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Pericardial mesothelioma (PM) accounts for 0.7% of all malignant mesotheliomas. Although asbestos exposure is a recognized etiological factor for pleural and peritoneal mesotheliomas, its role in the development of PM is controversial. The aim of this study is to describe the characteristics of PM cases occurred in Lombardy, a highly industrialized Region of Northern Italy. From the Lombardy Mesothelioma Registry we selected the incident cases of PM registered in the Lombardy Region between 2000 and 2009 and we abstracted clinical characteristics and history of asbestos exposure. We identified 8 cases (6 men and 2 women), with a median age at diagnosis of 55.5 years, representing 0.3% of all mesothelioma cases (n = 3059). The age-standardized incidence rate was 0.09 per million/year. Occupational exposure to asbestos was documented in 5 of the 7 cases for which we obtained an interview. Our findings support the role of asbestos in the pathogenesis of PM., (Copyright © 2010 Elsevier GmbH. All rights reserved.)

Objectives: The epidemiology of extrapleural malignant mesothelioma is rarely discussed and the risk of misdiagnosis and the very low incidence complicate the picture. This study presents data on extrapleural malignant mesothelioma from the Italian National Mesothelioma Register (ReNaM)., Methods: ReNaM works on a regional basis, searching for cases and interviewing subjects to investigate asbestos exposure. Classification and code criteria for certainty of diagnosis and exposure modalities are set by national guidelines. Between 1993 and 2004, 681 cases were collected. Incidence measures and exposure data refer to the ReNaM database. Age-standardised rates were estimated by the direct method using the Italian resident population in 2001. Correlations between the incidence of pleural and non-pleural malignant mesothelioma for the 103 Italian provinces were analysed., Results: Standardised incidence rates (Italy, 2004, per million inhabitants) were 2.1 and 1.2 cases for the peritoneal site (in men and women, respectively), 0.2 cases for the tunica vaginalis testis, and 0.1 in the pericardial site, varying widely in different parts of the country. Mean age at diagnosis for all extrapleural malignant mesothelioma cases was 64.4 years and the men/women ratio was 1.57:1. Median latency was over 40 years for all extrapleural sites combined. The correlation between pleural and peritoneal mesothelioma was 0.71 (Pearson's r coefficient, p<0.001). Modalities of exposure to asbestos fibres were investigated for 392 cases., Conclusions: The rarity of the disease, the low specificity of diagnosis and difficulties in identifying the modalities of asbestos exposure call for caution in discussing aetiological factors other than asbestos.

We report a case of a malignant pericardial mesothelioma of the epithelioid type in a 39-year-old man. He had a history of nodular sclerosing Hodgkin's disease treated with irradiation of the cervical and mediastinal regions 24 years before, and of infarction of the anterior wall of the left ventricle, after which a percutaneous coronary intervention was carried out 7 years previously. He was admitted to a cardiology unit with progressive dyspnea. On examination, a hemorrhagic pericardial fluid collection of 600 ml was detected which was successfully drained. On the next day, the patient developed an electromechanical dissociation suggesting a pericardial tamponade, which was followed by circulatory arrest. At autopsy, the pericardial sac was found to contain 300 ml of partly clotted blood. The epicardial surface showed a diffuse thickening, suggesting a chronic fibrous pericarditis without a macroscopically evident distinct tumor mass. A rupture measuring 0.4 cm in diameter was detected in the right ventricular free wall, 1 cm below the level of the tricuspid valve. The diagnosis of a diffusely growing, malignant mesothelioma of the epithelioid type was made on the basis of histological and immunohistochemical examination of the thickened pericardium., (Copyright © 2010 Elsevier Inc. All rights reserved.)

