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Journal of Thoracic Oncology : Official... Dec 2022Primary pericardial mesothelioma (PPM) has no accepted standard-of-care treatment options with management and outcomes often extrapolated from diffuse pleural...
INTRODUCTION
Primary 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.
METHODS
Patients 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.
RESULTS
The 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.
CONCLUSIONS
PPM represents a distinct disease with no universally accepted treatment options. Our findings suggest that trimodality therapy may improve outcomes in selected patients with PPM.
Topics: Humans; Female; Young Adult; Adult; Middle Aged; Aged; Lung Neoplasms; Mesothelioma, Malignant; Pleural Neoplasms; Mesothelioma; Combined Modality Therapy; Thymus Neoplasms
PubMed: 36075530
DOI: 10.1016/j.jtho.2022.08.017 -
The National Medical Journal of India 2022Mesothelioma is a tumour arising from the mesothelial cells lining the pleura, pericardium, peritoneum, or the tunica vaginalis of testes. Primary pericardial...
Mesothelioma is a tumour arising from the mesothelial cells lining the pleura, pericardium, peritoneum, or the tunica vaginalis of testes. Primary pericardial mesothelioma is a rare tumour that can have varied manifestations and survival in patients with malignant pericardial tumours is generally dismal. The role of asbestos in pericardial mesotheliomas is less well established compared to that in pleural or peritoneal mesotheliomas. The prognosis is generally poor with the treatment options available. We present a middle-aged man with large pericardial effusion secondary to primary pericardial mesothelioma with no previous exposure to asbestos.
Topics: Asbestos; Heart Neoplasms; Humans; Male; Mesothelioma; Middle Aged; Pericardium; Peritoneal Neoplasms
PubMed: 36039621
DOI: 10.25259/NMJI_273_20 -
Current Problems in Cardiology Dec 2022Diffuse primary malignant pericardial mesothelioma (PMPM) is an extremely rare and highly invasive tumor of pericardium. The tumor can infiltrate myocardium in part of... (Review)
Review
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 echocardiographic 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.
Topics: Humans; Middle Aged; Echocardiography; Myocardium; Pericardium; Pericardial Effusion; Mesothelioma
PubMed: 36007620
DOI: 10.1016/j.cpcardiol.2022.101356 -
Respirology Case Reports Sep 2022Malignant pericardial mesothelioma (MPM) is a rare tumour that arises from the mesothelial cells of the pericardium. No standard treatment has been established owing to...
Malignant pericardial mesothelioma (MPM) is a rare tumour that arises from the mesothelial cells of the pericardium. No standard treatment has been established owing to a poor treatment response; therefore, MPM has a poor prognosis. We herein report a rare case of MPM in a 70-year-old man that was diagnosed immunohistopathologically using cell block sections of pericardial fluid and in which long-term survival for more than 3 years was achieved with only periodic pericardial drainage. Immunohistopathological staining investigations, especially BRCA1-associated protein 1 (BAP1) immunostaining using cell block sections of pericardial effusion, are effective in making a diagnosis of MPM. Well-differentiated papillary mesothelioma (WDPM) with BAP1 loss progresses to MPM in the long term, showing that BAP1 loss may induce phenotypical evolution of WDPM. BAP1 loss may also progress to malignant mesothelioma in situ and then to invasive mesothelioma. BAP1 immunohistochemistry should be considered for the early diagnosis of MPM.
PubMed: 35950141
DOI: 10.1002/rcr2.1004 -
Cancers Jun 2022Malignant mesothelioma (MMe) is a rare malignancy originating from the linings of the pleural, peritoneal and pericardial cavities. The best-defined risk factor is...
Malignant mesothelioma (MMe) is a rare malignancy originating from the linings of the pleural, peritoneal and pericardial cavities. The best-defined risk factor is exposure to carcinogenic mineral fibers (e.g., asbestos). Genomic studies have revealed that the most frequent genetic lesions in human MMe are mutations in tumor suppressor genes. Several genetically engineered mouse models have been generated by introducing the same genetic lesions found in human MMe. However, most of these models require specialized breeding facilities and long-term exposure of mice to asbestos for MMe development. Thus, an alternative model with high tumor penetrance without asbestos is urgently needed. We characterized an orthotopic model using MMe cells derived from mice chronically injected with asbestos. These MMe cells were tumorigenic upon intraperitoneal injection. Moreover, MMe cells showed mixed chromosome and microsatellite instability, supporting the notion that genomic instability is relevant in MMe pathogenesis. In addition, microsatellite markers were detectable in the plasma of tumor-bearing mice, indicating a potential use for early cancer detection and monitoring the effects of interventions. This orthotopic model with rapid development of MMe without asbestos exposure represents genomic instability and specific molecular targets for therapeutic or preventive interventions to enable preclinical proof of concept for the intervention in an immunocompetent setting.
PubMed: 35804881
DOI: 10.3390/cancers14133108 -
Translational Cancer Research May 2022Primary malignant pericardial mesothelioma (PMPM) is a highly malignant tumor originating in the pericardium serosum with clinical manifestations presenting as...
BACKGROUND
Primary malignant pericardial mesothelioma (PMPM) is a highly malignant tumor originating in the pericardium serosum with clinical manifestations presenting as constrictive pericarditis, with pericardial tamponade and heart failure. Malignant pericardial mesothelioma is rare and has a poor prognosis, with an average survival time of 6-10 months.
