-
Journal of Thoracic Oncology : Official... Dec 2022
Topics: Humans; Lung Neoplasms; Mesothelioma, Malignant; Heart Neoplasms; Heart
PubMed: 36410967
DOI: 10.1016/j.jtho.2022.09.224 -
Current Treatment Options in Oncology Oct 2000Primary pericardial mesothelioma is a rare but lethal disease. Altogether there are about 150 cases reported in the literature. In most cases the diagnosis is made at... (Review)
Review
Primary pericardial mesothelioma is a rare but lethal disease. Altogether there are about 150 cases reported in the literature. In most cases the diagnosis is made at autopsy or postoperatively. Clinical signs and symptoms are typically nonspecific and are similar to compromised cardiac function. Surgical resection remains the main treatment modality. When the disease is localized and completely resected, long-term survival can result. Most often the tumor invades the myocardium or the great vessels and therefore is at best palliative in relieving pericardial tamponade or constriction. Addition of chemotherapy or radiation has been disappointing. Newer therapeutic approaches for malignant pleural mesothelioma are likely to influence the treatment of pericardial mesothelioma in the future.
Topics: Antineoplastic Agents; Clinical Trials as Topic; Combined Modality Therapy; Heart Neoplasms; Humans; Mesothelioma; Pericardium; Radiotherapy; Survival Rate
PubMed: 12057155
DOI: 10.1007/s11864-000-0045-6 -
Current Treatment Options in Oncology Oct 2002Pericardial mesothelioma is a rare cancer for which treatment options are limited. Operative intervention in pericardial mesothelioma is primarily for effusion control,... (Review)
Review
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.
Topics: Heart Neoplasms; Humans; Mesothelioma; Pericardium; Time Factors
PubMed: 12194802
DOI: 10.1007/s11864-002-0002-7 -
QJM : Monthly Journal of the... Sep 2016
Topics: Aged; Asbestos; Echocardiography; Fatal Outcome; Heart Neoplasms; Humans; Magnetic Resonance Imaging, Cine; Male; Mesothelioma; Pericardium; Pleural Neoplasms; Radiography, Thoracic
PubMed: 27318362
DOI: 10.1093/qjmed/hcw099 -
Seminars in Thoracic and Cardiovascular... 2022Primary pericardial mesothelioma is a rare malignancy of the mesothelial lining of the pericardium. This study aimed to evaluate the clinical characteristics and...
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.
Topics: Female; Heart Neoplasms; Humans; Lung Neoplasms; Male; Mesothelioma; Mesothelioma, Malignant; Pleural Neoplasms; Propensity Score; Thymus Neoplasms; Treatment Outcome; United States
PubMed: 34320396
DOI: 10.1053/j.semtcvs.2021.07.020 -
Advances in Anatomic Pathology Jul 2023Primary 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...
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.
Topics: Humans; Prospective Studies; Mesothelioma, Malignant; Mesothelioma; Pleural Neoplasms; Sarcoma; Heart Neoplasms
PubMed: 37104724
DOI: 10.1097/PAP.0000000000000399 -
Seminars in Thoracic and Cardiovascular... 2022
Topics: Heart Neoplasms; Humans; Mesothelioma; Mesothelioma, Malignant; Pericardium; Treatment Outcome
PubMed: 34469797
DOI: 10.1053/j.semtcvs.2021.08.017 -
European Heart Journal Feb 2024
Topics: Humans; Mesothelioma; Pericardium; Heart Neoplasms
PubMed: 38085299
DOI: 10.1093/eurheartj/ehad763 -
BMC Cardiovascular Disorders Mar 2023Pericardial calcification is usually a marker of chronic diseases, and its occurrence in rapidly progressing malignant primary pericardial mesothelioma (PPM) is...
BACKGROUND
Pericardial 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 PRESENTATION
A 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.
CONCLUSION
We 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.
Topics: Female; Humans; Middle Aged; Mesothelioma; Diagnosis, Differential; Pericardium; Pericarditis, Constrictive; Mesothelioma, Malignant; Heart Neoplasms; Heart Failure; Thymus Neoplasms
PubMed: 36890475
DOI: 10.1186/s12872-023-03142-w -
Modern Pathology : An Official Journal... Sep 2023Primary pericardial mesotheliomas are extremely rare, accounting for <1% of all mesotheliomas, and their molecular genetic features and predisposing factors remain to be...
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.
Topics: Humans; Male; Female; Aged; Lung Neoplasms; Neoplasm Recurrence, Local; Mesothelioma, Malignant; Mesothelioma; Pleural Neoplasms; Heart Neoplasms; Thymus Neoplasms; Ubiquitin Thiolesterase; Biomarkers, Tumor
PubMed: 37295554
DOI: 10.1016/j.modpat.2023.100237