Primary malignant pericardial mesothelioma (PMPM) is an extremely rare malignant tumor, and it is difficult to diagnose definitively before death. We present a case in which PMPM was diagnosed at autopsy. We consider this case to be highly suggestive and report it here.

A 78-year-old male presented with transient loss of consciousness and falls. The transient loss of consciousness was considered to result from complications of diastolic dysfunction due to pericardial disease, fever with dehydration, and paroxysmal atrial fibrillation. Ultrasound cardiography (UCG) and computed tomography showed cardiac enlargement and high-density pericardial effusion. We considered pericardial disease to be the main pathogenesis of this case. Cardiac magnetic resonance imaging and gadolinium contrast-enhanced T1-weighted images showed thick staining inside and outside the pericardium. Pericardial biopsy was considered to establish a definitive diagnosis, but the patient and his family refused further treatment and examinations, and the patient was followed conservatively. We noticed a thickening of the pericardium and massive changes in the pericardium on UCG over time. We performed an autopsy 60 h after the patient died of pneumonia. Giemsa staining of the autopsy tissue showed an epithelial-like arrangement in the pericardial tumor, and immunostaining showed positive and negative factors for the diagnosis of PMPM. Based on these findings, the final diagnosis of PMPM was made.

PMPM has a poor prognosis, and early diagnosis and treatment are important. The temporal echocardiographic findings may provide a clue for the diagnosis of PMPM.

Diffuse primary malignant pericardial mesothelioma (PMPM) is an extremely rare and highly invasive tumor of pericardium. The tumor can infiltrate myocardium in part of cases, and will contribute to grave prognosis. Herein, we reported a 58-year-old man of diffuse PMPM with myocardial involvement, and summarized 39 cases of diffuse PMPM. Multimodal echocardiography, which combined conventional, tissue Doppler and speckle tracking echocardiography, was applied to diagnose diffuse PMPM with myocardial involvement, and assess the myocardial function. The common features were pericardial effusion, pericardial masses and thickened pericardium. The other echocardiographyic characteristics were extensive and heterogenous echo, adhesion with pericardium and myocardium, reduced motion of adhered myocardium, and constriction performance. Diffuse PMPM with myocardial involvement revealed decreased LV diastolic function, and decreased LV and RV systolic function. Especially in systolic function, the unique strain features were reduced longitudinal strains from segmental to global and from epicardial to endocardial, relative 'septal sparing' pattern, and decreased transmural gradient of longitudinal strain. Our findings suggested that multimodal echocardiography not only can identify this disease, but also can provide detailed information of myocardial dysfunction, which provides a reference for clinicians to develop an optimal individualized treatment.

Primary malignant pericardial mesothelioma is a rare tumor that is very difficult to diagnose. Furthermore, it is a lethal disease, because patients usually have progressed at the time of referral.

We report a 44-year-old man with primary malignant pericardial mesothelioma. He was referred to our hospital for the diagnosis and treatment of a massive pericardial effusion and huge tumor. Pericardiocentesis was performed, but we could not obtain definitive diagnosis, and the cardiac tamponade continued along with the signs/symptoms. He required surgical intervention for the diagnosis and treatment. After surgery, his signs/symptoms improved. He received adjuvant therapy, although he died 7 months after surgery.

Primary malignant pericardial mesothelioma is a rare tumor. The most common signs and symptoms are related to constriction of the heart by the tumor and/or effusion. Even if the pericardial fluid specimen obtained by pericardiocentesis is negative for malignant cells, primary malignant pericardial mesothelioma should be included in the differential diagnosis. Because the malignancy is usually advanced at the time of diagnosis, it has been difficult to cure. Radiation and chemotherapy have been used in addition to surgery, but have been minimally effective.

The outcome of our patient with pericardial malignant mesothelioma was dismal. The indications for surgical intervention should be carefully considered except for critical cases requiring alleviation of immediate life-threating conditions.

Malignant mesothelioma most commonly arises in the pleura and peritoneum but also occurs rarely at other anatomical sites with mesothelial tissue, namely, the pericardium and tunica vaginalis testis (TVT). This review provides a better understanding of the epidemiology of mesothelioma of these extrapleural sites.

