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CARDIAC TUMOURS

CARDIAC TUMOURS. Dr Rakesh K Senior Resident. INTRODUCTION. Cardiac tumors are mostly an incidental finding. Cardiac masses –Most common DDs Thrombus Vegetations Tumor is an uncommon event. Cardiac tumors Incidence. Primary Tumors of the heart are rare.

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CARDIAC TUMOURS

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  1. CARDIAC TUMOURS Dr Rakesh K Senior Resident

  2. INTRODUCTION • Cardiac tumors are mostly an incidental finding. • Cardiac masses –Most common DDs • Thrombus • Vegetations • Tumor is an uncommon event.

  3. Cardiac tumors Incidence • Primary Tumors of the heart are rare. • Prevalence of 0.001% to 0.03% in autopsy series. • Secondary Cardiac Tumors are 30 times more common. • The incidence is 0.7% to 3.5% (average- 2.3%) in the general population.

  4. Primary cardiac tumors • Benign >80% Vs Malignant < 20% • Cardiac myxomaconstitute half of them(50%).

  5. Metastatic tumors • The incidence -1.7% to 14% (average, 7.1%) in cancer patients.(General population Avg. incidence-2.3%) • Cardiac metastases can occur either by • Direct extension( Ca Lung, Ca breast) • Blood stream( melanoma) • Lymphatic( Lymphomas ) • Intra cavitary diffusion through the IVC (RCC/Uterine tumors). • Pericardial metastasis - 69% • Epicardial - 34% • Myocardial - 32% • Endocardial metastases -5%

  6. 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. • 80% of benign tumors in children are rhabdomyomas, teratomas, and fibromas.

  7. Incidence of benign cardiac tumors

  8. Incidence of primary malignant cardiac tumors

  9. CLINICAL MANIFESTATIONS • The clinical manifestations can be divided into four general mechanistic categories: • Systemic manifestations • Embolic manifestations • Cardiac manifestations • Phenomena secondary to metastatic diseases

  10. SYSTEMIC MANIFESTATIONS • Lab findings • Leukocytosis • –Polycythemia • –Anemia • –Thrombocytosis or • –Thrombocytopenia • –Hypergammaglobulinemia • –Increased ESR. • Constitutional symptoms • Fever • Fatigue • Malaise • Weight loss • Myalgia • Arthralgia • Muscle weakness • Raynaud phenomenon.

  11. These systemic manifestations are believed to be produced by secretory products released by the tumor or by tumor necrosis like IL-6. • Most commonly seen in - Cardiac Myxoma

  12. EMBOLIC PHENOMENON • Can be Systemic or Pulmonary embolism. • Tumor emboli or Thromboemboli from tumor surface. • The propensity to embolize depends • The origin of the tumor (intramural or intra cavitary) • The type of the tumor (soft/friable/pedunculated) • Associated RWMA/LVD/Thrombophilic states • Most commonly seen in Cardiac Myxoma (specially with villous surface)

  13. The brain is the most common site of emboli. • Brain emboli most commonly causes TIA or ischemic stroke but rarely ICH can be the presentation. • Coronary Artery Embolism can be seen rarely and mimicks as MI. • Pulmonary embolization is also rare, more with Right sided tumors and Left sided tumors in presence of Intracardiac L-R shunt.

  14. CARDIAC MANIFESTATIONS Mechanisms 1. Direct mechanical interference with myocardial or valvularfunction. 2. Interruption of coronary blood flow. 3. Interference with electrophysiologicconduction. 4. Pericardial effusion.

  15. 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.

  16. DIAGNOSTIC APPROACH • For diagnosis –High index of suspicion needed. • When cardiac tumor is considered in the DD, Echocardiography (TTE/TEE) has to be taken. • TEE is better due to • Proximity to heart • No interference with lung and bone • High frequency transducer with good resolution • Chest CT with contrast enhancement and CMR with contrast are superior modalities for delineation of the extent of tumor involvement/in doubtful cases.

  17. Features suggestive of malignant cardiac tumors • Large broad-based lesion occupying most of the affected cardiac chamber • Hilar lymphadenopathy • Extensive pericardial involvement • Hemorrhagic pericardial effusions. • For definite diagnosis HPR is mandatory. • Less invasive methods such as cytologic evaluation of pericardial or pleural fluids might yield diagnosis in some cases only.

  18. PET scan useful in metastatic work up. • CAG may be needed before surgery to know the feeding vessel to the mass. • More invasive methods of tumor biopsy through intravenous route, mediastinoscopy or even thoracotomy may be necessary to obtain a definitive diagnosis.

  19. ECHO EVALUATION Acardiac 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 • Localiased Hypertrophy of muscle

  20. The first step - to ensure it is not an artifact. • Appropriate transducer selection, scanning technique, and evaluation from multiple examining windows will help to distinguish artifacts from actual anatomic structures. • Experience really counts..