CASE DESCRIPTION
Herein, we report the case of a 57-year-old female who developed chest tightness and panic for no obvious reason. She was diagnosed with tuberculous pericarditis via multiple examinations including positron emission tomography/computed tomography (PET/CT), pleural biochemical routine, tuberculin purified protein derivative (PPD) test, T cell spot (T-SPOT) test, and echocardiography, and was experienced intermittent relief after anti-tuberculosis treatment. On 21 July, 2020, pericardiectomy was performed due to poor therapeutic effect, and the postoperative pathological diagnosis was malignant mesothelioma. After discussing treatment plans and considering the prognosis, the patient opted for palliative care. Subsequently, her symptoms gradually worsened, with chest tightness, shortness of breath, palpitations at rest, frequent arrhythmias, heart failure, cardiogenic shock, and multiple plasma chamber effusions. This case showed that the most common misdiagnosis of PMPM is tuberculous pericarditis, which needs to be differentiated from pleural mesothelioma with pericardial metastasis.
CONCLUSIONS
The diagnosis of PMPM is usually made by pathologic surgery or histopathological examination to determine the specific disease location. In addition, pericardiocentesis fluid exfoliation cytology, imaging and echocardiography can assist diagnosis. Due to the lack of effective treatment for PMPM, timely surgery and postoperative adjuvant chemotherapy are needed to improve the quality of life of patients and prolong their survival time.
PubMed: 35706788
DOI: 10.21037/tcr-22-778 -
Korean Circulation Journal Jun 2022
PubMed: 35656906
DOI: 10.4070/kcj.2022.0060 -
Cureus Apr 2022This case report presents a 60-year-old gentleman with a significant smoking history and possible asbestos exposure who was referred to the emergency department for...
This case report presents a 60-year-old gentleman with a significant smoking history and possible asbestos exposure who was referred to the emergency department for atrial fibrillation with a rapid ventricular rate and symptoms of heart failure. Labs showed normal brain natriuretic peptide and troponin I. His echocardiography finding suggested constrictive pericarditis with an ejection fraction of 60%. A computed tomography scan was concerning for a pericardial mass. Left and right heart catheterization hinted more toward constrictive physiology; however, some findings were concerning for restrictive physiology. Hence, cardiac magnetic resonance imaging was done, which established the diagnosis of constrictive pericarditis. Pericardiectomy was planned with a maze procedure for atrial fibrillation. However, a malignant neoplasm was seen on a frozen biopsy. Hence, surgery was limited to partial pericardiectomy, as the patient had advanced infiltrative neoplasm that had resulted in constrictive pericarditis. The final pathology report confirmed the diagnosis of malignant pericardial mesothelioma mixed type. Malignancy is usually diagnosed in an advanced stage, like in our case, due to nonspecific initial presentation. A literature review suggests that there is a lack of established consensus on treatment. The response to therapy also seems to be poor and results only in palliation of symptoms, with a median survival of six months from diagnosis despite optimum medical management.
PubMed: 35602795
DOI: 10.7759/cureus.24270 -
Journal of Cardiology Cases May 2022Primary pericardial mesothelioma is an extremely rare tumor, of unclear etiology, nonspecific presentation, with a delay in diagnosis, and a poor prognosis. We present...
Primary pericardial mesothelioma is an extremely rare tumor, of unclear etiology, nonspecific presentation, with a delay in diagnosis, and a poor prognosis. We present the case of a woman with pericardial mesothelioma, whose main manifestation was cardiac tamponade, currently alive three years after diagnosis and undergoing chemotherapy treatment. < Cardiac tamponade can be the first clinical manifestation of a neoplastic disease, including pericardial mesothelioma, so an adequate diagnostic approach should always be carried out in patients with this clinical picture. Advances in the treatment of pulmonary mesotheliomas have allowed chemotherapy regimens to be adjusted to the treatment of pericardial mesotheliomas, which can increase survival in patients with this type of cancer, which is still considered to have a poor prognosis.>.
PubMed: 35582078
DOI: 10.1016/j.jccase.2021.10.010 -
Cancers May 2022Mesothelin (MSLN) is a protein expressed in the mesothelial cell lining of the pleura, peritoneum, and pericardium; its biological functions in normal cells are still... (Review)
Review
Mesothelin (MSLN) is a protein expressed in the mesothelial cell lining of the pleura, peritoneum, and pericardium; its biological functions in normal cells are still unknown. Experimental studies using knockout mice have suggested that this molecule does not play an important role in development and reproduction. In contrast, it has been observed that this molecule is produced in abnormal amounts in several malignant neoplasms, such as mesotheliomas and pancreatic adenocarcinomas. Many molecular studies have also demonstrated that mesothelin is overexpressed in HSOCs. Here, we discuss the current knowledge of mesothelin and focus on its role in clinical and pathological diagnoses, as well as its impact on the prognosis of HSOC. Moreover, regarding the binding of MSLN to the ovarian cancer antigen CA125, which has been demonstrated in many studies, we also report on signal transduction pathways that may play an important role in the spread and neoplastic progression of this lethal neoplasm. Given that mesothelin is overexpressed in many solid tumours and has antigenic properties, this molecule could be considered an antigenic target for the treatment of many malignancies. Consequently, we also review the literature to report on mesothelin-targeting therapies for HSOC that have been recently investigated in many clinical studies.
PubMed: 35565412
DOI: 10.3390/cancers14092283