Cardiac tumours

CARDIAC TUMOURS

INTRODUCTION Primary tumors of the heart are rare across

all age groups, with a reported prevalence of 0.001% to 0.03% in autopsy series.

Secondary involvement of the heart by extracardiac tumors is 20 to 40 times more common than by primary cardiac tumors.

In the fetus, there is a higher proportion of germ cell tumors.

About 75% of all primary cardiac tumors are regarded as benign neoplasms and cardiac myxoma accounts for at least half of them.

By virtue of their anatomic locations, primary cardiac tumors are capable of producing myriad cardiac, embolic, and systemic symptoms

Of the remaining 25% (malignant neoplasms) the majority are sarcomas; lymphomas are the next most common.

The diagnosis of primary cardiac tumors is frequently challenging.

The symptoms associated with most primary cardiac tumors are nonspecific, and this elusiveness often results in a delay in the diagnosis of disease.

Extra Cardiac Tumors can involve the heart by direct invasion from adjacent malignancies (lung, breast), by lymphatic spread, or by metastatic spread of distant disease (lymphoma, melanoma).

Nonprimary cardiac tumors can affect the heart by: Invasion of the pericardium, epicardium, myocardium, or endocardium Production of biologically active substances Toxic effects of treatment on the heart (e.g., radiation or chemotherapy)

Myocardial involvement by metastatic disease is less common than pericardial involvement, but does occur, particularly with lymphoma or melanoma.

Intramyocardial masses can project into or compress cardiac chambers, resulting in hemodynamic compromise.

Endocardial involvement is rarely seen.

EPIDIMEOLOGY The types of tumors predominantly seen in

adults (>16 years) are different from that seen in children (<16 years)

85% of benign tumors in adults are myxomas, lipomatous tumors, and papillary fibroelastomas

82% of benign tumors in children are rhabdomyomas, teratomas, and fibromas.

The most common malignant tumors in adults are angiosarcomas, rhabdomyosarcomas, mesotheliomas, and fibrosarcomas

In children they are rhabdomyosarcomas, fibrosarcomas, malignant teratomas, and neurogenic sarcomas.

CLINICAL MANIFESTATIONS The clinical manifestations can be

divided into four general mechanistic categories:

1. Systemic manifestations2. Embolic manifestations 3. Cardiac manifestations 4. Phenomena secondary to metastatic

diseases

SYSTEMIC MANIFESTATIONS Constitutional symptoms of fever, chills,

fatigue, malaise, and weight loss. In addition, myalgia, arthralgia, muscle

weakness, and Raynaud phenomenon. Routine laboratory tests may reveal

leukocytosis, polycythemia, anemia, thrombocytosis, thrombocytopenia, hypergammaglobulinemia, and increased ESR.

These systemic manifestations are believed to be produced by secretory products released by the tumor or by tumor necrosis.

Most commonly seen in : Cardiac Myxoma

Caused by : Increased Serum IL-6 levels.

EMBOLIC PHENOMENON Systemic or pulmonary embolism via

tumor emboli or thromboemboli from or formed on the surface of the tumor, respectively.

The propensity to embolize depends on the origin of the tumor (intramural or intracavitary), the type of the tumor, and the friability of the intraluminal tumor surface.

Brain emboli most commonly causes TIA or ischemic stroke but rarely ICH can be the presentation.

Neurological manifestations depend upon the area affected.

Coronary Artery Embolism can be seen rarely and mimicks as M.I.

Pulmonary embolization is also rare, more with Right sided tumors and Left sided tumors in presence of Intracardiac L-R shunt.

CARDIAC MANIFESTATIONSMechanisms1. Direct mechanical interference with myocardial or valvular function 2. Interruption of coronary blood flow 3. Interference with electrophysiologic conduction4. Accumulation of pericardial fluid.

PCT that are completely intramural or myocardial are typically asymptomatic, especially if the sizes are small.

