Timely diagnosis of primary pericardial mesothelioma
Hsiao-Chun Chen 1 | Shihi
Chang2 |Ming-Chon Hsiung 1*
|
Wei-Kang Kwang 3 | Tien-Ping Tsao1 | Yung-Nien Yang 1|
Hsiao-Chun Chen and Shihi Chang contributed equally to this article.
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 (Fig 1, VIDEO_1, and VIDEO_2). We
then performed examinations to rule out diagnosis of infiltration
pericardium. Upon hospitalization, Non-contrast Computed tomography (CT)
scan of the chest and abdomen revealed a thickened pericardium with
pericardial effusion encasing the heart and a few pleura effusion.
(Fig.2). 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(GATA Binding Protein 3) and CK(Creatine Kinase),
and focal positivity for D2-40(Podoplanin). The immunostatin for
BAP-1(Breast cancer type 1 susceptibility protein associated protein-1)
showed loss of nuclear positivity in the tumor cells that, coupled with
morphology, accord with a primary pericardial mesothelioma diagnosis.
(Fig 3) We continued with PET for further evaluation which showed
increased FDG uptake of the entire pericardium, confirming the diagnosis
of previous examinations. (Fig 4) Primary pericardial mesothelioma is a
highly malignant tumor that 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 5,
VIDEO_3, and VIDEO_4). The patient passed away after two months of
chemotherapy.
VIDEO_1, and VIDEO_2
Parasternal view in Transthoracic echocardiography showing a thickened
pericardium with heterogeneous mass (arrowhead) and large pericardial
effusion. (LV: Left Vetricular, RV: Right Ventricular, Ao: Aorta, PE:
Pericardial Effusion)
VIDEO_3, and VIDEO_4
Transthoracic echocardiography presenting heterogeneous hypoechoic mass
(star key) surrounding the whole heart with septal bounce after the
drainage of pericardial effusion and pleural effusion. (LV: Left
Vetricular, RV: Right Ventricular, LA: Left Atrium, RA: Right Atrium,
Ao: Aorta )
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