Pericardial Mesotheliomas

Abstract

Primary pericardial mesothelioma (PM) is a rare tumor arising from the mesothelial cells of the pericardium. It has an incidence of <0.05% and comprises <2% of all mesotheliomas; however, it is the most common primary malignancy of the pericardium. PM should be distinguished from secondary involvement by the spread of pleural mesothelioma or metastases, which are more common. Although data are controversial, the association between asbestos exposure and PM is less documented than that with other mesotheliomas. Late clinical presentation is common. Symptoms may be nonspecific but are usually related to pericardial constriction or cardiac tamponade, and diagnosis can be challenging usually requiring multiple imaging modalities. Echocardiography, computed tomography, and cardiac magnetic resonance demonstrate heterogeneously enhancing thickened pericardium, usually encasing the heart, with findings of constrictive physiology. Tissue sampling is essential for diagnosis. Histologically, similar to mesotheliomas elsewhere in the body, PM is classified as epithelioid, sarcomatoid, or biphasic, with the biphasic type being the most common. Combined with morphologic assessment, the use of immunohistochemistry and other ancillary studies is helpful for distinguishing mesotheliomas from benign proliferative processes and other neoplastic processes. The prognosis of PM is poor with about 22% 1-year survival. Unfortunately, the rarity of PM poses limitations for comprehensive and prospective studies to gain further insight into the pathobiology, diagnosis, and treatment of PM.

Case Report: Early diagnosis and bevacizumab-based chemotherapy for primary pericardial mesothelioma: a case with occupational asbestos exposure history

“…This choice was made based on the patient's preference for palliative chemotherapy over surgical intervention following a full discussion of treatment plans. Due to its rarity, there is no consensus on the treatment of PPM ( 1 , 2 , 5 , 6 ). Currently, treatment options for PPM are adapted from the more often studied diffuse pleural mesotheliomas ( 1 , 2 , 18 ); surgery is the most widely-used approach, followed by chemotherapy ( 5 , 6 , 18 ).…”

“…Due to its rarity, there is no consensus on the treatment of PPM ( 1 , 2 , 5 , 6 ). Currently, treatment options for PPM are adapted from the more often studied diffuse pleural mesotheliomas ( 1 , 2 , 18 ); surgery is the most widely-used approach, followed by chemotherapy ( 5 , 6 , 18 ). However, in a review of 103 published PPM cases, it was found that chemotherapy, but not surgery, provided a statistically significant survival benefit.…”

“…The diagnosis of PPM remains challenging, which leads to the situation that the diagnosis is usually made after surgery or at autopsy ( 2 , 10 , 15 , 18 , 21 ). The delayed diagnosis of PPM may be due to its nonspecific syndromes, its similar presentation to benign pericardial diseases, and its non-definitive etiology ( 1 , 6 – 8 , 10 – 15 ). Our patient also initially had nonspecific symptoms and inconclusive image findings.…”

“…Primary pericardial mesothelioma (PPM) is an exceedingly rare malignant cancer accounting for only 0.7% of all malignant mesotheliomas, with an annual standardized incidence rate of approximately 0.36 per 10 million person-years (1)(2)(3)(4)(5). PPM has a poor prognosis with a median survival of less than six months (1,(4)(5)(6), which has been partly attributed to its frequently delayed diagnosis (6)(7)(8). In many PPM cases, the time from presentation to definite diagnosis may last for several months (4)(5)(6)(7) or even over one year (6,(9)(10)(11)(12)(13)(14).…”

“…In many PPM cases, the time from presentation to definite diagnosis may last for several months (4)(5)(6)(7) or even over one year (6,(9)(10)(11)(12)(13)(14). This slow recognition is likely due to its nonspecific syndromes, its similar presentation to benign pericardial diseases, and its non-definitive etiology (1,(6)(7)(8)(10)(11)(12)(13)(14)(15). It has been suggested that early detection of this disease is the only hope for survival (2).…”