocardiography 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

12 Echocardiography - TEE vs TTE
Transthoracic echocardiography – the initial diagnostic tool Transesophageal 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

Both provide noninvasive, high resolution images of the heart, but MRI generally is preferred. cMRI -detailed anatomic images T1- and T2-weighted sequences help to identify the chemical microenvironment within a tumor CT -useful when calcification is present and MRI is not available or is contraindicated.

14 CORONARY ANGIOGRAPHY  Mapping the blood supply of tumors arising from the epicardial surfaces vital to the success of excising such tumors. Significant involvement of coronary arteries - resection and grafting of such arteries

15 Role of Transvenous biopsy
Limited data are available on the risks and benefits considered reasonable if potential benefits outweigh risks and there is diagnostic dilemma

16 PET SCAN Identifying cardiac involvement in patients with
-metastatic tumors -atrial myxoma - lipomatous septal hypertrophy

17 Benign 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

18 Myxomas 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) cell Produce VEGF-induction of angiogenesis and the early stages of tumor growth & IL 6 -responsible for the constitutional features.

19 Myxoma ( lepidic ) cells

20 Myxomas Typically pedunculated and gelatinous in consistency; smooth, villous or friable. About 35 % of are friable or villous, these tend to present with emboli Myxoma triad – constitutional features,emboli and valvular obstruction Calcification , necrosis and cystic changes seen Mostly (90%) solitary

21 Myxomas The cardiovascular manifestations depend upon the anatomic location of the tumor. Mostly seen in females 40-60 years of age Characteristic tumor plop may be heard 80% -originate in the left atrium, most of the remainder is found in the right atrium constitutional symptoms (eg, weight loss, fever) and laboratory abnormalities


23 Myxoma - ASSOCIATIONS Characteristics Syndromic associations
Younger than 40 years Biatrial ,ventricular , valves all first degree relatives should be screened Recurrent – 30% No gender predilection LAMB (lentigines, atrial myxomas,mucocutaneous myxomas, and blue nevi), NAME (nevi, atrial myxomas, myxoid neurofibroma, and ephelides Carney syndrome (atrial, cutaneous and mammary myxomas, lentigines, blue nevi, endocrine disorders and testicular tumours)

24 Myxoma Carney syndrome Histological features Mutations in :
PRKAR1A (protein kinase A regulatory subunit 1A ) MYH8 - myosin isoform PAS Vimentin S100 and NSE - POSITIVITY

25 Treatment Prompt resection-due to the risk of embolization or cardiovascular complications, including sudden death Result of surgical resection is good ( <5% mortality) Cardiac autotransplantation

26 Lipomas Lipomas 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 regions They may also occur on valves

27 Lipomas Symptoms are generally related to local tissue encroachment (arrhythmias, conduction block, sudden death) valvular tumors can cause insufficiency and symptoms of heart failure The 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.

28 Lipoma

29 Lipomatous 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 embryogenesis The septal hypertrophy may be as much as 2 – 3 cm in thickness seen in older patients and in those who are obese

30 Lipomatous hypertrophy of the interatrial septum
this disorder is associated with the presence of coronary artery disease in proportion to the degree of atrial septal thickness Lipomatous 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

31 Pericardial lipomas Pericardial 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 cardiomegaly Benign pericardial lipomas can infiltrate the myocardium. If the ventricular septum is invaded, communication between the pericardial space and the right ventricular cavity may result.

32 Papillary fibroelastomas
third most common primary cardiac tumor in adults Their appearance is compared to pom-pom or sea anemones

33 Clinical features Symptoms -caused by embolization of the tumor/thrombus. most common clinical presentation- stroke /TIA. Commonly attached to the aortic valve 30%- incidentally diagnosed by echocardiography, at cardiac surgery, or at autopsy

34 TREATMENT SURGERY – 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) tumors Recurrence not reported


36 Rhabdomyomas develop almost exclusively in children
before the age of one year; most common tumor in children 80 to 90 percent are associated with tuberous sclerosis found in the ventricles or on the atrioventricular valves. Most rhabdomyomas regress spontaneously resection required only if child is symptomatic Symptoms are caused by obstruction of blood flow through the heart or consist of rhythm disturbances Present with features of preexcitation on the ECG


38 Rhabdomyositis A rare form of cardiomyopathy in infants
Tumor nodules are not grossly apparent Microscopically, the cardiac muscle fibers and conduction system are diffusely involved with rhabdomyomatous histologic changes Recurrent atrial tachycardia and sudden death from intractable ventricular tachycardia

39 Why rhabdomyomas regress?
Characterised by high expression of ubiquitin which starts expressing from 32 week onwards Absent mitotic activity This ubiquitin is responsible for the degradation of rhabdomyoma cells and hence tumor regression Characteristic spider cells are formed in the process.