Large intramural tumors that are within or pressing on major cardiac conduction pathways may, however, cause a wide variety of arrhythmias, including complete heart block and asystole in more severe cases.

In addition, large intramural tumors may also compress the cardiac cavities, obstruct the ventricular outflow tract, or contribute to insufficiency of the mitral valve.

Intracavitary component tend to cause more symptoms for patients specially if pedunculated and mobile because of their tendency to interfere with valvular and myocardial function.

For left atrial tumors, intracavitary lesions that are pedunculated and mobile can interfere with the mitral valve and produce clinical findings of mitral regurgitation that include fatigue, dyspnea, orthopnea, paroxysmal nocturnal dyspnea, cough, hemoptysis, chest pain, pulmonary edema, and peripheral edema.

However, findings atypical for mitral regurgitation, such as the aforementioned systemic and constitutional symptoms, should prompt further investigation.

These symptoms can be sudden in onset, intermittent, and positional.

Physical examination may reveal signs of pulmonary congestion with an S3 and loud and widely split S1, a holosystolic murmur most prominent at the apex with radiation to the axilla, a diastolic murmur from turbulent blood flow through the mitral orifice, and a tumor “plop.”

TUMOR PLOPThe ultrafast computed tomographic (UFTC) and Echo studies show the tumor obstructing the mitral orifice with a piston-like action, first pushing a bolus of blood into the ventricle and then coming to rest in the mitral orifice. This sudden bolus of blood plus tumor in the ventricle causes a third sound, called a "tumor plop," while relieving the left atrium of that volume and causing an abrupt y-descent in the left atrial pressure.

Sound is evanescnent and positional

It occurs later than an OS but earlier than an S3.

For intracavitary tumors located in the right atrium, findings of right-sided heart failure that include fatigue, peripheral edema, ascites,hepatosplenomegaly, and elevated JVP with a prominent a wave are the most common cardiac presentations.

Because of the right atrial location, the diagnosis is often delayed, with an average time interval from presentation to the correct diagnosis of 3 years.

Patients frequently present with rapidly progressive right-sided heart failure and also new-onset heart murmurs because of mechanical interference with the tricuspid valve.

In patients with a PFO, the buildup of right atrial pressure can produce right-to-left intracardiac shunting with resulting systemic hypoxia, cyanosis, clubbing, and polycythemia.

On occasion, patients may also present with superior vena cava syndrome caused by a large right atrial tumor.

SVC SYNDROME In the midthird of the mediastinum, the left

and right brachiocephalic veins join to form the SVC.

The SVC then extends caudally, courses anterior to the right main bronchus, and terminates in the superior aspect of the right atrium.

The SVC is joined posteriorly by the azygos vein and runs posterior to and to the right of the ascending aorta.

During its course the SVC is adjacent to the right paratracheal, azygous, right hilar, and subcarinal lymph node groups.

Blood flow in the venous system is under low pressure and the vessel itself is thin walled.

Any inflammatory process in the mediastinum or enlargement of the lymph nodes or ascending aorta can cause the SVC to be compressed

First described by William Hunter in 1757 in a patient with a syphilitic aneurysm of the ascending aorta.

Now the most common cause of SVC syndrome is malignancy, of which lung carcinoma is the most frequent, followed by lymphoma and metastatic cancer.

The severity of the syndrome depends on the rapidity of onset of the obstruction and its location.

The more rapid the onset, the more severe the symptoms because collateral veins do not have time to distend and accommodate the increased blood flow.

A typical manifestation consist of facial edema, dyspnea, and cough.

Facial edema is seen most frequently; it is worse in the morning and gets better during the day as the patient ambulates.

Other symptoms that occur less frequently include stridor, headache, syncope, dizziness, hoarseness, and confusion.

