Timely diagnosis of primary pericardial mesothelioma

Hsiao-Chun Chen ¹ | Ming-Chon Hsiung¹* | Shihi Chang²|

Wei-Kang Kwang ³ | Tien-Ping Tsao¹ | Yung-Nien Yang ¹|

¹ Heart center, Cheng Hsin General Hospital, Taipei Taiwan.

² Plastic surgery hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing China

³ Pathology, Cheng Hsin General Hospital, Taipei Taiwan.

*Corresponding author. Tel:(886)2-28264000-2508. E-mail:mhsiung0007@gmail.com

Timely diagnosis of primary pericardial mesothelioma

We present a case of a 66-year-old male with dyspnea and bilateral lower-extremity edema. The patient was a nonsmoker and denied history of prior asbestos exposure. Chest radiography (CXR) revealed bilateral pleural effusions and moderate cardiomegaly. Transthoracic echocardiography demonstrated an irregular, thickened pericardium with heterogeneous echogenicity tumor mass concomitant with large circumferential pericardial effusion, thus ruling out infiltration pericardium. (Fig 1); upon hospitalization, Non-contrast Computed tomography (CT) scan of the chest and abdomen showed pericardial effusion encasing the heart with small bilateral pleural effusions. Pericardiocentesis with pigtail drainage was immediately performed and sent for cytology testing, and the report suspected malignant. Consequently, pericardiotomy and biopsy were performed and reported malignant mesothelioma. The micro showed pericardial tissue with epithelioid type mesothelioma. The tumor cells revealed positivity for Calretinin, GATA-3 and CK, and focal positivity for D2-40.The immunostatin for BAP-1 showed loss of nuclear positivity in the tumor cells that, coupled with morphology, accord with a primary pericardial mesothelioma diagnosis. (Fig 2) We continued with PET for further evaluation which showed increased FDG uptake of the entire pericardium, confirming the diagnosis of previous examinations. (Fig 3) Primary pericardial mesothelioma is poorly diagnosed, has unfavorable prognosis, and is extremely rare, even among heart tumors, with an incidence of <0.002% and accounting for less than 5% of all mesotheliomas. [1] Patients often show nonspecific but typical symptoms like constrictive pericarditis, cardiac tamponade, and heart failure. [2] From the limited literature, up to 75 percent of cases were diagnosed postmortem [3] and cytologic analysis of pericardial fluid were often negative [4]. Echocardiography is the most commonly used investigative tool but is low in the identification of pericardial mesotheliomas. In our case, despite the large amount of pericardial effusion of the first echocardiography, the infiltrated like pericardium was still detected. In addition, cytology after pericardiocentesis reported suspicion of malignancy, which followed by pericardiotomy and biopsy, lead to our timely diagnosis. Echocardiography after the drainage of pericardial effusion displayed a notable heterogenous echogenicity mass (Fig 4). Surgical resection could be curative for localized cases, but our patient refused surgical treatment and is currently receiving chemotherapy.

References:

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[2] Dipak P, Tiffany W, Deborah R, et al. Echocardiographic Diagnosis of Malignant Mesothelioma Involving the Pericardium.Echocardiography. 2005;Vol 22, No6:538-39.

[3] Takeda K, Ohba H, Hyodo H, et al. Pericardial mesothelioma: hyaluronic acid in pericardial fluid. American Heart Journal.1985;110:486-88.

[4] Elizabeth, McGehee, David, et al. Treatment and Outcomes of Primary Pericardial Mesothelioma: A Contemporary Review of 103 Published Cases. Clinical Lung Cancer. 2018 Mar 20(2):e152-e157.