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Current Cardiology Reports Jul 2021Tumors of the pericardium are rare, but a wide variety of congenital, infectious, inflammatory, and neoplastic processes have been reported. Pericardial tumors can be... (Review)
Review
PURPOSE OF REVIEW
Tumors of the pericardium are rare, but a wide variety of congenital, infectious, inflammatory, and neoplastic processes have been reported. Pericardial tumors can be categorized as non-neoplastic or neoplastic. Neoplastic lesions can be further divided into benign or malignant, with malignancies being either primary or secondary (metastatic). Clinical, radiographic, and pathologic features of the most common entities are discussed.
RECENT FINDINGS
Metastatic neoplasms involving the heart and pericardium are far more common than primary pericardial neoplasms. Of primary pericardial malignancies, mesothelioma is the most common; notably, cytology of effusion fluid is relatively insensitive to the diagnosis. The prognosis for most malignancies of the pericardium, primary or secondary, is poor. Increasingly, clinically recognized diseases that involve the pericardium include Erdheim-Chester and IgG4-related disease. This article provides a comprehensive review of the most recent literature to develop a structured framework to the differential diagnosis of pericardial tumors.
Topics: Diagnosis, Differential; Heart Neoplasms; Humans; Mesothelioma; Mesothelioma, Malignant; Pericardium
PubMed: 34269901
DOI: 10.1007/s11886-021-01548-6 -
Echocardiography (Mount Kisco, N.Y.) Oct 2023A 46-year-old woman underwent pericardiocentesis and pericardial window for recurrent pericardial effusion. She presented 17 months later with signs and symptoms...
A 46-year-old woman underwent pericardiocentesis and pericardial window for recurrent pericardial effusion. She presented 17 months later with signs and symptoms consistent with constrictive pericarditis. Cardiac magnetic resonance imaging revealed an infiltrative mass surrounding the pericardium. A transcutaneous core needle biopsy of the pericardium confirmed the diagnosis of pericardial mesothelioma.
PubMed: 37694436
DOI: 10.1111/echo.15687 -
Histopathology Mar 2024Mesothelioma is a rare malignancy of the serosal membranes that is commonly related to exposure to asbestos. Despite extensive research and clinical trials, prognosis to... (Review)
Review
AIMS
Mesothelioma is a rare malignancy of the serosal membranes that is commonly related to exposure to asbestos. Despite extensive research and clinical trials, prognosis to date remains poor. Consistent, comprehensive and reproducible pathology reporting form the basis of all future interventions for an individual patient, but also ensures that meaningful data are collected to identify predictive and prognostic markers.
METHODS AND RESULTS
This article details the International Collaboration on Cancer Reporting (ICCR) process and the development of the international consensus mesothelioma reporting data set. It describes the 'core' and 'non-core' elements to be included in pathology reports for mesothelioma of all sites, inclusive of clinical, macroscopic, microscopic and ancillary testing considerations. An international expert panel consisting of pathologists and a medical oncologist produced a set of data items for biopsy and resection specimens based on a critical review and discussion of current evidence, and in light of the changes in the 2021 WHO Classification of Tumours. The commentary focuses particularly upon new entities such as mesothelioma in situ and provides background on relevant and essential ancillary testing as well as implementation of the new requirement for tumour grading.
CONCLUSION
We recommend widespread and consistent implementation of this data set, which will facilitate accurate reporting and enhance the consistency of data collection, improve the comparison of epidemiological data, support retrospective research and ultimately help to improve clinical outcomes. To this end, all data sets are freely available worldwide on the ICCR website (www.iccr-cancer.org/data-sets).
Topics: Humans; Peritoneum; Pleura; Retrospective Studies; Mesothelioma; Mesothelioma, Malignant; Pericardium; Pathology, Clinical
PubMed: 38044849
DOI: 10.1111/his.15106 -
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 -
Annals of Translational Medicine Dec 2022Pericardial mesothelioma (PeM) is a rare disease with non-specific symptoms at the onset, because of its rarity, the relevant literature is limited to case reports and...
BACKGROUND
Pericardial mesothelioma (PeM) is a rare disease with non-specific symptoms at the onset, because of its rarity, the relevant literature is limited to case reports and small case series, with no cases exceeding 100 in more than 20 years. As the most common initial symptoms are chest tightness and shortness of breath, early diagnosis is difficult, and the beginning of treatment is easily delayed. We present a rare case of difficult-to-diagnose PeM in which the diagnosis was clarified by surgery and the patient achieved a long survival, providing clinicians with our experience in treating this disease at an early stage of diagnosis and early treatment.
CASE DESCRIPTION
A 65-year-old female patient attended Affiliated Hospital of Zunyi Medical University, complaining about chest tightness and shortness of breath after activity for the last 2 months, accompanied by edema of the lower limbs in last month. A well circumscribed anterior-mediastinal, partially cystic mass was observed on the chest computed tomography. The patient's heart was compressed by the mass, and the patient had cardiac tamponade symptoms. Cardiac ultrasound showed the enlargement of the right heart, a widened pulmonary artery, pulmonary hypertension, and severe tricuspid regurgitation. The nature of the mass could not be determined prior to the surgery. Anterior superior mediastinal tumour resection and partial pericardial resection and closed thoracic drainage in a median open chest were performed, and pathohistological analysis revealed localized pericardial, epithelioid mesothelioma. In a follow-up after 19 months patient was generally well and without specific discomfort.