  21. DIFFIRENTIAL DIAGNOSIS OF MASS

  22. DIFFIRENTIAL DIAGNOSIS OF MASS

  23. The Nodes of Arantius (nodes of the semilunar cusps) are thickenings of the tunica intima layer covering the ventricular aspect of the leaflets of the aortic valve. (Shown as blue marking) Lambl’s excrescences are mobile, thin, filiform structures that occur at sites of valve closure.

  24. The clinical context in which the image was obtained is critical. • The is movement of the mass and related structures during a motion image. • Progression of an image over time may also indicate the pathologic process. • If a cardiac mass changes in size from one image to the next, suspicion of a cardiac tumor is much higher. • Knowledge regarding certain characteristic presentation.

  25. A specific type of cardiac involvement the extension of renal cell carcinoma up the inferior vena cava. • RCC appears like a “fingerlike” projection into the right atrium (RA) from the inferior vena cava. • Uterine tumors also occasionally present in this fashion.

  26. Case series of 75 cases cardiac masses 46% 27% 8% 12% attached to Valves Myxomas 41 % Thrombi 16% Fibroelastoma 13% RCC 9% 7%

  27. BENIGN TUMORS

  28. MYXOMAS • Most common type of primary cardiac tumor. • >50% of all primary tumors. • Most commonly presents in adults 30 to 50years and 65% in Women. • Arises from multipotentmesenchymal cells. • 5 to 10% myxomas are familial. • Hallmark histological feature is –Myxoma cells or Lepidic cells. • Myxoma cells can produce VEGF & IL-6.

  29. Lepidic cell

  30. Majority of the patients with myxoma will present with at least one of the classic triad of obstructive cardiac, embolic, and constitutional or systemic symtoms/signs. Obstructive Cardiac Findings • Dizziness • Dyspnea ( may be positional) • Cough • Pulmonary edema • Heart failure.

  31. On Physical examination • If LA is involved with myxoma • Loud S1 and some times S1 split occurs. • Holosystolic murmur of MR • MDM from turbulent blood flow through the mitral orifice • Occasionally a tumor “plop.” • Signs of pulmonary congestion

  32. TUMOR PLOP • The sudden bolus of blood plus tumor protruding into the ventricle causes a third sound, called a "tumor plop“. • Sound is evanescent and positional. • It occurs later than an OS but earlier than an S3. • 80-120 ms after A2. • There would be an abrupt y-descent in the left atrial pressure after tumor plop.

  33. In RA myxomas • Findings of rapidly progressive right-sided heart failure seen • Fatigue • Peripheral edema • Ascites • Hepatosplenomegaly • Elevated JVP with a prominent a wave • If PRO present- systemic hypoxia, cyanosis, clubbing, and polycythemia • SVC obstruction in large RA tumor • The diagnosis is often delayed.

  34. The obstructive cardiac findings are due to the mechanical interference of mitral valve by the tumor. • Tumor embolism • Most common cardiac tumor to embolise • Virtually to every organ, mostly to CNS. • Symptoms depend on the location of the tumor and the patency of the foramen ovale.

  35. Investigations • ECGs may show LA enlargement • Atrial arrythmias and conduction disorders are rare. • CXR shows evidence of elevated LA pressure, such as LA enlargement (1/3cases), vascular redistribution, prominent pulmonary trunk • Pulmonary edema, is found in about 1/2 of patients with LA myxoma.

  36. Intracardiac tumor calcification is a rare in LA myxomas but it is seen in most of the cases with RA myxoma. • Echocardiography is the most commonly used modality for diagnostic purposes, and TEE is the preferred echo modality. • Most cardiac myxomas appear as spherical or ovoid masses with lobular contour on CT and CMR scans.

  37. Contrast-enhanced CT reveals that most myxomas have an overall attenuation lower than that of myocardium • On CT- two thirds of myxomas are heterogeneous, whereas one third appear homogeneous.

  38. 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.

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

  40. “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)

  41. 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). • Mutations in PRKAR1A( protein kinase A 1regulatory subunit) or MYH8-myosin isoform. • HPR- PAS, Vimentin, S-100 and NSE positivity.

  42. Sporadic cardiac myxoma • Women of middle age. • Isolated lesions in the left atrial aspect of interatrial septum. • Lower recurrence rate (roughly 3%) than those mentioned previously. Carney syndrome • No age or gender predilection. • Single or multiple • Any intracardiac location • Recurrence rate 20% despite adequate surgical excision.

  43. Treatment • 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.

  44. LIPOMAS AND LIPOMATOUS HYPERTROPHY • Benign lipomatous tumor is the second most common primary neoplasm of the heart. • It can be divided into two major groups on the basis of the degree of encapsulation • Lipoma • Lipomatous hypertrophy of the atrial septum (LHAS).

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