Introduction :Cardiac tumours are rare entities that require due diligence during evaluation to differentiate benign from the malignant masses. Most patients remain asymptomatic in the initial stages and require investigation when the masses grow larger in size. Most of these tumours are benign in nature and cause symptoms due to their position, number, friability and attachment to vital structures. As they grow in size, they may cause compressive symptoms, shower emboli, causevalvar dysfunction or even arrhythmias. 2D echocardiography is the first modality of investigation whichprovides information about the size, number, location and attachment of the cardiac masses describing them in detail. Computer Tomography Scan or Cardiac Magnetic Resonance Imaging may be needed to ascertain diagnosis and stage malignancies. Positron Emission Tomography with FluorodeoxyGlucose may also be needed to confirm and stage the malignancies. However, a definitive diagnosis is established only after tumour excision and subjecting it for histopathological evaluation. Secondary metastasis is more common rather than primary carcinomas; secondaries arise from most commonly,the lung and breast carcinoma, melanoma, soft-tissue sarcoma, and renal cell carcinoma.
Cardiac tumors are rare occurences in cardiac clinical practice. Most of these tumors are benign, accounting for about 85-90% of the cardiac masses;of which, most commonly encountered in the operating theatres are themyxomas[1]. Most cardiac surgeons are exposed to cardiac tumors, especially myxomas,early on from their residency days; and some of these areassigned to the juniors in the department to operatein the initial years of their cardiac surgery training period. Thus, it is important to investigate these cases thoroughly to differentiate between the benign andmalignant ones. We encountered various types ofcardiac masses with interesting modes of presentation over the last decade, which we are presenting in this case series.
Case 1:
A 34 year old man presented to us with dysnoea on exertion of 3 months duration. On echocardiography, there was a mass in the left atrium measuring 4 cm x 3 cm having a wrecking ball effect on the mitral valve. There was mild mitral regurgitation. Left atrium was dilated. He underwent excision of the myxoma which was attached to the inter atrial septum by a stalk.Histopathological examinationconfirmed the same. Patient is on regular follow up with us.
Pictures of the left atrial mass -in 2D Echocardiography andafter excision.
Case 2:
An 8year old child presented with fever of 3 months duration.He was thoroughly evaluated for pyrexia ofunknown origin.ESR was raised. Also, his C-Reactive protein was positive.Three consecutive blood cultures were negative. His vitamin D3 and serum calcium levels were normal and there was nohypercoagulable state.2D echocardiography, computer tomography scan and magnetic resonance imaging revealed multiple, well-defined, ovoid, heterogenous masses varying from 1 to 5 cm in size, in the right ventricle(RV), right ventricular outflow tract(RVOT) and main pulmonary artery(MPA)with occlusion of the right lower lobar artery. On conventional CPB, branch pulmonary arteries were dissected up to hila. Right atrium and main pulmonary arterywere opened. Large masses were seen in the RV and its outflow tract measuring 5 and 3 cm respectively. Tumour emboli were seen filling the left upper, left lower, right upper and right middle lobe branch pulmonary arteries. All masses were excised. Right lower lobe branch pulmonary artery was totally occluded by the embolus and could not be removed.Histopathologicalexamination revealed acalcified amorphous tumour (CAT) with predominantly fibrinous acellular material with large areas of dystrophic calcification and focal collection of inflammatory cells admixed with proliferating fibroblasts.
2D ECHO shows multiple masses in the RVOT in the short axis PLAX view.
Excised tumour tissue on subjecting for histopathological examination revealed a Calcified Amorphous Tumour which is a rare entity.
Case 3:
A 40 year old female patient came withc/odyspnoea and occasional palpitations since 3 months. She was treated conservatively for a cerebrovascular accident with left hemiparesis a year before this.During this period, she noticed painful skin lesions in her feet and hands. After thorough evaluation for various autoimmune/immunological disorders, it was deduced to be Behcet's disease and she was started on a course of steroids.Her symptoms improved gradually.However, during evaluation, 2D echocardiography revealed a large left atrial mass. Mass was measuring 4.7cm x 3 cm with its stalk attached to the lower part of the inter atrial septum,had multiple fronds, protruding through the mitral valve into the left ventricle.This was excised in toto. Histopathological features were consistent with left atrial myxoma. Behcet's disease is a rare form of multi-systemic vasculitis that affects both arteries and veins and it can have frequent relapses. Cardinal features of Behcet's disease are uveitis, oral ulcers and genital ulcers, but it can manifest in other systems too including central nervous system, cardiovascular and gastrointestinal system.Cardiac involvement may occur in the form of intracardiac thrombus,myxomas, endocarditis, myocarditis, pericarditis, endomyocardial fibrosis, coronary arteritis, myocardial infarction, and valvular disease.