Common findings on examination include facial edema, distended neck and chest veins, arm edema, and facial plethora

Physical examination may reveal findings of peripheral edema, hepatosplenomegaly, ascites, elevated JVP with prominent a wave and steep y descent, and an early diastolic murmur or holosystolic murmur that exhibits significant respiratory or positional variation.

LV tumors can obstruct the LVOT and produce findings of left-sided heart failure and syncope as well as atypical chest pain from obstruction of a coronary artery by either direct tumor involvement or tumor emboli.

Physical examination may reveal evidence of pulmonary edema, low blood pressure, and systolic murmurs that mimic the findings of aortic or subaortic stenosis.

The murmurs and blood pressure may display considerable positional variation.

In the case of malignant primary cardiac tumors, such as angiosarcomas and primary cardiac lymphomas, malignant hemorrhagic pericardial effusion may be present.

Life-threatening cardiac tamponade and cardiac rupture leading to sudden death may also occur

METASTATIC DISEASES Truly metastatic diseases are by

definition features of malignant primary cardiac tumors.

Common sites of metastases :lung, brain, and bone,although metastases to the liver, lymph node, adrenal gland, spleen, and skin are also seen.

DIAGNOSTIC APPROACH The key to proper and timely diagnosis

of a primary cardiac tumor lies in the consideration of primary cardiac tumor in the differential diagnosis especially when atypical features are present.

When cardiac tumor is considered in the differential diagnosis, the most ideal initial method of evaluation is echocardiography either transthoracic or transesophageal, depending on the clinical circumstances.

TEE is especially advantageous in evaluating right atrial tumors.

3D echocardiography can assess the size of cardiac masses and describe complex anatomy of the heart.

If a cardiac lesion is identified, chest CT with contrast enhancement and CMR with contrast are superior modalities for delineation of the extent of tumor involvement

CT and CMR are particularly good at depicting the pericardium and great vessels and evaluating the extent of disease, and CT can also detect calcification, which is important in the differential diagnosis.

Features suggestive of malignant cardiac tumors include a large broad-based lesion occupying most of the affected cardiac chamber, hilar lymphadenopathy, extensive pericardial involvement, and hemorrhagic pericardial effusions.

Depending on the clinical settings, tissue diagnosis may be made with less invasive methods such as cytologic evaluation of pericardial or pleural fluids.

However, a negative finding on biopsy performed through these less invasive methods does not rule out a diagnosis of malignancy because the false-negative rate of these methods can be significant.

More invasive methods of tumor biopsy through mediastinoscopy or even thoracotomy may be necessary to obtain a definitive diagnosis.

Of note, the great majority of malignant cardiac tumors represent local or distant metastases by an extracardiac tumor, and most malignant primary cardiac tumors are diagnosed at an advanced stage with distant metastases already present.

ECHO EVALUATION A cardiac mass is defined as an abnormal structure within or immediately adjacent to the heart. There are three basic types of cardiac masses: Tumor Thrombus Vegetation

The first step in assessing a possible cardiac mass is to ensure it is not an artifact caused by electrical interference, characteristics of the ultrasound transducer/system, or various physical factors influencing image formation from the reflected ultrasound signals.

Appropriate transducer selection, scanning technique, and evaluation from multiple examining windows will help to distinguish artifacts from actual anatomic structures

DIFFIRENTIAL DIAGNOSIS OF MASS

A specific type of cardiac involvement by tumor that should be recognized by the echocardiographer is the extension of renal cell carcinoma up the inferior vena cava.

A “fingerlike” projection of a tumor may protrude into the right atrium (RA) from the inferior vena cava, and the tumor can be followed retrograde (from a subcostal approach) back to the kidney.

Correlation with other wide-view imaging techniques is needed for full delineation of the tumor extent. Uterine tumors also occasionally present in this fashion.

