Primary Pericardial Mesothelioma Research Articles

Constrictive Pericarditis Caused by Primary Pericardial Mesothelioma: A Case Series.

Constrictive Pericarditis Caused by Primary Pericardial Mesothelioma: A Case Series.

Recurrent Tumor Suppressor Alterations in Primary Pericardial Mesothelioma

Primary pericardial mesotheliomas are extremely rare, accounting for <1% of all mesotheliomas, and their molecular genetic features and predisposing factors remain to be determined. Here, we report the clinicopathologic, immunohistochemical, and molecular genetic findings of 3 pericardial mesotheliomas without pleural involvement. Three cases diagnosed between 2004 and 2022 were included in the study and analyzed by immunohistochemistry and targeted next-generation sequencing (NGS); corresponding nonneoplastic tissue was sequenced in all cases. Two patients were female and 1 was male, aged between 66 and 75 years. Two patients each had prior asbestos exposure and were smokers. Histologic subtypes were epithelioid in 2 cases and biphasic in 1 case. Immunohistochemical staining identified expression of cytokeratin AE1/AE3 and calretinin in all cases, D2-40 in 2 cases, and WT1 in 1 case. Staining for tumor suppressors revealed loss of p16, MTAP, and Merlin (NF2) expression in 2 cases and loss of BAP1 and p53 in 1 case. Abnormal cytoplasmic BAP1 expression was observed in an additional case. Protein expression abnormalities correlated with NGS results, which showed concurrent complete genomic inactivation of CDKN2A/p16, CDKN2B, MTAP, and NF2 in 2 mesotheliomas and of BAP1 and TP53 in 1 mesothelioma each, respectively. In addition, 1 patient harbored a pathogenic BRCA1 germline mutation, which resulted in biallelic inactivation in the mesothelioma. All mesotheliomas were mismatch repair proficient and showed several chromosomal gains and losses. All patients died from disease. Our study demonstrates that pericardial mesotheliomas share common morphologic, immunohistochemical, and molecular genetic features with pleural mesothelioma, including recurrent genomic inactivation of canonical tumor suppressors. Our study adds new insights into the genetic landscape of primary pericardial mesothelioma and highlights BRCA1 loss as a potential contributing factor in a subset of cases, thereby contributing to refined precision diagnostics for this rare cancer.

A Peculiar Presentation of Tamponade: Primary Pericardial Mesothelioma

Primary pericardial mesothelioma is an exceedingly rare pathology (incidence 0.002%) and is most often diagnosed post-mortem. Consequently, the natural history, clinical presentation, investigation, and management algorithms of the pathology are not well established.

Pericardial Mesotheliomas.

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.

Primary pericardial mesothelioma complicated by pericardial calcification

BackgroundPericardial calcification is usually a marker of chronic diseases, and its occurrence in rapidly progressing malignant primary pericardial mesothelioma (PPM) is extremely rare. Therefore, this atypical imaging appearance contributes to more frequent misdiagnosis of PPM. However, no systematic summary currently exists of the imaging characteristics of malignant pericardial calcification in PPM. In our report, its clinical characteristics are discussed in detail, to provide a reference to reduce the misdiagnosis rates of PPM.Case presentationA 50-year-old female patient was admitted to our hospital, presenting primarily with features suggestive of cardiac insufficiency. Chest computed tomography revealed significant pericardial thickening and localized calcification, suspicious of constrictive pericarditis. A chest examination performed through a midline incision showed a chronically inflamed and easily-ruptured pericardium that was closely adherent to the myocardium. Post-operative pathological examination confirmed a diagnosis of primary pericardial mesothelioma. Six weeks postoperatively, the patient experienced symptom recurrence and abandoned chemotherapy and radiation therapy. Nine months postoperatively, the patient died of heart failure.ConclusionWe report this case to highlight the rare finding of pericardial calcification in patients with primary pericardial mesothelioma. This case illustrated that confirmation of pericardial calcification cannot completely rule out rapidly developing PPM. Therefore, understanding the different radiological features of PPM can help to reduce its rate of early misdiagnosis.

