Syncope and Cardiac Tamponade: Multimodality Imaging of Primary Cardiac Lymphoma.

Primary cardiac lymphoma (PCL) is among the rarest heart neoplasms. Its estimated incidence is about 1%-2% among primary cardiac tumor and 0.5% of extranodal lymphoma. It usually causes heart failure, pericardial effusion, tamponade, and arrhythmias. Prognosis is poor; treatment is combined medical and surgical. We described the case of a 62-year-old male with PLC that presented with syncope and cardiac tamponade, submitted to R-CHOP therapy because of failure of surgery. Clinical state is stable 3 months after diagnosis and first chemotherapy cycle. Copyright:

INTRODUCTION

Primary malignant cardiac neoplasms are extremely rare, among them primary cardiac lymphoma (PCL) constitutes a rare entity, especially in not immunocompromised patients. Clinical presentation is heterogeneous, and it is generally related to location, size, and infiltration. Symptoms include dyspnea, heart failure, pericardial effusion, superior vena cava syndrome, arrhythmias, and shock due to cardiac tamponade or blood flow obstruction. The presumption diagnosis relies heavily on the use of multiple imaging techniques, including echocardiography (TTE), cardiac computed tomography, and cardiac magnetic resonance. We present a case of PCL showed up with syncope and cardiac tamponade.

CASE REPORT

A 62-year-old male patient, without any cardiovascular risk factor, started complaining persistent cough. Two weeks later, the patient complained of dyspnea, hypotension, and recurrence syncope. On admission, he had dyspnea, hypotension, and facial edema. Blood pressure was 90/55 mmHg, heart rate 107/min, and arterial blood oxygen saturation 93% in room air. He had muffled heart sounds at cardiac auscultation; the ECG showed sinus tachycardia without any repolarization abnormalities. TTE depicted normal biventricular dimensions and function, moderate circumferential pericardial effusion (18 mm), and dilated and immovable inferior vena cava; at pw-Doppler, signs of tamponade were present. In the right chamber there was an infiltrating mass, not well defined because of the pericardial effusion [Figure 1a]. Urgent TTE -guided pericardiocentesis with subcostal approach was made; 950 ml of serosanguinous liquid had been drained with clinical benefit. The next transthoracic TTE revealed a bulky infiltrating mass developing on the lateral wall of the right atria with irregular echogenicity, infiltration of the interventricular groove, the right ventricle, and partially of the right outflow tract; it had movable and jagged ends [Figure 1b]. The right coronary artery seemed traversing the tumor [Figure 1c]. In the next days, chest Computed Tomography (TC) confirmed the mass sized 70 mm × 50 mm × 75 mm, located along right cardiac chambers, infiltrating right ventricular outflow tract and even the aortic root with encasement of the right coronary artery [Figure 2]. It also revealed pulmonary embolism of the right lobar branches requiring anticoagulation. Serological analyzes for Cytomegalovirus, Epstein–Barr virus, C hepatitis virus, and HIV were negative; cytological evaluation of the pericardial drainage showed activated mesothelial cells. The case was discussed in heart team, and the patient underwent surgical biopsy. At the operating field, there was a big bleeding purple mass extended to the anterior and the inferior wall of the right ventricle. Extemporaneous histological examination revealed poorly differentiated cancer. Because of the high risk of hemodynamic impairment due to infiltration of the myocardial tissue and of the nearby structures such as the right coronary artery, the mass could not surgically removed. The definitive histopathological–immunohistochemical examination highlighted large atypical lymphoid cells CD20 positive, while the proliferative index Ki67/Mib 1 was 70%, compatible with cardiac high-grade B cell lymphoma [Figure 3]. For the definitive diagnosis of primitive cardiac tumor versus metastases, a 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) was performed and revealed intense uptake of the cardiac mass with no other areas of hypermetabolism [Figure 4]. R-CHOP chemotherapy was started; 3 months later, cardiac magnetic resonance and 18F-FDG PET demonstrated a reduction in tumor size [Figure 5]. The patient is still alive and he is keeping on his chemotherapy treatment and imaging follow-up.

Figure 1

Transthoracic echocardiography: Four-chamber view shows moderate pericardial effusion and a mass along the right ventricle (asterisk in a); after pericardiocentesis, the mass is better defined (asterisk in b); at parasternal short-axis view right coronary seems to be enclosed in the mass (c)

Figure 2

Chest computed tomography shows heterogeneously enhancing infiltrating mass (*) with pleural (white circle) and pericardial (arrows) effusion (a), encasement of the right coronary artery (arrow, b)

Figure 3

Proliferation of atypical lymphoid cells in H and E, ×40 (a), CD20 positivity, ×40 (b), and high Ki67 proliferative index, ×40 (c)

Figure 4

Total body 18F-fluorodeoxyglucose positron emission tomography (coronal section) shows only cardiac uptake

Figure 5

Cardiac magnetic resonance at Steady state free precession (SSFP) sequences (*a) and 18F-fluorodeoxyglucose positron emission tomography (coronal section) show a reduction in tumor size (*b)

DISCUSSION

Primary malignant cardiac neoplasms are rare, and among these, PLC represents an extremely slight minority, especially in not immunocompromised patients.[12] While the incidence of cardiac involvement by systemic lymphoma identified by autopsy varies widely ranging from 9% to 24%, PCL represents just 1%–2% of resected cardiac tumors and the 0.5% of the extranodal lymphoma. Clinical presentation is heterogeneous.[34] The heart chamber most frequently involved, as reported by Petrich et al., is the right atrium and pericardial effusion with cardiac tamponade is frequent (in 58% of patients, and among them, 34% had a tamponade physiology). In their study cohort, the most frequent clinical feature was dyspnea (in 64% of patients at the beginning), followed by constitutional symptoms and chest pain, whereas syncope without arrhythmias is not mentioned.[5] Because they are rapidly fatal if left untreated, they must be early distinguished from other primary malignant cardiac tumors. Multimodality imaging allows clinicians to make a presumption diagnosis and to differentiate them from other more common cardiac malignant tumors, such as angiosarcoma.[6] TTE is the first-line evaluation; lymphomatous involvement may appear as hypoechoic mass infiltrating the right heart, often with a pericardial effusion. Cardiac computed tomography and cardiovascular magnetic resonance are useful for further mass details such as size, location, extent, and tissue characterization; 18-FDG PET-TC confirms cardiac isolation and contributes to establishing diagnosis and treatment response.[7] This tumor, as in our case, frequently extends along the epicardial surfaces and tends to encase adjacent structures, including coronary arteries and the aortic root.[8] In a French case series of 13 patients with PLC, endomyocardial biopsy was made for the diagnosis just in two cases, while they choose the surgical approach in the others.[9] We preferred the surgical approach as well, after a heart team discussion, for a diagnostic and therapeutic attempt. Treatment is combined medical and surgical. Chemotherapy should always be considered; R-CHOP therapy is the treatment of choice with a reduced dosage to prevent complications such as heart rupture, thromboembolism, and arrhythmias.[1011] Literature includes data about remission in the 60% of the cases.[12] Surgery is first-line treatment for tumor debulking, especially when the tumor results in hemodynamic impairment.[13] Prognosis is unfavorable, and it is related to the presence of left heart involvement, extracardiac location, the immune system status, and diagnosis delay.[14]

CONCLUSION

PCL is a primary, very rare, malignant cardiac neoplasm. Multiple imaging techniques allow an accurate diagnosis and lead to the appropriate clinical management.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.