BENIGN TUMORS MYXOMAS1. Most common type of primary cardiac

tumor2. 30% to 50% of all primary tumors3. Annual incidence of 0.5 per million

population4. Most commonly presents in adults

30 to 50 years and 65% in Women

5. 4.5% to 10% myxomas are familial6. Arises from multipotent mesenchymal

cells7. Typically benign but embolic tumor

fragments can be deposited along arterial circulation.

The pathogenesis of cardiac myxoma is poorly understood, especially for those tumors that occur sporadically.

Studies have, however, shed more light on the pathogenesis of familial cases of cardiac myxomas.

Carney syndrome accounts for the majority of familial cases of cardiac myxoma and for 7% of all cardiac myxomas.

CARNEY SYNDROME Autosomal Dominant syndrome Myxoma in cardiac and several

extracardiac locations Spotty skin pigmentation Endocrine hyperactivity Other tumors (such as testicular Sertoli

cell tumor, pituitary adenoma, and thyroid tumors).

Carney syndrome show no age or gender predilection, can be single or multiple, can occur in any intracardiac location, and tend to recur with a rate of 20% despite adequate surgical excision.

In contrast, sporadic cases of cardiac myxoma tend to occur in women of middle age and as isolated lesions in the left atrial aspect of interatrial septum.

Sporadic cases have a lower recurrence rate (roughly 3%) than those mentioned previously.

Mutations in PRKAR1A, a phosphorylated perinatal myosin isoform.

Majority of the patients will present with at least one of the classic triad of obstructive cardiac, embolic, and constitutional or systemic signs.

Obstructive cardiac findings Dizziness dyspnea Cough pulmonary edema heart failure.

Result of mechanical interference of mitral valve by the tumor and account for the most common presenting findings in the triad. Tumor embolism Most common cardiac tumor to embolise and virtually to every organSymptoms depend on the location of the tumor and the patency of the foramen ovale.

INVESTIGATIONS2/3rd have abnormal ECGs mainly showing LA enlargement while atrial arrythmias and conduction disorders are rare2/3rd pts CXR shows evidence of elevated LA pressure, such as LA enlargement, vascular redistribution, prominent pulmonary trunk Pulmonary edema, is found in 53% of patients with LA myxoma.

Cardiomegaly is seen in 37% and 50% of LA and RA myxomas, respectively.

Intracardiac tumor calcification is a rare finding in LA myxomas but is in 56% of patients with right atrial myxoma.

The typical imaging modalities used for diagnostic and preoperative assessment purposes include CT, CMR, and echocardiography.

Most cardiac myxomas appear as spherical or ovoid masses with lobular contour on CT and CMR scans.

Two thirds of myxomas are heterogeneous, whereas one third appear homogeneous on CT.

Contrast-enhanced CT reveals that most myxomas have an overall attenuation lower than that of myocardium

Echocardiography is the most commonly used modality for diagnostic purposes, and TEE is the preffered echo modality.

83% in LA and 12.7% occur in RA and 1.3% are biatrial.

Only 1.7% and 0.6% of myxomas occur in the LV and RV respectively.

Usually pedunculated tumors with a fibrovascular stalk attaching to the subendothelial base of IAS in the region of the fossa ovalis.

Rarely, cardiac myxoma can involve heart valves directly.

Size usually 4 to 8 cm in diameter with maximum reported as 16cms.

The mean weight is 37gm. Half of the cardiac myxomas have a

smooth compact surface, and half have a villous surface.

Myxomas with a villous surface are more likely to embolize.

The treatment is prompt surgical resection

Complete excision is the goal, although this may not be possible in all instances.

Postoperative mortality in most series ranges from 0% to 7.5%.

Recurrence rate is 3% which can be local or in extracardiac locations, such as the brain, lung, skeletal muscle, bone, kidney, gastrointestinal tract, skin, and other soft tissue sites.

Myxomas may be sessile or pedunculated, spheric or polypoid.

“Syndrome myxoma” refers to one of several genetic disorders which should be suspected in patients< 40 years with multiple cardiac myxomas.