Multimodality Therapy in Patients With Primary Pericardial Mesothelioma

IntroductionPrimary pericardial mesothelioma (PPM) has no accepted standard-of-care treatment options with management and outcomes often extrapolated from diffuse pleural mesothelioma. Disease-specific research is needed to better define PPM. We report our institutional experience with PPM highlighting the potential role for multimodality therapy. MethodsPatients with PPM diagnosed by a multidisciplinary team of medical oncologists, thoracic surgeons, thoracic pathologists, and radiologists between January 2011 and January 2022 were followed to February 2022. Clinicopathologic features and treatment outcomes were annotated. Overall survival (OS) was defined from the date of pathologic diagnosis. ResultsThe median age at diagnosis of the 12 patients identified with having PPM was 51 (range: 21–71) years old. Most patients were of female sex (n = 8; 67%), 75% of the samples were epithelioid (n = 9), and 25% were nonepithelioid (two sarcomatoid and one biphasic). Most cases (92%, 11 of 12) had expression of at least two mesothelial markers on immunohistochemistry. The median OS of the cohort was 25.9 months. Five patients had an OS greater than 12 months; four of whom received pericardial radiation. Three of the patients who received radiation did so as part of a trimodality approach (surgical resection, adjuvant chemotherapy, and radiation); the OS for patients who received trimodality therapy was 70.3 months versus 8.2 months for those who did not. ConclusionsPPM represents a distinct disease with no universally accepted treatment options. Our findings suggest that trimodality therapy may improve outcomes in selected patients with PPM.

An Autopsy Case of Primary Malignant Pericardial Mesothelioma -A Comprehensive Review of 95 Cases Reported in Japan-

An Autopsy Case of Primary Malignant Pericardial Mesothelioma -A Comprehensive Review of 95 Cases Reported in Japan-

Primary malignant pericardial mesothelioma in a patient with a history of thoracic radiotherapy and asbestos exposure: A case report

Primary malignant pericardial mesothelioma in a patient with a history of thoracic radiotherapy and asbestos exposure: A case report

Malignant primary pericardial mesothelioma presenting as effusive constrictive pericarditis: a case report study

BackgroundPrimary pericardial mesothelioma (PPM) is a rare malignancy with a high prevalence of mortality. The diagnosis is usually challenging using a variety of imaging modalities and invasive procedures and is generally performed at the later stages of the disease or in autopsy. This case study points to an unconventional presentation of PPM and the challenges in diagnosing this rare mortal malignancy.Case presentationThis study presents a 44-year-old woman with no remarkable medical history with an initial diagnosis of effusive constrictive pericarditis at first hospitalization. Imaging evaluations, including transthoracic echocardiography and chest computed tomography scan, demonstrated visible thickened pericardium, pericardial effusion, and mass-like lesions in pericardium and mediastinum. The definite diagnosis of primary pericardial mesothelioma was established after pericardiectomy and histopathology examinations. Chemotherapy with pemetrexed and carboplatin was administrated to the patient, and she has been through four cycles of chemotherapy with no complications to date.ConclusionConstrictive pericarditis is an uncommon presentation of PPM. Due to the high mortality rate and late presentation, difficulties and uncertainties in diagnosis, being aware of this rare malignant entity in different cardiac manifestations, particularly when there is no clear explanation or response to treatment in such conditions, is highly important.

Primary Pericardial Mesothelioma: A Population-Based Propensity Score-Matched Analysis