Pictures of 2D Echo showing mass in the left atrium and on excision in toto.
Case 4:
A 37 yr old female patient, post renal transplant (2021)was admitted with post transplantation pyelonephritis few months ago. Was treated with antibiotics, but developed thrombus along the right internal jugular central line. So, the central line was switched to the other side and shewas started on low molecular weight heparin (LMWH) injections. Unfortunately, she developed thrombus again on left side. LMWH injections were continued. But screening echo revealed a 2.3 cm thrombus in the right atrium.Since she didn't want any surgical intervention, she was discharged on oral anticoagulants (Acenocoumarol). On recent follow up, 3 months later, thrombus has reduced in size to 1 cm. Since patient has refused any surgical intervention, she is being continued on oral anticoagulants.
Right atrial thrombus on 2D Echocardiography
Case 5:
A 26 year old, female, school teacher presented with a vague chest pain and backache. Was evaluated thoroughly. 2D ECHO revealed a hugeright atrialmass measuring6 x 5 cm in the RA, arising from the free wall, not attached to the inter atrial septum, nor extending into SVC or IVC.On Magnetic Resonance Imaging,mass was hyperintense with a thin hypointense rim. With fat suppressionin T1W and T2W images,there was a suppressed signal within the lesion with Indian ink artifact at the rim. This was consistent with lipomaand later, was confirmed by histopathological examination. Lipomas are encapsulated, benign neoplasms typically composed of mature fat cells. The reported incidence of primary cardiac lipomas is 0.17-0.19% at
autopsy, accounting for 8.4% of all heart and pericardial primary tumors. Cardiac lipoma may involve the endocardium, myocardium, and pericardium. Cardiac adipose tissue infiltration is divided into true lipomas, lipomatous hypertrophy of the interatrial septum (LHIS), and Lipomatous infiltration. Cardiac lipomas are uncommon primary cardiac tumours.
Images of 2D Echo and MRI scan showing lipoma along with its intra-operative pictures
Case 6
30 yr old patient came with h/o giddiness, speech disturbances, fever and vomiting to OPD. He also had left hemiparesis a month prior and was on medications for the same after being evaluated by a local physician. He was also a chronic smoker and tobacco chewer. During evaluation, his MRI brainrevealed embolic shower to the brain causing acute non-hemorrhagicinfarcts involving cerebellum and cerebrum along with areas of chronic infarcts; suggesting multiple episodes of embolic shower. He was started on anticoagulants. Loss of power in the left upper and lower limb with speech deficits recovered slowly.2D ECHO was done to locate the source of emboli. It revealed two echogenic masses in the left ventricular cavity. Larger mass was attached by a long stalk to posteromedial papillary muscle body, situated about 4 cm from the mitral valve annulus. Biventricular function was normal. Cardiac MRI showed two, well defined pedunculated, enhancing, mass lesions in the left ventricle. Bilateral carotid doppler showed normal vessels. Surgery was done by trans-septal right atrialapproach. There was a cluster of 4-5 small masses; each mass was attached by a separate, small, thin flimsy stalk to the posteromedial papillary muscle. All the masses were excised. Mitral valve was normal and competent on saline insufflation test. Routine chest closure done. Histopathological examination revealed a calcified amorphous tumour.
2D ECHO and MRI images showing the location of the tumour. Histopathology of the excised masses was consistent with calcified amorphous tumour of the heart.
Cardiac tumours are broadly to classified as benign and neoplastic tumours. Neoplastic tumours can be primary or secondary tumours. Secondary cardiac tumors are more often metastatic lesions from carcinomas arising from lung, breast, melanoma, soft tissue sarcoma and renal carcinoma. Secondary cardiac tumours are 20 to 30 times more common than the primary cardiac tumours, comprising an average of 2.3% cases in the general population [2,3]. Secondary involvement may occur by metastasis which may be a contiguous spread or a spread through blood stream, lymphatics or intracavitary spread. Pericardial metastases are the most common, accounting for 70% of the secondary tumours; followed by epicardial and myocardial involvement which constitute about 25% of the total seconadary metastases[4]. Lymphatic spread causes pericardial metastases whereas hematogenous spread results in myocardial involvement [5]. Multimodal imaging of these tumours using 2D ECHO, Cardiac Computed Tomography or Magnetic Resonance Imaging and FDG-Positron Emission Tomography helps in defining the masses, differentiating the benign from the malignant ones, staging the malignancies and planning further treatment. Most intra-cavitary tumours can be excised. Malignancies and secondaries are treated by multidisciplinary approach involving surgery, chemotherapy, radiotherapy, radiofrequency ablation and immunotherapy as deemed appropriate for each type of tumour [6].
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