We conducted a systematic review of the epidemiologic and clinical literature on pericardial mesothelioma and mesothelioma of the TVT. We also analyzed U.S. Surveillance, Epidemiology, and End Results cancer registry data to describe incidence patterns of these malignancies.

An etiologic role of asbestos exposure has been hypothesized for pericardial and TVT mesotheliomas, but no analytical case-control epidemiologic studies exist to test this relationship. A substantial proportion of cases with these malignancies report no known asbestos exposure. In large occupational cohorts with heavy asbestos exposures, no cases of pericardial or TVT mesothelioma have been reported. Trends in the incidence of these malignancies do not match those of pleural mesothelioma, which correspond to historical trends of commercial asbestos use. A male preponderance of pericardial mesothelioma is not evident.

In the absence of analytic epidemiologic studies, the etiologic role of environmental risk factors for mesothelioma of the pericardium and TVT remains elusive.

Malignant mesothelioma is a rare but aggressive tumor, with a high misdiagnosis rate and overall bleak prognosis. In 0.7% of all cases, the origin is the pericardium.

The present study is a review of the literature published in recent decades focusing on the advances in clinical manifestations, radiological findings, diagnosis, differential diagnosis, and treatment of malignant pericardial mesothelioma (MPM).

No clear relationship has been established between the etiologies and the development of MPM. Clinical symptoms and signs are nonspecific when present. The main presentations are chest pain and dyspnea. Imaging plays an important role in the detection, characterization, staging, and posttreatment follow-up. The definitive diagnosis is made on the basis of pathological findings. Chest radiography and echocardiography are common techniques used initially, but their roles are limited. Computed tomography and magnetic resonance imaging have an advantage in depicting the thickened pericardium, mediastinal lymph node, tumor, and the extension of adjacent structures. Surgery is the most important treatment modality and remains palliative in most cases, while the roles of chemo- and radiotherapy are unsatisfactory.

Clinical trials of malignant pleural and peritoneal mesothelioma remain important for MPM management. Multimodality treatment of surgery, chemotherapy, radiotherapy, and immunotherapy is expected to have a role in the treatment of MPM.

Primary pericardial malignant mesothelioma is an extremely rare tumor even among all mesotheliomas with about 350 cases reported in the literature so far. Typically, it has an insidious presentation, with nonspecific signs and symptoms, and usually results in constrictive pericarditis, cardiac tamponade or congestive heart failure through either a massive effusion or direct tumurous constriction or invasion to the heart. With the exception of several case reports, the outcome is uniformly dismal and patients typically die within six months of diagnosis. We report a 24 year old male with long history of pleuretic chest paint and admissions with a diagnosis of idiopathic pericarditis, eventually presenting with increasing symptoms of heart failure and a large mobile ball like mass in the heart at echocardiographic and computed tomography studies. At operation, an atypical invasive cardiac tumor was discovered. Complete resection of the tumor was impossible and the patient died from progression of the disease 4 months later.

Primary mesothelioma of the pericardium is a rare tumor and carries a dismal prognosis. This case report presents a 38-year-old man who suffered from recurrent pericardial fluid. Initial symptoms were unspecific, with dry cough and progressing fatigue. Pericardiocentesis was performed, but analyses for malignant cells and tuberculosis were negative. After recurrence a pericardiectomy was planned. At operation, partial resection of tumor tissue surrounding the heart was performed. Histopathologic examination including immunohistochemical staining for calretinin showed a biphasic mesothelioma. During the postoperative period the patient's condition ameliorated, but symptoms recurred and the patient died 3 months after diagnosis and 15 months after the first symptoms. At autopsy, the pericardium was transformed by the tumor that also expanded into the mediastinum and had set metastases to the liver. A review of 29 cases presented in the recent literature indicates a higher incidence of malignant pericardial mesothelioma among men than women. Median age was 46 (range, 19-76) years. In pleural mesotheliomas, exposure to asbestos is a known risk factor. However, in primary pericardial mesotheliomas the evidence for asbestos as an etiologic factor seems to be less convincing (3 exposed among 14 cases). Symptoms are often unspecific and cytologic examination of pericardial fluid is seldom conclusive (malignant cells demonstrated in 4/17 cases). Partial resection of the tumor can give a period of symptom reduction. Only a few patients have been treated with chemotherapy. Median survival of patients with pericardial mesotheliomas is approximately 6 months.

