A 59-year-old male presented with a 3-month history of flu-like illness, palpitations, and atypical chest pain irradiating to the shoulders. The patient also noted also a decrease in his appetite with progressive weight loss of 4 kilos in 1 month.
1 month before admission, he had an exacerbation of his symptoms, with a new onset of cough, dyspnea increasing with inspiration, and chest pain improving when bending forward. In addition, he had some rare episodes of low-grade fever with night sweats.
The patient's past medical history revealed asthma, chronic sinusitis, and acute intoxication with paracetamol. When he first consulted his physician, he was diagnosed with pericarditis and a right pleural effusion treated with colchicine (1 mg, 1 tablet daily), and steroids (0.25 mg/lg, then 0.5 mg/kg).
An EKG showed a slow atrial fibrillation of 50 beats per minute, with low voltage QRS. A chest X-ray revealed a bilateral pleural effusion with cardio mediastinal enlargement.
A CT scan showed a moderate bilateral pleural effusion, with a pericardial thickening of 5 mm and mild pericardial effusion.
A pseudo-intrapericardial mass in the right anterior mediastinum and centered on the right coronary artery with multiple mediastinal adenopathy was also described.
This presentation encouraged the physician to search for granulomatous lesions or tuberculosis or even to think about Wegener's disease, but malignancy was also an option.
An additional abdominal ultrasound was suggestive of hepatomegaly.
An ultrasound-guided thoracentesis of the pleural effusion was done, suggesting an exudative effusion indicating malignancy, especially lymphoma.
Transthoracic echocardiography showed an effusive-constrictive pericarditis pattern. A decrease of >25% in the peak E wave velocity by pulsed wave doppler, due to a respiratory variation of the mitral inflow velocity was noticed, in addition to a visually respiratory phasic shift of the interventricular septum toward the left ventricle cavity during inspiration with an early diastolic mitral septal annulus velocity. Furthermore, an expiratory diastolic flow reversal of the hepatic vein is seen with a mild to moderate circumferential pericardial effusion. These findings encouraged the physician to perform more investigations with a transesophageal echocardiography.
A transesophageal echocardiography revealed a mild circumferential effusion with a thick pericardium. An infiltrating mass in the right atrioventricular groove involved the right atrium and the right ventricular free wall, extending to the posterior free wall of the right atrium and probably the interatrial septum. The superior vena cava appeared thick also and probably infiltrated, as for the right pulmonary artery.
A decrease in the emptying velocity <25 cm/s of the left atrial appendage with a suspicious thrombus was noticed. A pattern in favor of a primary cardiac tumor was most likely (Figures 1A-C).
MRI may be used for evaluation of cardiac masses, extracardiac structures, involvement, and characterization of masses in the differentiation of tumors from intracavitary mural thrombi. It also helps in discriminating between benign versus malignant cardiac masses, emphasizing infiltration, invasion, and other signs of metastasis; but in our case, we opted for PET scan, being the gold standard in this area. Primary cardiac lymphoma can be hypointense on T1-weighted imaging and hyperintense on T2-weighted imaging, but it can demonstrate heterogeneous signal intensity. Contrast enhancement is frequent and may be homogenous or heterogeneous.
A PET scan was done to study the extension of the tumor. A high-fatty diet with low carbohydrates 24 h before PET (no cereals, pasta, dry beans, fruits, sugar, honey, candy, desserts, starchy vegetables, alcohol, bread, rice, gravies, jams, milk, or caffeine) was initiated. The patient fasted for 6 h. There was no need to suppress the physiological myocardial uptake.
The patient was positioned ~60 min after the intravenous administration of 8.3 mCi of FDG (fluorodeoxyglucose uptake). A non-contrast CT scan was acquired from the skull base to the mid thighs. A 3D emission scan of the same area was acquired. The patient's fasting glucose level was 94 mg/dl.
The PET scan showed a cardiac mass infiltrating the right heart cavities and the interatrial septum, involving the pericardium. Diffuse metastatic mediastinal adenopathy with bilateral pleural effusion and metastatic pleural implants was observed.
Two nodules in the upper lobes with bilateral cervical adenopathy and a right supraclavicular adenopathy and a retroperitoneal adenopathy with omental thickening and peritoneal implants in the pelvis were observed. It also revealed bilateral adrenal lesions with right renal mass. These findings were suggestive of cardiac sarcoma or cardiac lymphoma of the heart (Figure 1D) (Supplementary Video 1).
For a definitive diagnosis, a percutaneous biopsy of the right supraclavicular adenopathy was performed (Figure 2). Pathology confirmed the diagnosis of a non-Hodgkin's cardiac lymphoma.
The patient was treated with high doses of dexamethasone to relieve symptoms. He developed a severe symptomatic bradycardia and long pauses suddenly. A single chamber pacemaker VVI was implanted as a backup because the patient was in atrial fibrillation and no need for atrial lead in this case.
Even with symptomatic treatments, he complained of severe shortness of breath, due to his severe bilateral pleural effusion. Bilateral pleural chest drainage was done as a salvage procedure because the patient was in severe respiratory distress. After the procedure, he experienced a rare complication: a re-expansion pulmonary edema. He was moved to the intensive care unit (ICU) and supportive therapy was given for stabilization and symptoms relief. After recovery, he complained of a new onset of diplopia. Brain CT was done to rule out cerebral metastasis with a lumbar puncture. These exams didn't reveal any abnormalities. Eight cycles of chemotherapy with the R-CHOP protocol. Rituximab, Cyclophosphamide, doxorubicin, vincristine, and prednisolone were initiated. After these cures, a new PET CT was performed.
Complete metabolic treatment response was noted with a persistent focal prostatic uptake at the right base. An echocardiography declared a complete remission. There were no more infiltrated tissues, and the right ventricle regained its function (Figure 3).
Few months later, the patient came to the hospital complaining of an external chest pain with palpation and no dyspnea or any other symptoms. On physical examination, a palpable subcutaneous thoracic mass was noticed. A total body CT scan revealed some new large left supra- and retro-clavicular, mediastinal, and pleuropericardic masses. The largest one was 6 cm. A PET scan confirmed a recurrence of his lymphoma. Three large hypermetabolic mediastinal masses with cervical, mediastinal, axillar, and hilar lymphadenopathy were observed. An invasion of the subpleura with a pulmonary parenchymal focus was also noticed.
Two subcutaneous nodules were detected in front of the right pectoral muscle with a left pleural effusion and a markedly increased uptake at any site including new site of disease (Deauville score 5). A new cycle of chemotherapy began.
Two months later, a PET scan for control was done with complete remission. Two weeks after, the patient was sent for bone marrow transplant to prevent further recurrences. The patient remained actually stable.