Myxoma syndromes include 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).

LIPOMAS AND LIPOMATOUS HYPERTROPHY Benign lipomatous tumor is the second

most common primary neoplasm of the heart and can be divided into two major groups primarily on the basis of the degree of encapsulation

Lipoma Lipomatous hypertrophy of the atrial

septum (LHAS).

Sporadic with equal occurences in both genders.

Clinically, mostly asymptomatic and typically are incidental findings.

Can occur at any atrial or ventricular surface most commonly in the subepicardial and subendocardial locations, although intramyocardial lesions have also been reported.

Subendocardial lipomas with a prominent intracavitary component can result in symptoms of heart failure.

Subepicardial tumors are usually asymptomatic, but large lesions may cause compression of the heart and produce pericardial effusion.

Intramyocardial lipoma may interfere with electrical conduction in the heart and cause arrhythmias.

TEE followed by CT is the modality of choice as they display a low attenuation signal similar to subcutaneous or mediastinal fat.

Size typically ranges from 1 to 8 cm in diameter.

The treatment of symptomatic cardiac lipomas is surgical resection, and the postoperative prognosis is excellent.

LHAS LHAS are massive fatty deposits of the

atrial septum, is a nonencapsulated excessive accumulation of fat in the atrial septum at the level of the fossa ovalis that is more than 2 cm in thickness and typically occurs in elderly, obese patients.

Mean age of diagnosis - 70yrs.

Clinically LHAS does not cause any symptoms. Can result in rhythm disturbances and even

SCD due to fatty tissue infiltration into atrial myocyte tissue, which alters the architecture and function of the myocytes.

In rare instances in which the tumor protrudes into the right atrium and the superior vena cava, patients can present with symptoms related to blood flow obstruction.

CT and CMR are the most desirable as they are superior to ECHO in differentiating between fat and connective tissue.

Atrial septum is thickened to up to 7 cm, whereas normally it is less than 1 cm but this thickening always avoids the fossa ovalis, giving the atrial septum a dumbbell or hourglass shape.

LHAS with symptomatic arrhythmias can be managed medically

Surgical management should be restricted to hemodynamically compromised patients.

PAPILLARY TUMORS OF HEART VALVES Papillary fibroelastoma is the third most

common primary cardiac tumor INCIDENCE 0.33% Fibroelastic growth distinct from lambl

excrescences and does not spontaneously regress which may have focal calcification

Clinically mostly asymptomatic.

A series showed 50% pts having TIA,stroke,angina, myocardial infarction, and dyspnea.

Cerebral embolic symptoms are present in>50% of the symptomatic patients.

Rarely, patients present with SABE and PE and sudden death have also been reported.

Firmly attached to the valvular or mural endocardium.

TEE is the recommended imaging modality for the diagnosis for lesions measuring 2 cm or more.

Generally appears as a round, oval or irregular lesion well demarcated

80% to 90% are found on the valvular endocardium most commonly on Aortic Valve.

44% have a 1-to 3-mm stalk, and this mobile type of papillary fibroelastoma appears more likely to give rise to embolism.

The treatment of papillary fibroelastoma is surgical excision or tumor shaving from the valvular leaflets with either reconstitution or, less commonly, replacement of the valve.

Asymptomatic patients with small, left-sided, nonmobile-type can be observed and excision for large >1cm mobile symptomatic cases.

RHABDOMYOMA Most frequent PCT in infants and children. 80% in <1 year but adult presentation also

seen while rare. Arrhythmias represent the most common

presentation in adults Imaging: well-circumscribed masses with

hyperintense T1- and T2-weighted spin-echo images and hypointense images after the administration of gadolinium.

70% atrial origin and 30% ventricular In symptomatic patients with

arrhythmias, antiarrhythmic drugs may be used

Surgical excision is indicated if the drugs fail to control symptoms.

