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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 -
Revista Espanola de Anestesiologia Y... Mar 2023Extrapleural pneumonectomy, usually associated with pericardial and diaphragmatic reconstruction with prosthetic material, is one of the surgical techniques used in the...
Extrapleural pneumonectomy, usually associated with pericardial and diaphragmatic reconstruction with prosthetic material, is one of the surgical techniques used in the treatment of malignant pleural mesothelioma. Herniation of the abdominal viscera towards the thorax through the prosthetic material at the diaphragmatic level is a rare but potentially serious complication of these procedures, which must be diagnosed quickly for urgent repair. We present the case of a patient who presented with gastric herniation in the early postoperative period of a left pneumonectomy due to pleural mesothelioma. The clinical findings were mild, but supported by imaging tests, they confirmed the diagnostic hypothesis and facilitated the solution of the condition. Possible contributing factors are reviewed and the need for early diagnosis and treatment is emphasized to avoid ischemia of herniated abdominal viscera in the thoracic cavity, due to the risk of necrosis and contamination by fecaloid material.
Topics: Humans; Mesothelioma, Malignant; Pneumonectomy; Mesothelioma; Pleural Neoplasms; Prostheses and Implants
PubMed: 36842686
DOI: 10.1016/j.redare.2021.11.009 -
Current Oncology Reports Apr 2023This review aims to summarise the pathobiological role of mesothelin and the current data on therapeutic antibodies targeting mesothelin in solid tumours. (Review)
Review
PURPOSE OF REVIEW
This review aims to summarise the pathobiological role of mesothelin and the current data on therapeutic antibodies targeting mesothelin in solid tumours.
RECENT FINDINGS
High mesothelin expression is restricted to the pericardium, pleura, peritoneum and tunica vaginalis. Mesothelin does not seem to have any normal biological function in adult normal tissues. Mesothelin is highly expressed in mesothelioma, serous ovarian cancer, pancreatic cancer and some gastric cancer and adenocarcinoma of the lung and is responsible for tumour proliferation, metastasis, resistance to chemotherapy or radiation and evasion of immune system. To date, antibody, antibody drug conjugates and bispecific antibodies with immune checkpoints have been investigated in mesothelin expressing malignancies. After a couple of decades of clinical investigation in antibody targeting mesothelin, the therapeutic benefit is relatively modest. Novel delivery of mesothelin targeting agents, more potent payload in antibody drug conjugates and immune checkpoint inhibitor, may improve therapeutic benefit.
Topics: Humans; Adenocarcinoma; GPI-Linked Proteins; Immunoconjugates; Pharmaceutical Preparations; Mesothelin
PubMed: 36763234
DOI: 10.1007/s11912-023-01367-8 -
Radiographics : a Review Publication of... Mar 2023A diverse spectrum of benign entities and malignant neoplasms originate from the monotonous mesothelium that lines the serosal membranes of the pleural, pericardial, and...
A diverse spectrum of benign entities and malignant neoplasms originate from the monotonous mesothelium that lines the serosal membranes of the pleural, pericardial, and peritoneal cavities. The mesothelium of myriad sites shows a common origin from the lateral plate mesoderm; primary mesothelial tumors thus demonstrate similar pathogenesis, imaging findings, and treatment options. Significant changes have been made in the 2021 World Health Organization (WHO) classification schemata of the pleural and pericardial tumors on the basis of recent advances in pathology and genetics. While malignant mesotheliomas are biologically aggressive malignancies that occur primarily in patients exposed to asbestos with attendant poor survival rates, well-differentiated papillary mesothelial tumors and adenomatoid tumors charter a benign clinical course with an excellent prognosis. Mesothelioma in situ is a newly characterized entity represented by recurrent unexplained pleural effusions without any identifiable mass at imaging or thoracoscopy. Immunohistochemical markers based on , and gene mutations help differentiate diffuse mesotheliomas from benign mesothelial proliferations and localized mesotheliomas. Cross-sectional imaging modalities, including US, CT, MRI, and fluorine 18-fluorodeoxyglucose (FDG) PET/CT, permit diagnosis and play a major role in staging and assessing surgical resectability. Imaging studies are invaluable in providing noninvasive and quantitative assessment of tumor response in patients with unresectable disease. Owing to significant overlap in patient characteristics and pathomorphology, accurate diagnosis based on advanced histopathology techniques and genetic abnormalities is imperative for optimal management and prognostication. While patients with nonepithelioid pleural mesotheliomas benefit from immunotherapy, novel targeted therapies for -, -, and -altered mesotheliomas are under consideration. RSNA, 2023 Quiz questions for this article are available through the Online Learning Center.
