A case report of an extremely rare and aggressive tumor: primary malignant pericardial mesothelioma

Abstract

Primary pericardial malignant mesothelioma (PMPM) is extremely rare with an incidence less than 0.0022%. It comprises 0.7% of all mesothelioma cases. To date, approximately 350 cases of pericardial mesothelioma have been reported in the literature. Its typical presentation is insidious, with nonspecific signs and symptoms, and usually results in constrictive pericarditis, cardiac tamponade and congestive heart failure either by a serous effusion or by direct tumorous constriction of the heart. With the exception of several case reports, the outcome is uniformly fatal, and patients typically die within six months of diagnosis. Here we report a 72-year-old Cauca -sian male with persistent pericardial and pleural effusion. He was diagnosed with PMPM after pericardectomy. He had only one cycle of chemotherapy with cisplatin and pemetrexed. He developed acute kidney injury as result of chemotherapy. He died 1 month after diagnosis and 6 months after the first symptoms.

Keywords: medicine; oncology; primary pericardical malignant mesothelioma..

Figure 1

Computed tomography scan of the chest (A) and (B) coronal view revealed a heterogeneous, vague soft tissue-mass-like area with rim enhancement in the pericarinal region, irregular thickening with diffuse enhancement of the parietal pericardium and a complex, loculated bilateral pleural effusion without lung parenchymal mass lesions or pleural nodules. There was a few perihilar lymphadenopathy sizing less than 1 cm (not shown). The rest of organs including liver, spleen and kidney were unremarkable (not shown).

Figure 2

Computed tomography scan of the chest (A) and (B) coronal view revealed a heterogeneous, vague soft tissue-mass-like area with rim enhancement in the pericarinal region, irregular thickening with diffuse enhancement of the parietal pericardium and a complex, loculated bilateral pleural effusion without lung parenchymal mass lesions or pleural nodules. There was a few perihilar lymphadenopathy sizing less than 1 cm (not shown). The rest of organs including liver, spleen and kidney were unremarkable (not shown).

Figure 3

A) Post-pericardiectomy specimen reveals hypercellular atypical cells with hyperchromatic nuclei and prominent nucleoli. These poorly differentiated malignant cells shows both spindled (shown here) and epithelioid morphology, characteristic for biphasic type of mesothelioma (Hematoxylin-eosin stain); B) the proliferation of atypical spindle cells infiltrates and invades fat tissue, which is regarded as typical feature of this type of malignancy. Immunohistochemically, the malignant cells are positive for calretinin (patchy); C), pancytokeratin (diffuse); D) and WT-1 (focal); E) the immunohistochemical profile is consistent with mesothelioma; F) Immunostaining with SV40, which has been suggested to be associated with mesothelioma, is negative in this case. As well, PCR with SV40 gene amplification is negative (not shown).

Figure 4

On autopsy, A) the pericardial sac was noted to be markedly thickened and expanded and encasing the heart. Both right and left ventricles were compressed by the tumor. The thickness of the pericardium was 20mm and adhered to the pleura (not shown). The heart was otherwise normal with no intramuscular tumor; B) there were set metastasis in the liver; C) There are pleomorphic spindle-shaped neoplastic cells infiltrating the pericardium, morphologically and immunohistochemically compatible with sarcomatous mesothelioma. The tumor cells had large nuclei, prominent nucleoli, and occasional mitotic figures; D) metastases were also identified in a paratracheal lymph node.