Primary pericardial mesothelioma is a rare malignancy of the mesothelial lining of the pericardium. This study aimed to evaluate the clinical characteristics and survival outcomes of these patients using a United States population-based cancer database. We queried the Surveillance, Epidemiology, and End Results program (1973-2015). Primary pericardial mesothelioma patients with complete follow-up data were included, and primary pleural mesothelioma patients were identified as controls. Propensity-score matching was used to balance individual characteristics. Kaplan-Meier analysis and log-rank tests were performed to compare overall survival. Forty-one primary pericardial mesothelioma and 15,970 primary pleural mesothelioma patients were identified. Before matching, when compared to the pleural mesothelioma counterparts, primary pericardial mesothelioma patients were younger (median 57 vs 73 years, P < 0.001), more likely to be female (46.3% vs 20.2%, P < 0.001), more likely to be nonwhite (24.4% vs 8.4%, P=0.001), and less likely to have been diagnosed in the most recent study decade (2006-2015, 34.1% vs 43.5%, P=0.002). The overall 1- and 2-year survival rates were 22.0% and 12.2%, with a median survival of 2 months (IQR: 1-6). After 1:2 nearest neighbor propensity-score matching, 38 pericardial mesothelioma and 76 matched pleural mesothelioma cases were identified. The 2 matched groups had comparable baseline characteristics, including age, sex, race, year of diagnosis, histological type, and cancer history. Compared to their pleural mesothelioma counterparts, primary pericardial mesothelioma patients were less likely to receive chemotherapy (23.7% vs 50.0%, P=0.01) and had worse overall survival (median survival: 2 vs 10 months, log-rank P=0.006). Primary pericardial mesothelioma has worse survival outcomes than pleural mesothelioma, with a median survival of only 2 months. These patients should seek care from experienced multidisciplinary teams at tertiary care centers that handle high volumes of mesothelioma patients.

The value of 18F-FDG PET/CT in two cases of Primary Pericardial Mesothelioma

The value of 18F-FDG PET/CT in two cases of Primary Pericardial Mesothelioma

Case Report: Primary Pericardial Mesothelioma

BACKGROUND: Primary pericardial mesothelioma is an extremely rare mesodermal tumor, among oncological diseases, with a very low incidence. About 350 cases have been described around the world, most of them diagnosed post-mortem. The short term prognosis is poor, regardless of the treatment, which is mostly palliative. We present a case report regarding this pathology. CASE REPORT: 69 year old patient hospitalized with dyspnea and severe anemia. Complementary workup showed pleural effusion and pericardial effusion. Histopathology report of the pericardial fluid revealed pericardial mesothelioma. EVOLUTION: Due to recurrence of pericardial effusion, it was decided to perform pericardiectomy and left pleurectomy as palliative treatment, to alleviate the patient’s symptoms. Chemotherapy was planned after the procedure, but the patient’s general condition deteriorated severely leading to his death. CONCLUSION: Primary pericardial mesothelioma is a rare tumor, whose symptoms are characterized by the associated pericardial effusion, making the diagnosis difficult due to it low incidence. The prognosis is poor in short- term, and there isn’t an established protocol for the treatment with results that showed improve in mortality. KEYWORDS: MESOTHELIOMA, PERICARDIAL EFFUSION, PERICARDIECTOMY, PLEUROTOMY.

Timely diagnosis of primary pericardial mesothelioma

Timely diagnosis of primary pericardial mesothelioma

A case report of malignant primary pericardial mesothelioma with atypical imaging appearance: multimodality imaging with histopathological correlation.

Background Primary pericardial mesothelioma is a rare primary cardiac malignancy, with three main histopathological types, sarcomatoid histotype being the rarest. The imaging features were atypical due to concomitant extensive calcification, which resulted in aggravated differential diagnosis.Case summary A 45-year-old man presented to our hospital with non-specific clinical symptoms. According to clinical history, a mediastinal tumour had been suspected with a previous unsuccessful attempt of transthoracic computed tomography-guided biopsy at an overseas hospital with limited data of performed imaging procedures. Multimodality imaging at our centre revealed extensively calcified solid masses in the pericardium, invading the left atrium. As the imaging features suggested an atypical primary pericardial malignancy, a diagnostic thoracoscopy was performed. Histopathological analysis of specimen revealed sarcomatoid type of pericardial mesothelioma with areas of necrosis and foci of osteogenic differentiation. Despite planned treatment, 2 weeks after histological diagnosis, the patient passed away due to perforated peptic ulcer-related sepsis.Discussion The presence of extensive calcification in the lesion resulted in a challenging imaging workup and diagnosis. Initial differential diagnosis included primary or metastatic calcification-prone tumour, secondary calcification due to haemorrhage after previous interventional procedure and other pathologies, such as tuberculous pericarditis, calcified amorphous tumour, among others. Calcification may be part of the histological tumour characteristics; however, proper history taking is crucial as concomitant diseases, previous treatment, and interventional procedures may alter the imaging pattern.