CONCLUSIONS
Differential diagnosis of the anterior mediastinal mass is broad. In patients with a mediastinal tumour who have significant symptoms, are difficult to diagnose and can tolerate surgery, the thoracic surgeon can use surgery as early as possible to make a definitive diagnosis, save the patient's life, and/or improve the patient's quality of life, experienced pathologist is essential to make fast and correct diagnosis.
PubMed: 36660732
DOI: 10.21037/atm-22-4719 -
World Journal of Gastrointestinal... May 2016Mesothelin, C-ERC/mesothelin is a 40-kDa cell surface glycoprotein that is normally present on normal mesothelial cells lining the pleura, peritoneum, and pericardium.... (Review)
Review
Mesothelin, C-ERC/mesothelin is a 40-kDa cell surface glycoprotein that is normally present on normal mesothelial cells lining the pleura, peritoneum, and pericardium. Moreover, mesothelin has been shown to be overexpressed in several human cancers, including virtually all mesothelioma and pancreatic cancer, approximately 70% of ovarian cancer and extra bile duct cancer, and 50% of lung adenocarcinomas and gastric cancer. The full-length human mesothelin gene encodes the primary product, a 71-kDa precursor protein. The 71-kDa mesothelin precursor is cleaved into two products, 40-kDa C-terminal fragment that remains membrane-bound via glycosylphosphatidylinositol anchor, and a 31-kDa N-terminal fragment, megakaryocyte potentiating factor, which is secreted into the blood. The biological functions of mesothelin remain largely unknown. However, results of recent studies have suggested that the mesothelin may play a role of cell proliferation and migration. In pancreatic cancer, mesothelin expression was immunohistochemically observed in all cases, but absent in normal pancreas and in chronic pancreatitis. Furthermore, the expression of mesothelin was correlated with an poorer patient outcome in several human cancers. The limited mesothelin expression in normal tissues and high expression in many cancers makes it an attractive candidate for cancer therapy. The present review discusses the expression and function of mesothelin in cancer cells and the utility of mesothelin as a target of cancer therapy.
PubMed: 27190694
DOI: 10.4291/wjgp.v7.i2.218 -
AME Case Reports 2023Malignant mesothelioma (MM) is a rare cancer with poor prognosis. It is less common that two serosal cavities are involved when the patient seeks medical attention...
BACKGROUND
Malignant mesothelioma (MM) is a rare cancer with poor prognosis. It is less common that two serosal cavities are involved when the patient seeks medical attention firstly. The current first-line chemotherapy for advanced MM is a combination with cisplatin and pemetrexed. However, nedaplatin, a second-generation platinum-based antitumor agent, has the similar therapeutic effects as cisplatin but lower toxicity and higher water solubility. To our knowledge, this is the first case of co-existing pericardial and pleural MM treated with nedaplatin and pemetrexed and responding well.
CASE DESCRIPTION
A 33-year-old woman, who had worked in a kiln for more than 10 years, suffered from dyspnea and chest tightness for 6 days. Chest computed tomography (CT) showed a massive pericardial effusion. She was diagnosed tuberculous pericarditis and received 6 months antituberculosis treatment (rifampicin, isoniazide, pyrazinamide, ethambutol). But it was ineffective and she was re hospitalized again due to massive pleural effusion and pericardial effusion. She was diagnosed with co-existing pericardial and pleural MM finally based on pleural biopsy and cytology of pericardial effusion. She was responding well excitedly to chemotherapy with nedaplatin and pemetrexed with high tolerance. Bone marrow toxicity or recurrent massive pericardial or pleural effusion were not observed during chemotherapy. However, she gave up chemotherapy and has survived for 22 months, from the onset symptoms.
CONCLUSIONS
In terms of clinical tolerance and less adverse reactions, we suggest that chemotherapy of nedaplatin with pemetrexed may be a more appropriate treatment in advanced MM. Further clinical trials are warrant.
PubMed: 37942039
DOI: 10.21037/acr-22-102 -
Cardiovascular Ultrasound Apr 2018Little is known about the echocardiographic characteristics of primary malignant pericardial mesothelioma (PPM) due to its rarity. The aim of this study was to explore...
BACKGROUND
Little is known about the echocardiographic characteristics of primary malignant pericardial mesothelioma (PPM) due to its rarity. The aim of this study was to explore the sex-specific echocardiographic patterns of PPM and risk factors for in-hospital mortality.
METHODS
A retrospective information retrieval was conducted for cases of PPM reported from China during 1981 and 2015. The diagnosis was made by histopathological examinations and only cases with echocardiographic descriptions were included. Data on the clinical and echocardiographic findings were collected. Difference in clinical, sex-specific echocardiographic characteristics and findings across different time periods were assessed. Logistic regression analysis was performed to explore echocardiographic risk factors for in-hospital mortality.