No recurrence Prognosis of this disease is excellent

FIBROMAS AND HAMARTOMAS Cardiac fibroma: 2nd most common PCT

in infants and children. 90% in children <1 year No sex predilection Majority appear to be nonfamilial. Majority of cardiac fibromas behave like

hamartomas with no tendency to recur or to grow aggressively.

Usually cause arrhythmogenic disorders as VT,AF, and RBBB and LBBB.

They typically appear as subendocardial yellow-tan nodules or plaques.

The first line of treatment is antiarrhythmic drugs, allowing the regression of lesions.

Other treatments include surgical excision, electrophysiologic mapping and cryoablation, or direct-vision cryoablation of nodular tumors.

Clinically, heart failure, arrhythmias, and syncope are presenting findings while less common presenting findings include sudden death and atypical chest pain.

1/3rd of patients with cardiac fibroma can remain asymptomatic.

ECG may show evidence of LVH,RVH, BBB, AV blocks and VTs

CXR may reveal cardiomegaly with or without a focal bulge, and calcification is visible in 15% of cases.

ECHO : cardiac fibroma usually appears as a solitary homogeneous echogenic lesion

For preoperative assessment, CT and CMR are more desirable for evaluating the “resectability” of the lesion which can delineate heterogeneity of the mural mass, the degree of calcification, and its three-dimensional characteristics.

The average size of the tumor is 5 cm Generally single lesions, and they are

commonly found in the ventricular septum or the left ventricular free wall, with occurrence in the right ventricle or the atria in less than 10% of cases.

The differential feature favoring fibroma over rhabdomyoma is calcification, which occurs in fibromas but not in rhabdomyomas

Complete surgical resection in symptomatic cases is recommended.

The postoperative prognosis is typically good,

For large, unresectable tumors, cardiac transplantation may be considered if symptoms such as arrhythmias persist.

PRIMARY VASCULAR TUMORS HEMANGIOMA LYMPHANGIOMA HEMOENDOTHELIOMA

Extremely rare Benign with tendency to recur No age gender prediliction Ususally asymptomatic but can present

with palpitations, arrhythmias, heart failure, pericardial effusion, ventricular outflow tract obstruction, pseudoangina, cerebral embolism, and, in more extreme cases, sudden death.

Kasabach-Merritt syndrome: Thrombosis, consumative coagulopathy and thrombocytopenia involving giant coronary hemangioma.

ECHO is a sensitive, noninvasive method for detection of the tumor, with cardiac hemangioma appearing typically as a hyperechoic lesion.

TUMOR BLUSH : Typically seen on coronary angiogram.

No layer specific. Ventricles>Atrial Treatment is surgical resection.

MALIGNANT TUMOURS 1/4TH of PCT are malignant, and most common

of them are sarcomas followed by lymphomas. Mets are 20-40 times more common than PCT

and mostly come from lung and breast cancer. Hodgkin disease, non-Hodgkin lymphomas,

malignant melanoma,numerous primary gastrointestinal malignant neoplasms, and various types of sarcomas arising from extracardiac locations can also secondarily involve the heart.

The more common malignant PCT are1.Angiosarcomas 2.Leiomyosarcomas3.Rhabdomyosarcomas4.Malignant fibrous histiocytomas5.Undifferentiated sarcomas6.Fibrosarcomas7.Malignant lymphomas

Sarcomas showing high mitotic activity (>5 mitotic figures/10 high-power fields), extensive tumor necrosis, and poor cellular differentiation have a worse prognosis than sarcomas without these features.

The presence of metastases also confers a poorer prognosis.

On CT or CMR : large, heterogeneous, broad-based masses that frequently occupy most of the affected cardiac chambers.

CMR is usually the method of choice for imaging of sarcomas.

The tumors may also show evidence of extension into other cardiac chambers and the pericardium, and there may also be associated pericardial effusions and hilar lymphadenopathy.