Ischemic hepatitis, otherwise known as "shock liver", is characterized by a massive, but transient increase in serum transaminase levels, usually associated with cardiac failure. A patient who did not have a predisposition to hypoglycemia was discovered at home with disturbed consciousness caused by hypoglycemia. She had been diagnosed as having constrictive pericarditis several years earlier and had developed ischemic hepatitis. Though the high serum transaminase levels were rapidly normalized, severe jaundice gradually developed and the patient finally died of multiple organ failure. Hypoglycemia, which is considered secondary to reduced gluconeogenesis in the exhausted liver, is a rare complication of constrictive pericarditis.

A 31-year-old woman presented with a 3-month history of progressing fatigue and effort dyspnea. Echocardiography depicted a tumor within the free wall of the right ventricle and right atrium, located on both sides of the tricuspid annulus. Computed tomography showed disseminated circular shadows sized up to 7 mm--most likely metastatic lesions--in both lungs. The diagnosis of low-grade mesothelioma bifasicum was confirmed with histopathologic and immunohistochemical studies of the samples taken by thoracoscopy from parietal pleura, lung tumor, and diaphragm region. Chemotherapy, which included gemcitabine and carboplatin, resulted in transient improvement of the clinical status of the patient and reduction of the tumor mass lasting several months followed by progression of the disease. Significant amounts of pleural fluid and huge tumors within both pleural cavities emerged. The patient died due to respiratory and circulatory insufficiency 11 months following the diagnosis.

Pericardial mesothelioma is a rare cancer for which treatment options are limited. Operative intervention in pericardial mesothelioma is primarily for effusion control, for cytoreduction before multimodal therapy, or to deliver and monitor innovative intrapericardial therapies. Misdiagnosis is common. Early detection of the disease is the only hope for survival. Echocardiography, pathologic examination of pericardial fluid and pericardial biopsy, Gallium-67 scintigraph, Ber-EP4 antibody, and immunohistochemical procedures can be used. Magnetic resonance imaging is emerging as the best modality for demonstrating the nature and extent of the constrictive process, and the infiltration to the cardiac wall and great vessels. Failure of surgical techniques is usually associated with mesothelioma with entrapped heart, a large solid tumor mass, and a long history of pericardial effusion. If the tumor is localized, resection is the only hope for this rare, but lethal, entity. No single treatment modality is efficient by itself. The exact role of intracavitary chemotherapy or irradiation remains to be defined. Preliminary clinical application of photodynamic therapy and attempts at inhibiting the effects of growth factors, such as vascular endothelial growth factor and platelet-derived growth factor, and vaccine treatments are being explored. Adenoviral molecular chemotherapy recently completed phase I testing. Clinical trials for pleural mesothelioma remain important as clinicians seek to improve the outcome for patients with pericardial mesothelioma. Early diagnosis and multidisciplinary patient care is essential for improved surgical outcome. In the future, combined therapeutic strategies involving radical surgery, radiotherapy, adjuvant chemotherapy, and immunomodulation may have a role in the treatment of pericardial mesotheliomas.

A previously healthy 57-year-old woman with peripheral edema and exertional dyspnea had diminished right breath sounds and edema of both legs. Chest radiography showed massive right pleural effusion, and abdominal computed tomography showed ascites. During cardiac catheterization, pressure curves of both ventricles showed "dip-and-plateau" patterns. We diagnosed constrictive pericarditis and conducted pericardiectomy. During surgery, we found a thick fibrous pericardium surrounding the entire heart and a band of calcium in the atrioventricular groove. Histological examination of excised pericardial tissue showed fibrosis, hyalinization, and calcification, with thickening of < or = 18 mm. Cases of localized pericardial thickening, including constricting bands in the atrioventricular groove, are rare and many such complications occur postoperatively. We report a rare case of primary constrictive pericardial band resulting in massive right pleural effusion and ascites.