Topics: Humans; Adenomatoid Tumor; Positron Emission Tomography Computed Tomography; Mesothelioma; Mesothelioma, Malignant; Pleural Neoplasms; Neoplasms, Mesothelial; Biomarkers, Tumor
PubMed: 36757881
DOI: 10.1148/rg.220128 -
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 -
Proceedings of the National Academy of... Jan 2023is a powerful tumor suppressor gene characterized by haplo insufficiency. Individuals carrying germline mutations often develop mesothelioma, an aggressive malignancy...
is a powerful tumor suppressor gene characterized by haplo insufficiency. Individuals carrying germline mutations often develop mesothelioma, an aggressive malignancy of the serosal layers covering the lungs, pericardium, and abdominal cavity. Intriguingly, mesotheliomas developing in carriers of germline mutations are less aggressive, and these patients have significantly improved survival. We investigated the apparent paradox of a tumor suppressor gene that, when mutated, causes less aggressive mesotheliomas. We discovered that mesothelioma biopsies with biallelic mutations showed loss of nuclear HIF-1α staining. We demonstrated that during hypoxia, binds, deubiquitylates, and stabilizes HIF-1α, the master regulator of the hypoxia response and tumor cell invasion. Moreover, primary cells from individuals carrying germline mutations and primary cells in which was silenced using siRNA had reduced HIF-1α protein levels in hypoxia. Computational modeling and co-immunoprecipitation experiments revealed that mutations of residues I675, F678, I679, and L691 -encompassing the C-terminal domain-nuclear localization signal- to A, abolished the interaction with HIF-1α. We found that binds to the N-terminal region of HIF-1α, where HIF-1α binds DNA and dimerizes with HIF-1β forming the heterodimeric transactivating complex HIF. Our data identify as a key positive regulator of HIF-1α in hypoxia. We propose that the significant reduction of HIF-1α activity in mesothelioma cells carrying biallelic mutations, accompanied by the significant reduction of HIF-1α activity in hypoxic tissues containing germline mutations, contributes to the reduced aggressiveness and improved survival of mesotheliomas developing in carriers of germline mutations.
Topics: Humans; Heterozygote; Hypoxia-Inducible Factor 1, alpha Subunit; Mesothelioma; Mesothelioma, Malignant; Mutation; Tumor Suppressor Proteins; Ubiquitin Thiolesterase
PubMed: 36656861
DOI: 10.1073/pnas.2217840120 -
The Heart Surgery Forum Dec 2022Primary malignant pericardial mesothelioma (PMPM) is an extremely rare and lethal cardiac tumor. This article presents a 62-year-old man with recurrent pericardial...
Primary malignant pericardial mesothelioma (PMPM) is an extremely rare and lethal cardiac tumor. This article presents a 62-year-old man with recurrent pericardial fluid. The patient's clinical symptoms and imaging features were nonspecific. Initial diagnosis was constrictive pericarditis. After admission, the patient's symptoms worsened, and echocardiography indicated increased pericardial effusion. To diagnose and improve the patient's symptoms, pericardiotomy was performed; however, the procedure was unsuccessful because the pericardium was densely adherent to the myocardium. Histopathological examination, including immunohistochemical staining of the pericardial specimen revealed malignant mesothelioma. We recommended adjuvant therapy for the patient with cis-platinum and pemetrexed; however, the patient and his family refused treatment. The patient was discharged 11 days after surgery. The patient survived for more than 15 months with surgical treatment. In this report, the patient's symptoms improved, and the patient survived beyond the median survival after surgical treatment. Conclusion: The definitive diagnosis of PMPM mostly has been obtained from specimens obtained by surgery. Surgery is an effective treatment method because it prevents cardiac tamponade and can improve symptoms or prognosis, but complete resection is impossible.
Topics: Male; Humans; Middle Aged; Mesothelioma, Malignant; Pericardiectomy; Mesothelioma; Pericardium; Pericardial Effusion; Heart Neoplasms
PubMed: 36602504
DOI: 10.1532/hsf.5047 -
World Journal of Clinical Cases Nov 2022Primary malignant pericardial mesothelioma (PMPM) is an extremely rare malignant tumor, and it is difficult to diagnose definitively before death. We present a case in...
BACKGROUND
Primary malignant pericardial mesothelioma (PMPM) is an extremely rare malignant tumor, and it is difficult to diagnose definitively before death. We present a case in which PMPM was diagnosed at autopsy. We consider this case to be highly suggestive and report it here.
CASE SUMMARY
A 78-year-old male presented with transient loss of consciousness and falls. The transient loss of consciousness was considered to result from complications of diastolic dysfunction due to pericardial disease, fever with dehydration, and paroxysmal atrial fibrillation. Ultrasound cardiography (UCG) and computed tomography showed cardiac enlargement and high-density pericardial effusion. We considered pericardial disease to be the main pathogenesis of this case. Cardiac magnetic resonance imaging and gadolinium contrast-enhanced T1-weighted images showed thick staining inside and outside the pericardium. Pericardial biopsy was considered to establish a definitive diagnosis, but the patient and his family refused further treatment and examinations, and the patient was followed conservatively. We noticed a thickening of the pericardium and massive changes in the pericardium on UCG over time. We performed an autopsy 60 h after the patient died of pneumonia. Giemsa staining of the autopsy tissue showed an epithelial-like arrangement in the pericardial tumor, and immunostaining showed positive and negative factors for the diagnosis of PMPM. Based on these findings, the final diagnosis of PMPM was made.
CONCLUSION
PMPM has a poor prognosis, and early diagnosis and treatment are important. The temporal echocardiographic findings may provide a clue for the diagnosis of PMPM.
PubMed: 36483836
DOI: 10.12998/wjcc.v10.i33.12380 -
Cancers Nov 2022Malignant Mesothelioma (MM) is an aggressive neoplasm of the pleural mesothelium, less frequently peritoneal and exceptionally of the vaginal tunic of the testicle and...
Malignant Mesothelioma (MM) is an aggressive neoplasm of the pleural mesothelium, less frequently peritoneal and exceptionally of the vaginal tunic of the testicle and pericardium [...].
PubMed: 36428633
DOI: 10.3390/cancers14225540 -
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