40 Spider cells in rhabdomyoma

41 Fibromas  The second most common pediatric cardiac tumor but can also occur in adults Fibromas 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 ventricle Fibromas do not regress spontaneously.

42 Fibromas Heart 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.

43 Fibromas

44 Teratomas Teratomas arise within the pericardium
Although these tumors are generally benign High risk for developing hydrops fetalis in utero because of mechanical effects Cause cardiac tamponade .

45 Teratomas Treatment therefore requires either fetal tumor excision, or caesarean section and immediate operation on the newborn Because teratomas usually have a single blood supply and are not invasive, properly timed tumor surgery is straightforward and successful

46 Purkinje cell tumors/hamartomas
Consist of small, flat sheets of cells most frequently located in the left ventricle They are undetectable by echocardiographic or radiologic techniques. These are usually tumors of young children and present with incessant ventricular tachycardia ECG 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.

47 Paragangliomas neuroendocrine 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 tamponade the hormonally inactive tumors are more frequent in the pericardium

48 Paragangliomas Paragangliomas can be localized with echo
extremely vascular Coronary angiography is required to plan the operative resection

49 Paragangliomas Both benign and malignant paragangliomas occurring within the pericardium parasitize the cardiac blood supply and are very difficult to excise All intrapericardial paragangliomas require resection Cardiopulmonary 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 tumors If complete resection is not possible, cardiac transplantation may be required

Malignant tumors constitute approximately 15 percent of primary cardiac tumors Sarcomas are the most common Virtually all types of sarcomas have been reported in the heart Prognosis depends on Mitotic activity, extent of tumor necrosis and cellular differentiation.

51 Malignant 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 structures Increased mitoses


53 Angiosarcomas Angiosarcomas are composed of malignant cells that form vascular channels. Angiosarcomas arise predominantly in the right atrium

54 Rhabdomyosarcomas Rhabdomyosarcomas constitute as many as 20 percent of all primary cardiac sarcomas These 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.

55 Fibrosarcomas Fibrosarcomas and malignant fibrous histiocytomas are white fleshy ("fish flesh") tumors that are composed of spindle cells, and may have extensive areas of necrosis and hemorrhage These tumors tend to extensively infiltrate the myocardium

56 Leimyosarcomas Leimyosarcomas are spindle-celled, high-grade tumors that arise more frequently in the left atrium These sarcomas have both a high rate of local recurrence and systemic spread.

57 Mesothelioma  cardiac mesothelioma arise from the pericardium, where they are usually malignant produce tamponade and constriction These tumors will be seen with the usual diagnostic tools Pericardiocentesis may yield a cytologic diagnosis

58   Mesothelioma mesotheliomas also arise as benign tumors of the atrioventricular node where they may produce heart block Diagnosis of the AV nodal tumors causing heart block can be confirmed with echocardiography Resection is the treatment of choice for mesothelioma, but the prognosis with malignant pericardial mesotheliomas is very poor No much role for radiation and/or chemotherapy.

59 Treatment 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

60 Treatment 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 prognosis Adjuvant 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

61 Treatment and prognosis
The poor results with surgical resection have led to occasional attempts to treat patients with cardiac transplantation, if extracardiac disease is not present cardiac 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

62 Lymphomas and other tumors
Primary lymphomas arise in the myocardium – usually of diffuse B cell type Seen in immunocompetent as well as immunosuppressed HIV PTCL – Post Transplant Cardiac Lymphoma – associated with EBV Very aggressive tumors Chemotherapy main stay of therapy

Cardiac involvement may arise from hematogenous metastases, direct invasion from the mediastinum, or tumor growth into the vena cava and extension into the right atrium Malignant 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

64 Secondary 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

65 Secondary cardiac tumors
The specific symptoms will reflect the site of cardiac involvement, in a manner analogous to primary cardiac tumors The 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

66 Benign tumors of the heart

67 Malignant tumors of the heart

68 Imaging features of cardiac features – in a nut shell

69 Summary 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 benign In 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

70 Summary 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 atrium The 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.

71 Thank you

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