A 37-year-old female was admitted with new onset of shortness of breath. On physical examination she was dyspnoeic, heart rate was 110 beats/min, and blood pressure was 80/50 mmHg with a pulsus paradoxus of 22 mmHg. Neck veins were distended; heart sounds were distant; no pericardial friction rub was heard; dullness was found on both lung bases. Chest X-ray revealed bilateral pleural effusions and severe cardiomegaly. On electrocardiogram (ECG), low voltage and widespread ST segment elevations were found. Echocardiography demonstrated a large pericardial effusion (Figures 1(a) and 1(b)) with collapse of the right atrium and ventricle as well as classical hemodynamic changes, typical of tamponade.
On emergent pericardiocentesis, 770 cc of hemorrhagic fluid was aspirated. Cultures of the fluid were sterile, and neither acid fast bacilli nor malignant cells were found. On chemical analysis of the pericardial fluid a low glucose level of 3 mg% (normal: 60-80 mg%), a high LDH level of 6644 mg% (normal: usually <400 mg%), and 300 eosinophils/mL (17%, no normal values but probably <1%) were found. Pharyngeal and rectal cultures were negative for enteroviruses; serological tests for cytomegalic virus, Epstein Barr virus, Q-fever, and mycoplasma were negative; rheumatoid factor, anti-nuclear antibodies, anti-neutrophil cytoplasmic antibodies, and thyroid function tests were within normal limits. Computed tomography (CT) of the chest and abdomen was normal except for pericardial and bilateral pleural effusions and left lower lobe atelectasis (Figure 1(c)). Neither pericardial nor pleural calcifications or masses were seen. Benign pericarditis was suspected and the patient was treated with ibuprofen and colchicine for 16 weeks.
Three months later, the patient had recurrent dyspnea and clinical signs of cardiac tamponade and a large pericardial effusion was found on echocardiography. On repeat pericardiocentesis 1200 cc of sterile hemorrhagic fluid was aspirated. Tests for acid fast bacilli were again negative and on cytologic examination only reactive mesothelial cells were seen. Chemical characteristics of the pericardial fluid were similar to the previous analysis.
Since the patient immigrated to Israel from Ethiopia where TB is endemic and since she had hemorrhagic pericardial effusions with very low glucose levels and in the absence of a response to anti-inflammatory drugs, TB pericarditis was highly suspected and empiric antituberculous therapy was added to colchicine and prednisone. However, pericardial fluid reaccumulated during the following 4 weeks. An exploratory thoracoscopy and a pericardial window operation were performed and a pericardial biopsy was taken which showed large polygonal tumor cells with prominent nuclei and cytoplasmic vacuoles on Hematoxylin and Eosin staining. Cytokeratin and Calretinin staining were strongly positive in tumor cells, consistent with primary malignant mesothelioma. Subtotal pericardiectomy was performed. Tumor foci were also detected on histological examination of mediastinal fat (Figures 2(a), 2(b), and 2(c)). Since the pericardium was the only tissue originating from mesothelial cells in which malignant cells were found, a diagnosis of primary malignant pericardial mesothelioma (PMPM) was made. The patient denied any known contact with asbestos.
The patient was asymptomatic for a couple of months but on followup echocardiograms progressive encasement of the heart in tumor tissue was evident. Despite chemotherapy, a year later, the patient died.