We present a case of primary pericardial mesothelioma occurring in an asbestos-exposed 67-year-old man who underwent four aortocoronary bypass grafting seven years prior to the onset of the mesothelioma. Primary pericardial mesothelioma is a rare tumor whose association with asbestos is more infrequent than that of the much more common pleural form. Factors other than asbestos that may play a role include genetic predisposition, immune impairment, infections, radiation, dietary factors, and recurrent serosal inflammation. We consider that, in the presented case, inflammation and healing resulting from pericardiotomy might have had a synergistic effect with asbestos in the pathogenesis of the tumor. To our knowledge, this is the first reported case of primary pericardial mesothelioma arising in a patient exposed to asbestos who previously underwent cardiac surgery.

Malignant pericardial mesothelioma (MPeM) is a rare, primary pericardial tumor of mesodermal-origin. With respect to the etiology of MPeM, a history of exposure to asbestos has not been clearly demonstrated. MPeM is difficult to diagnose because of the non-specificity of the clinical complaints and symptoms. A known effective treatment does not exist and the prognosis is poor. In this case study, the possible etiologies of MPeM are discussed based on the extant literature. We report herein a patient with MPeM and no history of asbestos exposure who had chemo-radiotherapy for non-Hodgkin's lymphoma, and in whom a tumor spread from the pericardium through the pleura.

Pericardial diseases can be difficult to differentiate from myocardial conditions. Diagnosis can be challenging and often requires the use of different imaging modalities. Here, we describe a case which presented with common cardiac symptoms which were shown to be the result of a rare condition. A 62-year-old lady presented with left femoral artery embolism. Post-embolectomy she developed cardiac failure. Three months previously an acellular, sterile pericardial effusion had been drained. In 1993 a left mastectomy and axillary node clearance was performed for breast cancer. Adjuvant chemotherapy and radiotherapy were administered. Examination revealed a raised jugular venous pressure (JVP) with rapid Y descent and Kussmaul's sign. CT chest and abdomen found no recurrence of breast carcinoma. Cardiac MRI demonstrated thickened pericardium. At cardiac catheterisation haemodynamic responses consistent with constrictive pericarditis were seen. Pericardiectomy was performed. Histology revealed pericardial epithelioid malignant mesothelioma. 18-FDG-PET CT post-operatively was negative in the pericardium and pleura. Chemotherapy with pemetrexed and carboplatin was given. The patient died 9 months after presentation. Radiotherapy and asbestos exposure are both associated with pericardial mesothelioma and the aetiology in this case was not clear. The condition carries a poor prognosis and is invariable fatal although newer chemotherapeutic regimens have prolonged survival times.

An autopsy case of a primary pericardial mesothelioma in a 53-year-old arc cutter is reported. He had often had the chance to inhale dust generated by sharpening the slate pencils composed of talc. He was admitted for heart failure due to pericardial tumor, but later died. The tumor was mainly located on the pericardium with a thickness of about 2.5 cm. Small nodular disseminations were observed in the left parietal pleura. Microscopically, tumor cells were epithelial-like and rich in histochemical demonstrable hyaluronic acid. Findings of immunohistochemical markers revealed keratin (+), EMA (+), calretinin (+), and CEA (-), which were characteristics of mesothelioma of epithelial type. The number of asbestos bodies (AB) in the lung parenchyma was increased (2026 AB/gram dry lung tissue). Subsequent transmission electron microscopic examination equipped with an energy dispersive X-ray analyzer revealed that the fibers identified in the lungs were fibrous talc and actinolite. These findings suggested that this patient had been occupationally exposed to asbestos contaminated in the talc pencils, which induced the development of primary pericardial mesothelioma.

Primary pericardial malignant mesothelioma is rare tumor and its prognosis is quite poor due to its late presentation and difficulty of complete resection. We describe a rare case of primary pericardial malignant mesothelioma in a 46-year-old female with incomplete endocardial cushion defect (ECD). Incidentally, we found 2 masses in the pericardial spaces after pericardiectomy. We have successfully removed these 2 masses en bloc and performed a total repair of incomplete ECD. Pathologically, these masses were a primary pericardial malignant mesothelioma. This patient is doing well 4 months after surgery without any evidence of recurrence despite reported poor prognosis of "primary pericardial malignant mesothelioma".