Primary Pericardial Mesothelioma: Report of a Patient and Literature Review

Malignant pericardial mesothelioma is a very rare tumoraccounting for 4% of all cardiac and pericardial tumors. We introduce a rare case of primary pericardial mesotheliomasince patients with mesothelioma mostly present with nonspecific symptomsand one-time diagnosis plays a major role in treatment. Our patient is a 29-year-oldCaucasian man with a complaint of worsening dyspnea and weight loss over the prior three weeks. After transthoracic echocardiography and CT scan, the patient underwent open-heart surgery and biopsy was achieved. Finally, the diagnosis of primary pericardial mesothelioma was confirmed.

Pericardial Mesothelioma in a Dog: The Feasibility of Ultrasonography in Monitoring Tumor Progression

A 6-year-old neutered male Yorkshire Terrier presented with recurrent pericardial effusion. Although clinical examinations including computed tomography were inconclusive, an exploratory thoracotomy revealed multiple small nodules and plaques on the inner surface of the pericardial sac (Day 1). A subtotal pericardiectomy was performed to prevent cardiac tamponade due to the increasing pericardial effusion, and the resected section of the pericardium was histopathologically diagnosed with mesothelioma. After surgery, chemotherapy with intrathoracic carboplatin was commenced. During the course of the treatment, a detailed follow-up ultrasonographic scan was performed to detect early lesions disseminated on the pleura, originating from the primary pericardial mesothelioma. On Day 101, the minute pleural nodules, which were disseminated lesions as predicted, were successfully imaged by ultrasonography. As the clinical stage advanced, the nodules were observed to gradually increase in size and number, implying tumor progression. These observations highlight the feasibility of ultrasonography in detecting minute disseminated lesions at an early stage, monitoring tumor progression, and thereby, predicting the prognosis of canine pericardial mesothelioma.

Primary Pericardial Mesothelioma an Oncological Rarity

AbstractPrimary pericardial mesothelioma is a rare and lethal tumor, with a reported prevalence of &lt;0.002% [1]. Pleural and pericardial mesotheliomas, which are far more common are usually associated with exposure to asbestos. However, the cause of pericardial mesothelioma is less clear [2] and is influenced by other factors like genetic predisposition, immunological impairment, infection, radiation, dietary factors and recurrent serosal inflammation [3]. Clinical manifestations of the neoplasm include constrictive pericarditis, cardiac tamponade, and heart failure. Because of late presentation and few treatment approaches, primary pericardial mesothelioma carries a poor prognosis. In this paper, we present a case of primary pericardial mesothelioma in a 46 year female presenting as recurrent pericardial effusion along with constrictive pericarditis.

Treatment and outcomes of primary pericardial mesothelioma: A systematic review.

