Cardiac tumorsDr Shreetal Rajan Nair

Senior Resident, Department of Cardiology,Calicut Medical College

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 Barnes1942 : surgical resection of intrapericardial teratoma

by Beck 1951 : The first successful excision of a cardiac tumor

performed by Maurer 1952 : Goldberg diegnosed intracavitary myxoma by

angiocardiography1968 : Shattenberg used echo to diagnose cardiac

tumors

Classification Primary benign (75%)

malignant (25%)

Myxoma – 50%Rhabdomyoma – 20%Fibroma Papillary fibroelastomaLipoma Angiosarcoma RhabdomyosarcomaLeiomyosarcoma Lymphomas

secondary Breast

lungs

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

Incidence of benign tumors

Shapiro LM. Cardiac tumours: diagnosis and management. Heart 2001; 85: 218 - 22.

LITERATURE

Tumors of the heart - 1. Constitutional or systemic

2. Embolic 3. Cardiac 4. 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

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

Approach to a cardiac tumorDifferential diagnosis

PUOIE/CONNECTIVE

TISSUE DISEASESVALVULAR LESIONSCCF

CONDUCTION ABNORMALITIES

EMBOLISMSYNCOPEPERICARDIAL

EFFUSION

Diagnostic evaluation

Confirm the diagnosis

locate the lesion within the heart

Whether benign or malignant

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

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

CARDIAC MRI VS CTBoth 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.

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

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

Benign tumorsAround 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

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.

Myxoma ( lepidic ) cells

Myxomas Typically pedunculated and gelatinous in

consistency; smooth, villous, or friable.

Calcification , necrosis and cystic changes seen

Mostly (90%) solitary

About 35 % of are friable or villous, these tend to present with emboli.

Larger tumors -smooth surface and

associated with cardiovascular symptoms.

Myxomas The cardiovascular manifestations depend

upon the anatomic location of the tumor.

Mostly seen in females 40-60 years of age

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

Syndrome Myxoma 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)

Myxoma Carney syndrome Histological features

Mutations in :

PRKAR1A (protein kinase A regulatory subunit 1A )

MYH8 - myosin isoform

PAS

Vimentin

S100 and NSE - POSITIVITY

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

Papillary fibroelastomas

Second most common primary cardiac tumor in adults

Their appearance is compared to pom-pom or sea anemones

Clinical featuresSymptoms -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

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

Lipomas

Lipomas &fibrolipomas - characterized by a predominance of benign

fatty cells.

About half of these tumors occur in the subendocardial region.Others in the myocardial and subepicardial regions

They may also occur on valves

Lipoma

Lipomas Symptoms- generally related to local tissue

encroachment (arrhythmias, conduction block, sudden death)

valvular tumors – insufficiency & symptoms of heart failure

Diagnosis - echocardiography and the distinctive fat pattern on MRI.

Require resection.

Lipomatous hypertrophy of the interatrial septum 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 cm in thickness and is seen primarily in older patients and in those who are obese

Lipomatous hypertrophy of the interatrial septumAssociated with the presence of CAD in

proportion to the degree of atrial septal thickness

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

Pericardial lipomasIncidental finding and clinically insignificant.

Rarely assumes 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.

Rhabdomyomas Develop almost exclusively in children, mostly <1

year of age80 to 90% are associated with tuberous sclerosis Usually found in the ventricular walls/AV valves.Most regress spontaneouslyResection is usually not required unless symptomatic Symptoms –due to obstruction of blood flow through

the heart or consist of rhythm disturbancesPresent with features of preexcitation on the ECG

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

Why rhabdomyomas regress?High expression of ubiquitin which starts

expressing from 32 week onwards

Absent mitotic activity

Ubiquitin is responsible for the degradation of rhabdomyoma cells and hence tumor regression

Characteristic spider cells are formed in the process.

Spider cells in rhabdomyoma

Fibromas Second most common pediatric cardiac

tumor and can also occur in adults Histologically similar to fibromas arising

elsewhere in the body. Usually arise in the ventricular muscle and

may become quite large.Do not regress spontaneously. Arise approximately 5 times more frequently

in the LV than RV

FibromasMost common symptom, due to obstruction of

blood flow or interference with valvular function.