The incidence of primary malignant neoplasms of the pleura, the pericardium and the peritoneum in Germany has been rising since about the mid-1980s. A continuing rise is expected until about 2020, predominantly due to the peak of asbestos processing in Germany between 1965 and 1980. About 90% of the mesotheliomas stored in the files of the German Mesothelioma Registry in Bochum are asbestos related and therefore possibly due to occupational exposure that may be compensated by the professional associations. More than 500 mesotheliomas annually can be diagnosed in the German Mesothelioma Registry. In this first part of the series on mesotheliomas, current concepts on etiology and pathogenesis as well as diagnostic procedures and standards are discussed. At the present, specific chromosomal or genetic defects that constantly give rise to a mesothelioma are not known. The initiation and progression of malignant mesotheliomas is a highly complex mechanism that is based on individual genetic alterations. A reliable diagnosis is the basis for therapeutic, prognostic and medicolegal consequences; in general, it can be achieved by thoracoscopic inspection with selected biopsy. Surgery may gain a more important role in the therapy of malignant tumors of serosal membranes not only in palliative, but also in potential curative approaches if the diagnosis can be made at earlier tumor stages.

Malignant mesothelioma of the peritoneum, pericardium and tunica vaginalis is a rare neoplasm usually associated with exposure to asbestos. The disease is most common in males over the age 40 years with sings and symptoms of neoplastic disease with or without a palpable abdominal mass, abdominal pain and ascites. Diagnosis is often possibly only after direct vision and histologic examination. Different types of peritoneal mesothelioma occur in adults and in children. Only small proportion of all patients with mesothelioma can be cured surgically. Both radiotherapy and standard chemotherapy have only limited usefulness. The optimal management malignant mesothelioma continues to be defined.

The analysis of 335 cases of mesothelioma observed at the Ramazzini Foundation and the Bologna Institute of Oncology has shown: 1) a high percentage of correlation of these tumours with asbestos exposure; 2) a large number of population categories potentially exposed to asbestos fibres and therefore at risk of developing mesothelioma; and 3) the high risk of mesothelioma among people exposed in various circumstances to asbestos used in railroads and sugar refinery plants.

The diagnosis of malignant mesothelioma can pose several problems to the surgical pathologist. First, the morphological appearances of the tumour are known to be diverse with mimicry of a range of both reactive and neoplastic conditions. Second, due to the relative inaccessibility of the serosa, biopsy material is often scanty and fragmentary, producing a plethora of interpretive ambiguities. Third, adjunct techniques such as mucin histochemistry and immunohistochemistry, whilst useful in excluding malignant mesothelioma have little role in confirming the diagnosis. The accurate diagnosis of diffuse malignant mesothelioma is important for two reasons: (1) In relation to prognosis as it has an almost invariable fatal outcome, which contrasts with the other mesothelial neoplasms such as the benign adenomatoid tumour and the borderline malignant tumours, namely the well-differentiated papillary mesothelioma and multicystic mesothelioma; (2) In relation to occupational-related compensation claims following asbestos exposure. This review summarizes the aetiology of asbestos-induced neoplasia, possible mechanisms of tumour development and highlights potential diagnostic pitfalls.