e20027 Background: Primary pericardial mesothelioma (PPM) is a rare cancer, for which there is no consensus on treatment. We performed a retrospective systematic review of recently published PPM cases to characterize risk factors, treatment patterns, and clinical outcomes. Methods: Published literature from 2000-2016 was searched using the terms “primary pericardial mesothelioma”, “pericardial mesothelioma” and “malignant pericardial mesothelioma”. T-tests, Chi-square, and multivariable stepwise logistic regression were employed to correlate demographics, disease characteristics, and treatment with mortality. Results: We identified 6 case series and 84 case reports for a total of 103 PPM cases. Median age at diagnosis was 55; age range was 19 to 87. 39% were female and 61% were male. The mean time from diagnosis to death was 5.6 months. Tobacco exposure and asbestos exposure were reported in 15% and 25%, respectively. Neither exposure was predictive of mortality. 46% of patients underwent surgery, 8% received radiation, 37% received chemotherapy and 23% received no treatment. For 13% of patients, the treatment received, if any, was unknown. Radiation treatment was not associated with a mortality benefit (p = 0.54). A mortality benefit was seen for patients who had resection of their tumor (46% alive at last follow-up, p = 0.0061) but not in patients who had pericardiectomy (25% alive at last follow-up, p = 0.44). A mortality benefit was noted in those who received chemotherapy (p = 0.0022), specifically pemetrexed (p = 0.0038) or platinum agents (p = 0.003). Gemcitabine was not associated with a mortality benefit (p = 0.101). Conclusions: To the authors’ knowledge, this is the first comprehensive analysis of published PPM cases in the modern era. In this retrospective systematic review, a mortality benefit was seen with tumor resection and chemotherapy. Selection bias may account for some of this effect. However, given the inability to perform prospective studies in this population due to small patient numbers, it seems reasonable to offer surgical and medical therapy to eligible patients with PPM.

A Case Report of Primary Pericardial Malignant Mesothelioma Treated with Pemetrexed and Cisplatin

Primary pericardial malignant mesothelioma (PPM) is a very rare malignancy, with an incidence of less than 0.002% and represents less than 5% of all mesotheliomas. The cause of pericardial mesothelioma is uncertain that differ from pleural mesothelioma which is associated with asbestos exposure. This malignancy is terribly aggressive and has very poor prognosis with less than six months of overall survival. We present a case of a 71-year-old woman who was diagnosed with cardiac tamponade caused by PPM and received chemotherapy with pemetrexed and cisplatin for six months. During two years she was alive without disease progression. To better understand the clinical, pathologic features and treatment outcome of this entity, we reviewed 23 cases described in the English literature from 2009, together with our case, provided a total of 24 cases. Based on this review, we suggest that PPM must be considered in patients who have unexplained massive pericardial effusion and recommend chemotherapy with pemetrexed and cisplatin for the better outcome of PPM.

PERICARDIAL MESOTHELIOMA WITH THROMBOVASCULAR COMPLICATIONS (CASE FROM PRACTICE)

Primary mesothelioma of the pericardium is a rare heart tumor with a difficult diagnosis, revealed in vivo in less than a quarter of cases. The disease occurs at any age, more common in men and variably exhibits a broad spectrum of non-specific symptoms of congestive heart failure, constrictive pericarditis, pericardial effusion or cardiac tamponade. Patients are usually observed with peripheral edema, ascites, dyspnea, cough, chest pain and atrial fibrillation. Such symptoms, in the absence of cancer alertness, are erroneously attributed by doctors to more common cardiovascular diseases. As a result, primary mesothelioma is detected in 75-90% of cases only at necropsy. The article describes a case of detection at autopsy of primary pericardial mesothelioma sarcomatous type with invasion into the myocardium in a patient of 74 years old. The patient also suffered from concomitant coronary artery disease with a long history of chronic heart failure and recurrent pulmonary embolism, associated with deep vein thrombosis at the final stage of the disease. An objective study revealed signs of chronic heart failure. The laboratory data included mild iron deficiency anemia, insignificant leukocytosis and leukocyturia, as well as signs of moderate chronic kidney disease. Instrumental results corresponded to long-term course of hypertension, signs of congestive heart failure in the presence of atrial fibrillation, atherosclerosis of lower limbs arteries in patient with abdominal obesity. Thus, there were no clinical signs of pericardial damage in a standard examination of the patient. The article describes the complexity of the disease diagnosis, variable clinical picture, as well as the diagnostic value of various instrumental methods from the perspective of evidence-based medicine. It is noted that clinical alertness is still the most important factor in the lifetime diagnosis of pericardial mesothelioma. Disease should be considered in patients with recurrent pericardial effusion, constrictive pericarditis, pericardial mass or thickening of pericardium leaflets. For reliable diagnosis of a tumor, modern imaging studies and histological examination are required.