ANGIOSARCOMA Most common primary cardiac sarcoma

in adults, In adults 30 to 50 years old A slight male predilection exists occur sporadically. typically present with advanced disease,

with 66% to 89% of patients already demonstrating evidence of metastatic disease at initial presentation.

Initial findings may include dyspnea, chest pain, constitutional symptoms, arrhythmias, and evidence of heart failure.

pericardial effusion and cardiac tamponade may also be the presentation.

Hemorrhagic pericardial tamponade usually indicates tumor infiltration through the myocardium.

Less commonly stroke-like neurologic symptoms secondary to cerebral metastases, are the initial presentation in patients with cardiac angiosarcoma.

ECG reveals nonspecific ST changes, arrhythmias, and AV block.

CXR may show nonspecific changes like cardiomegaly, widened mediastinum, hilar lymphadenopathy, and pleural effusion.

TEE is the initial imaging modality of choice

CT and CMR for a better characterization of the tumor growth and involvement.

appear as low-attenuation, invasive, irregular nodular masses showing heterogeneous enhancement with the administration of contrast media on CT and heterogeneous mass on CMR with frequent pericardial involvement and hemorrhagic pericardial effusion

90% in RA and involvement of structures such as the tricuspid valve, pulmonary valve, and vena cava as well as extension into the pericardium may occur.

Immunohistochemical analysis is done for final diagnosis

aggressive neoplasms with a poor prognosis and mean survival of 9 to 10 months.

Common sites of metastases : lung, liver, brain, and bone

A multidisciplinary approach to the treatment of cardiac angiosarcoma is advocated including a combination of surgery, irradiation, adjuvant or neoadjuvant chemotherapy, and immunotherapy with interleukin-12 (IL-12).

Chemotherapy : doxorubicin, an anthracycline.

Surgery for complete tumor resection. Neoadjuvant chemotherapy may be

administered to reduce the tumor mass and to facilitate surgical excision. This includes combined doxorubicin and ifosfamide and a combination of docetaxel and gemcitabine.

The use of heart transplantation remains controversial in this setting.

For advanced-stage unresectable disease, palliative treatment including the use of metallic stents for superior vena cava syndrome and for severe right ventricular outflow tract obstruction may help improve the patient’s short-term quality of life.

USE OF MDM2 drugs Novel MDM2 drugs which genetically

reduce the expresion of p53 in the neoplastic cells are being probed to reduce the neo-angiogenesis in tumour progression.

RHABDOMYOSARCOMA most common primary sarcoma of the

heart in children. The average age at disease presentation

is in the second decade of life with slight male preponderance.

Heart failure, arrhythmias, cardiac murmurs, and constitutional symptoms are common manifestations of the disease.

Occasional cases are also associated with hypereosinophilia, hypertrophic osteoarthropathy, and polyarthritis.

Nonspecific ECG and CXR findings are often present.

ECHO followed by CT/CMR to delineate the extent of tumour.

In contrast to angiosarcomas, cardiac rhabdomyosarcomas show no predilection for a specific cavity, and multiple lesions are frequently present

aggressive neoplasms with a tendency to metastasize most commonly to the lung and lymph nodes, with survival <1 year.

The primary aim of treatment is complete surgical resection

Poor response to radiotherapy and chemotherapy.

In selected cases, heart transplantation may be considered if no obvious distant metastases are present

LEIOMYOSARCOMA Leiomyosarcomas are malignant

mesenchymal tumors with histologic and immunophenotypic evidence of smooth muscle differentiation.

Mean age : fourth decade, and there is no apparent sex predilection.

The common clinical presentations include dyspnea, pericardial effusions, chest pain, atrial arrhythmias, and heart failure.

70-80% arise from the left atrium, and they tend to extend into the pulmonary trunk.

Multiple origin in 30% of patients. ECHO followed by CT/CMR for diagnosis. rapidly growing tumors with a high rate

of local recurrence and distant metastases

prognosis is poor, with a mean survival of 6 months.