The available knowledge on the oncogenic risks of asbestos, the data on the uses of asbestos in railroads, with particular regard to the Italian State Railroads (Ferrovie dello Stato = FS), and the groups at risk due to the exposure to asbestos used in railroads were briefly reviewed. The available data on the pathological effects of such exposure, and particularly on the onset of mesotheliomas among machinists and other railroad workers, were also summarized. One hundred and thirty cases of mesothelioma (122 pleural, 1 pericardial, 6 peritoneal and 1 pleuro-peritoneal), related to the exposure to asbestos used in railroads, observed in various Italian regions, were reported. Fifty-three of these cases (among which 49 reported in the Emilia Romagna Region) were submitted to a detailed study at the Bologna Institute of Oncology. Seventy-seven cases of mesothelioma occurred among occupationally exposed FS workers, in particular machinists; 45 cases occurred among rolling-stock machinists and workers engaged in the repair and demolition of the rails of workshops not belonging to the FS; 3 cases occurred among travelling workers of rolling-stock, not belonging to the FS; and 5 cases were found in family members (1 daughter, 3 wives and 1 sister) of railroad workers. This series of cases, together with similar data from the literature, proves the existence of an actual health risk due to asbestos used in railroads, and indicates its gravity. On the basis of the available data, the following steps are considered necessary: the promotion of systematic epidemiological investigations, the adoption of preventive measures, the performance of medical oncological surveillance, and the automatic compensation for tumours following the exposure to the asbestos used in railroads.

The paper presents the European multicentric case-control study on risk for mesothelioma after non-occupational (domestic and environmental) exposure to asbestos. The study includes eight centres in seven European countries (Belgium, Denmark, Greece, Italy, Spain, Sweden and Switzerland). It is focused on the measurement of mesothelioma risk in relation to low intensity exposure to asbestos and to exposure to MMMF and other agents. It includes incident cases of pleural malignant mesothelioma (histologically diagnosed and verified) and a random sample of the population.

Background: Mesothelioma is not common in Taiwan; its clinical manifestations and biological behavior in the Chinese patients have thus rarely been discussed., Methods: The chart records of mesothelioma patients collected from 1979 to 1992 in Veterans General Hospital-Taipei were retrospectively reviewed., Results: Sixteen thoracic mesotheliomas were diagnosed in 15 patients, including 5 benign fibrous mesotheliomas, 1 malignant pericardial mesothelioma and 10 malignant pleural mesotheliomas. One patient suffered from benign fibrous mesothelioma initially, and the disease transformed into malignant mesothelioma 5 years later. Symptoms of benign fibrous mesothelioma were nonspecific and all 5 cases occurred in lower pleural cavities. One case presented with repeated hypoglycemic coma and the initial diagnosis was hepatoma. History of asbestos exposure probably occurred in 3 of 10 malignant pleural mesotheliomas. The median survival of malignant pleural mesothelioma after onset of symptoms was 14 months. Survival seemed better in epithelial type and those who received surgical treatment of malignant mesothelioma., Conclusions: The symptoms of mesothelioma were nonspecific. Benign fibrous mesothelioma should be considered in patients with palpable liver and hypoglycemic symptoms. Malignant transformation can occur in benign fibrous mesothelioma. Survival of malignant mesothelioma seems better in epithelial type and in those who received surgical treatment.

In the course of studies to elucidate the relative contribution of simian virus 40 (SV40) large T and small t proteins during oncogenesis, we observed the appearance of pericardial and pleural tumors in 100% of Syrian hamsters injected in the pleural space with wild type SV40. When SV40 was injected via the intracardiac or intraperitoneal routes, more than 50% of hamsters developed mesothelial tumors. Macroscopic, microscopic, ultramicroscopic, and histochemical characteristics identify these neoplasms and derived cell lines as mesotheliomas and mesothelioma-derived cell lines. The SV40 genome was integrated and expressed in the mesotheliomas and derived cell lines. The absence of mesotheliomas in hamsters injected with SV40 small t deletion mutants indicates that the small t protein plays an important role in the development of SV40-induced mesotheliomas. To the best of our knowledge, this is the first definitive report of virus-induced mesotheliomas in mammals.