RESULTS
A total of 64 patients with PPM were included, with a mean age of 39.2 ± 15.6 years and minor male dominance (40, 62.5%). The most common echocardiographic presentations were pericardial effusion (55, 85.9%), pericardial masses (36.4%) and thickening (17.3%), respectively. The positive rate of pericardiocentesis was only 20.9%. Six patients (15.4%) died among 39 cases reporting in-hospital outcome. Logistics analysis identified no clinical or echocardiographic parameters associated with in-hospital mortality (all P > 0.05).
CONCLUSIONS
The echocardiographic signs of PPM are basically nonspecific with massive pericardial effusion as the most common sign, although no echocardiographic gender differences or association with in-hospital mortality could be identified.
Topics: Adolescent; Adult; Aged; Child; Child, Preschool; China; Female; Heart Neoplasms; Hospital Mortality; Humans; Male; Mesothelioma; Middle Aged; Pericardial Effusion; Retrospective Studies; Sex Factors; Young Adult
PubMed: 29695235
DOI: 10.1186/s12947-018-0125-z -
Frontiers in Veterinary Science 2023Canine mesothelioma is a rare malignant tumor that mostly affects body cavities, such as the pericardial and pleural cavities. Chemotherapy plays a crucial role in the...
INTRODUCTION
Canine mesothelioma is a rare malignant tumor that mostly affects body cavities, such as the pericardial and pleural cavities. Chemotherapy plays a crucial role in the treatment of canine mesotheliomas. We aimed to compare the antitumor effects of single-agent and combination chemotherapeutic agents on patient-derived primary cultures of canine pericardial mesothelioma established in this study. We planned to generate xenograft models for future studies.
MATERIAL AND METHODS
Effusion samples were collected from three dogs with histologically diagnosed pericardial mesothelioma and used for primary culture. Cultured cells were characterized by immunostaining for pan-cytokeratin AE1/AE3, vimentin, Wilms' tumor suppressor gene 1 (WT1), and cytokeratin 5 (CK5). To assess the tumorigenic properties of cells in the effusion and generate a xenograft model, the cell suspension was injected into a severe combined immunodeficient (SCID) mouse either subcutaneously (SC) or intraperitoneally (IP). Lastly, chemosensitivity of established primary cultures against four drugs, doxorubicin, vinorelbine, carboplatin, and gemcitabine, by single-agent treatment as well as combination treatment of carboplatin at a fixed concentration, either 10 or 100 μM, and gemcitabine at different concentrations ranging from 0-1000 μM was assessed by cell viability assay.
RESULTS
Primary cultures were successfully generated and characterized by dual positivity for AE1/AE3 and vimentin and positive staining for WT-1 and CK5, confirming the mesothelial origin of the cells. In the xenograft models, SC mouse developed a subcutaneous mass, whereas IP mouse developed multiple intraperitoneal nodules. The masses were histopathologically consistent with mesotheliomas. The chemosensitivity assay revealed that carboplatin had the highest anti-tumor effects among the four tested single-agent treatments. Furthermore, carboplatin at 100 μM combined with gemcitabine at clinically relevant doses demonstrated the augmented anti-tumor effects compared to single-agent treatment.
DISCUSSION AND CONCLUSION
Primary cultures and xenograft models generated in this study could be useful tools for and studies of canine mesothelioma. Carboplatin is a highly effective chemotherapeutic agent against canine mesothelioma when used as a sole agent and in combination with gemcitabine.
PubMed: 38026668
DOI: 10.3389/fvets.2023.1267359 -
Expert Review of Respiratory Medicine Dec 2019: The surgical arm of the Mesothelioma and Radical Surgery (MARS) trial involved a multimodal approach, with combined therapy consisting of chemotherapy, complete gross... (Review)
Review
: The surgical arm of the Mesothelioma and Radical Surgery (MARS) trial involved a multimodal approach, with combined therapy consisting of chemotherapy, complete gross resection, and radiation therapy. However, the MARS trial did not compare surgery with chemotherapy, and the survival and quality of life outcomes of this trial's surgical arm were inferior to those of the non-surgical arm. Methods for achieving complete gross resection (macroscopic complete response [MCR]) include extrapleural pneumonectomy (EPP), wherein the pleura, lung, diaphragm, and pericardium are removed en bloc, and pleurectomy/decortication (P/D), wherein the affected lung is preserved. Nonetheless, the most effective therapy remains unclear.: Here, surgery post-neoadjuvant chemotherapy for malignant pleural mesothelioma with either EPP or P/D has been discussed, along with trimodal and bimodal therapies.: With the development of post-P/D radiation therapy, it is currently possible to truly compare EPP with P/D. Moreover, R0 resection cannot be achieved with either EPP or P/D; thus, both must incorporate debulking, although the two procedures are largely incompatible. Therefore, there is a need to rebuild the status of surgery as a multimodal therapy.
Topics: Combined Modality Therapy; Humans; Mesothelioma; Neoadjuvant Therapy; Pleural Neoplasms; Pneumonectomy; Quality of Life; Thoracic Surgical Procedures; Treatment Outcome
PubMed: 31596628
DOI: 10.1080/17476348.2019.1679119