Effective treatment of this progressively lethal disease is unknown.

LYMPHOMAS 1.3% to 2% of all primary cardiac

tumors. In both immunocompetent and

immunocompromised more in latter Commonly associated with HIV, Post

transplant lymphoproliferative disorder and EBV infections.

The average age : 62 to 67 with male predominance.

The common clinical presentations include chest pain, heart failure, pericardial effusion, palpitation, and arrhythmias.

Less common presentations of primary cardiac lymphomas are cardiac tamponade, pulmonary and systemic embolism.

ECG findings are nonspecific ECHO followed by CT/CMR.

Right side involvement more common. Size range from 3 to 12 cm in size with a

mean of 7 cm. Treatment : Early implementation of

anthracycline-based chemotherapy with or without radiation therapy has become the mainstay for treatment of primary cardiac lymphomas.

Radical surgical excision is generally discouraged.

Rituximab, a monoclonal antibody targeted against CD20, in combination with conventional chemotherapy,

60% of patients die of the disease within 2 months after the initial diagnosis

SECONDARY CARDIAC TUMOURS INCIDENCE-1.7% to 14% in cancer patients and

from 0.7% to 3.5% in the general population. Cardiac metastases can occur either by direct

extension, via the bloodstream or lymphatics, or by intracavitary diffusion through the inferior vena cava (IVC).

Pericardial metastasis (69%) is the most common, followed by epicardial (34%), myocardial (32%), and endocardial metastases (5%).

The pericardium is most often involved because of direct invasion by thoracic cancer, including breast and lung cancer.

Abdominal and pelvic tumors may reach the right atrium through the IVC. The most common tumor exhibiting this tendency is renal cell carcinoma.

In men: lung cancer followed by esophageal cancer and lymphoma

In women: lung cancer followed by lymphoma and breast cancer.

Clinical Manifestations of SCT peripheral edema are common clinical findings. Heart failure, cardiac arrhythmias, heart

blocks, acute myocardial infarction, myocardial rupture, systemic embolization, and superior vena cava (SVC) syndrome

ECG : ST-T wave changes (mimicking myocardial ischemia or injury), new atrial fibrillation or flutter, and low voltages, with electrical alternans indicating a significant pericardial effusion

COMPLICATIONS OF NEOPLASIA Pericardial Effusion Causes: malignant effusion, radiation- or drug-induced

pericarditis, idiopathic pericarditis infectious causes (TB Bacterial) iatrogenic secondary to procedures. Drug-induced pericarditis is typically seen

after high-dose anthracycline or cyclophosphamide therapy.

CARDIAC TAMPONADEUsual presentation of pericardial effusion.Immediate treatment is directed towards fluid drainage.Cytologic examination 85% of malignancy patients.Risk for recurrence is reduced by extended catheter drainage (3 ± 2 days; 11.5% recurrence) as opposed to simple pericardiocentesis.

Some have used intrapericardial instillation of chemotherapeutic agents or sclerosing agents, but it is not clear which approach is better.

Occasionally, percutaneous balloon pericardiotomy or pericardiectomy may be required, but patients with malignant effusions have such a poor prognosis

Therapy is directed at the underlying tumor

CARCINOID Tumors also can affect the cardiac

structures indirectly, as is seen in carcinoid heart disease.

Metastatic carcinoid tissue in the liver produces biologically active substances, including serotonin, which cause abnormalities of the right-sided cardiac valves and endocardium.

Typical changes include thickening, retraction, and increased rigidity of the tricuspid and pulmonic valve leaflets, resulting in valvular regurgitation or, less often, valvular stenosis.

Left-sided valvular involvement is rarely seen, possibly because of a lower concentration of the active molecules after passage through the lungs.

While metastatic carcinoid disease is rare, the echocardiographic findings are pathognomonic and may lead to the diagnosis in a patient in whom it was not considered previously.