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Clinical Case Reports Jun 2023Pleural mesothelioma (PM) with pericardial involvement is extremely rare. We now report a rare case of malignant PM with constrictive pericarditis as the first...
Pleural mesothelioma (PM) with pericardial involvement is extremely rare. We now report a rare case of malignant PM with constrictive pericarditis as the first presentation. A 59-year-old male diagnosed with constrictive pericarditis underwent pericardiectomy and pericardial pathology revealed mesothelial hyperplasia. Eight months after surgery, the patient was admitted to the hospital with chest tightness and wheezing for 5 days. Computed tomography scan of the chest showed a left lung expansion insufficiency, limited bilateral pleural thickening, pericardial thickening with a small amount of pericardial effusion, and multiple enlarged lymph nodes in the mediastinum, bilateral supraclavicular fossa, bilateral cervical roots, and right axilla. The pleural malignancy should be possibly considered. Pathology after pleural puncture showed malignant PM. Pathology after left supraclavicular lymph node puncture biopsy showed metastatic malignant mesothelioma. The diagnosis of this patient was clear. Although malignant PM rarely involves the pericardial constriction, we cannot ignore the fact that malignant PM involves the pericardium. The patient has been diagnosed with constrictive pericarditis, accompanied by pleural thickening and pleural effusion. Without other pathogenic factors, pleural biopsy should be aggressively performed in patients with constrictive pericarditis to determine the cause.
PubMed: 37351350
DOI: 10.1002/ccr3.7555 -
JFMS Open Reports 2023A 6.4 kg 3-year-old male neutered indoor-outdoor domestic shorthair cat was referred for further evaluation of non-resolving lethargy and hyporexia of 4 days'...
CASE SUMMARY
A 6.4 kg 3-year-old male neutered indoor-outdoor domestic shorthair cat was referred for further evaluation of non-resolving lethargy and hyporexia of 4 days' duration. Physical examination identified tachypnea with mild respiratory effort and muffled lung sounds bilaterally. Point-of-care ultrasound revealed a large volume of pleural and pericardial effusion (PCE), which was confirmed by thoracic radiography. Echocardiogram indicated normal cardiac function but revealed a mass-like structure along the left epicardium within the pericardial space. After 72 hours in hospital, re-evaluation via echocardiogram showed the epicardial mass lesion to have doubled in size and with apparent extension to involve the pericardium. The patient was hospitalized for 72 h of supportive care and intervention, including therapeutic pericardiocentesis, bilateral thoracocentesis, thoracic and cardiac imaging and infectious disease testing. On the third day of hospitalization, the patient developed cardiac tamponade. Further workup was discussed, including CT and subtotal pericardiectomy with biopsy, but the cat was euthanized due to clinical decline and rapid re-accumulation of effusion. Post-mortem histopathologic evaluation diagnosed an epicardial pleomorphic sarcoma, exclusive of mesothelioma or histiocytic sarcoma on immunohistochemistry (IHC).
RELEVANCE AND NOVEL INFORMATION
This report describes a case of epicardial undifferentiated pleomorphic sarcoma (UPS) in a young cat presenting with pleural and PCE. Pleomorphic sarcoma is a rarely reported mesenchymal neoplasia in the feline patient and has thus far primarily been identified in peripheral soft tissue structures. IHC is key to the correct histopathologic diagnosis. To our knowledge, epicardial UPS has not been previously reported in a cat.
PubMed: 37168487
DOI: 10.1177/20551169231162484 -
European Heart Journal. Case Reports Mar 2023Primary pericardial mesothelioma is an extremely rare disease. Prognosis is poor, with little effects of chemo- or radio-therapy. The majority of cases is diagnosed at...
BACKGROUND
Primary pericardial mesothelioma is an extremely rare disease. Prognosis is poor, with little effects of chemo- or radio-therapy. The majority of cases is diagnosed at autopsy.
CASE SUMMARY
A 22-year-old man, who presented with recurrent pericarditis and large pericardial effusion 2 months after a second BNT162b2 COVID-19 vaccine, underwent pericardiocentesis and pericardial window. Pathology specimen of pericardium revealed benign mesothelial inflammation, consistent with acute pericarditis. Four months later, he presented with a large pericardial mass manifesting in heart failure and underwent urgent pericardiectomy. A new pathology specimen immunostaining and fluorescence hybridization analysis revealed pericardial mesothelioma. Despite intensive care, the patient died 3 weeks later.
DISCUSSION
Primary pericardial mesothelial should be considered in the differential diagnosis of refractory recurrent pericarditis, even with prior biopsy-proven pericarditis or when a putative trigger (COVID-19 mRNA prior vaccination) is suspected, as was the case in this patient. Tumour diagnosis and identification consist of multimodal imaging and laboratory tests. A multidisciplinary, individualized care approach should be performed.
PubMed: 36937239
DOI: 10.1093/ehjcr/ytad100 -
Quantitative Imaging in Medicine and... Mar 2023
PubMed: 36915356
DOI: 10.21037/qims-22-556 -
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 -
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 -
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