Myocardial dysfunction and conduction disturbances

Echocardiography, supplemented with CT/MRI confirms the diagnosis.

Symptomatic tumors should be resected. Complete resection of very large tumors may require cardiac transplantation.

Fibromas

Teratomas Arise within the pericardium, but do not

originate from cardiac structures

Although generally benign,can have serious mechanical consequences by causing tamponade or through direct pressure on the heart.

Risk of death in-utero or immediately after birth

Teratomas Treatment therefore requires either fetal

tumor excision, or caesarean section and immediate operation on the newborn

Have a single blood supply and are not invasive, properly timed tumor surgery is straightforward and successful

Purkinje cell tumors/hamartomas

Consist of small, flat sheets of cells most frequently located in the left ventricle, and on the endocardial and epicardial surfaces

Undetectable by echocardiographic or radiologic techniques.

Tumors of young children and present with incessant ventricular tachycardia

ECGs often 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.

Paragangliomas Neuroendocrine tumors that can be hormonally

active or inactive. In tumors not producing catecholamines,

symptoms are due to cardiac compression or tamponade.

In contrast, cardiac paragangliomas that are hormonally active primarily produce norepinephrine and may cause systemic symptoms (eg, headache, sweating, tachycardia, hypertension)

Hormonally inactive tumors are more frequent in the pericardium

ParagangliomasMay be localized with echocardiography.

Extremely vascular

Coronary angiography -to plan the operative resection

Paragangliomas Paragangliomas (benign/malignant) within the

pericardium parasitize the cardiac blood supply and hence are very difficult to excise

All intrapericardial paragangliomas require resection

Cardiopulmonary bypass and even circulatory arrest may be required due to 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

PRIMARY MALIGNANT TUMORSMalignant tumors constitute approx 15% 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.

Malignant cardiac tumors

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

Angiosarcomas

Composed of malignant cells that form vascular channels.

Pathology may overlap with Kaposi's sarcoma, which can also involve the myocardium

Arise predominantly in the right atrium

Rhabdomyosarcomas

Constitute as many as 20% of all primary cardiac sarcomas

Most commonly found in adults, although they have also been described in children.

Multiple sites of myocardial involvement are common, No predominant localization within any area of the heart.

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

Leimyosarcomas

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.

Mesothelioma Although most arise in the pleura, these can also

arise from the pericardium, where they are usually malignant

Those arising in the pericardium produce tamponade and constriction

Seen with echocardiography, CT scan, MRI and sometimes by chest x-ray

Pericardiocentesis may yield a cytologic diagnosis

Mesothelioma

More rarely they may arise as benign tumors of the AV node where they may produce 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

The addition of radiation and/or chemotherapy has been attempted but has not been shown to be of value.

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.

Treatment and prognosisThe median survival is typically 6 to 12 months

although long-term survival has been reported with complete resection.

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

Treatment and prognosisThe 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.

Here, the heart is excised, tumor 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

Lymphomas and other tumors Primary lymphomas arising in the myocardium have

been reported.

In a review of 40 cases identified from the literature between 1995 and 2002, the outlook was generally poor

However, 38 percent of cases achieved a complete response with systemic therapy.

Other tumors may also arise in the heart, including paragangliomas and extramedullary plasmacytomas

SECONDARY 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 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

Secondary cardiac tumorsCardiac 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

Secondary cardiac tumors

The specific symptoms reflect the site of cardiac involvement.

The diagnostic evaluation is the same as that for primary cardiac tumors and relies upon echocardiography, MRI, and CT.

Resection of cardiac metastases has been used to provide symptom palliation and prolong life

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 history, a non- invasive diagnosis of cardiac tumours can usually be made by the following:-Histology based likelihood.-Tumour location.-Age at presentation.-Imaging characteristics

Benign tumors of the heart

Malignant tumors of the heart

Imaging features of cardiac features – in a nut shell

Summary 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.

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

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