Pericardial mesothelioma: epidemiological updating on the last decade. In the last decade, 109 cases of primitive malignant pericardial mesothelioma, histologically proved and reported in world medical literature have been added to the 206 cases described in a previous article. This illness, although rare, has been found more frequently in Japan (25 subjects) and in USSR (21 subjects). From the table in annex, the following points emerge: a) On average, pericardial mesothelioma tends to develop in fairly young people (compared to pleural or peritoneal mesothelioma). b) There is more proof that asbestos has an harmful effect on pericardial serosa. c) The most frequent clinical diagnoses refer mainly to acute pericarditis, constrictive pericarditis, cardiac tamponade and sometimes to various types of coronary heart disease, which autoptical examination reveals to be due to the presence of an external compression of the coronary arteries by pericardial mesothelioma. d) This illness, perhaps because of greater research and improved diagnostical means seems to be increasing when compared to the previous period (although this does not appear to be the case in our country).

A case of mesothelioma, apparently arising in the pericardium, is reported in a patient who, 15 years previously, had been treated for angina pectoris by dusting of the pericardial cavity with a mixture of fibrous dusts. At autopsy, transparent fibers and ferruginous bodies were present within the pericardium. Electron diffraction and microprobe analysis indicated that approximately two thirds of the fibers were tremolite and anthophyllite asbestos, and the remainder, fiber glass. Development of mesothelioma in laboratory animals has been reported after intrapleural deposition of asbestos and other fibers, but in humans, the link between exposure to asbestos and mesothelioma has always been based on epidemiologic data and the retrospective finding of asbestos in tissues. To our knowledge, this is the first example of a malignant mesothelioma in a human associated with direct mesothelial contact with fibrous dusts.

Two cases of malignant pericardial mesothelioma are described. Morphologically they corresponded to the epithelial or fibrous type, respectively. In one patient, an old-metal dealer, an increased number of asbestos bodies were found in the lung parenchyma and phase-microscopy demonstrated increased asbestos needles in the lung parenchyma. Since, beyond a certain fibre size, asbestos can induce bronchial carcinoma as well as pleural or peritoneal mesothelioma, it is likely that the pericardial mesothelioma had a similar aetiology.

A case of malignant mesothelioma (MM) in a 15 year old boy is reported. He had a prosthetic aortic valve implanted in 1980 at our centre for rheumatic aortic regurgitation. Fever, weight loss and chest pain as initial symptoms suggested possibility of prosthetic valve endocarditis. Dysphagia as another initial symptom was interesting and caused further diagnostic dilemma. Role of Computerised Tomography (CT) scan in diagnosis of MM is highlighted.

Three cases of malignant pericardial mesotheliomas are presented with evidence of occupational asbestos exposure. Examination results are compared with findings from experimental and epidemiological research on biological effects of asbestos dust. There are sufficient indications that time-limited effects of asbestos dust established either by measurements or assessment of the amount of concentration after a latency of more than 20 years are apt to result in the development of mesotheliomas of the pleura and peritoneum and, moreover, the pericardium. It is suggested that malignant pericardial mesothelioma also be recognized as another form of occupational disease caused by asbestos dust.

So-called mesothelioma of the atrioventricular node is a rare lesion and a cause of sudden, unexpected death. A case of congenital complete heart block accompanied by polyclonal immunoblastosis is presented. The histological features of our case are fundamentally similar to that of the cases previously reported. Immunohistochemical findings indicated that the tumor may have originated from aberrant epithelial or mesothelial tissue.

In order to determine the histogenesis of tumors of the atrioventricular node, so-called conduction tumors, two such tumors were serially blocked for electron microscopy. Ultrastructurally these tumors were composed of nests of cells arranged in small channels and tubules set in a connective tissue stroma. The cells lining the tubules were flattened or low cuboidal and had abundant microvilli over the lumen surface. The cells were joined by specialized junctions along their lateral adjacent borders, especially at the luminal surfaces, and intercellular spaces delineated by specialized junctions were frequent. Microvilli, intercellular spaces bounded by tight junctions, and complex intercellular junctions are features of mesothelial cells, and especially of benign mesothelioma of the genital tract. These results strongly suggest that the cardiac conduction tumor is derived from mesothelial cells and is in fact a mesothelioma of the atrioventricular node.