-
Journal of Cardiology Cases Oct 2021Primary malignant pericardial mesothelioma is an extremely rare disease. Malignant disease of the pericardium is an infrequent cause of cardiac tamponade. Hence, cardiac...
Primary malignant pericardial mesothelioma is an extremely rare disease. Malignant disease of the pericardium is an infrequent cause of cardiac tamponade. Hence, cardiac tamponade in the context of primary malignant mesothelioma of the pericardium is an uncommon clinical scenario. A 67-year-old male patient, an ex-smoker, complaining of progressive lethargy was referred to a hospital for investigation of persistent pericardial effusion. The pericardial fluid cytology was categorized as class Ⅲ. Thereafter, he was referred to our hospital for further evaluation. Fluorodeoxyglucose (FDG) positron emission tomography (PET) revealed FDG accumulation in the pericardium and mediastinal lymph node. Surgical biopsy of the pericardium was performed through a subxiphoid approach for a definitive diagnosis. Histopathological examination revealed diffuse infiltration of the pericardium by a malignant tumor consisting of epithelioid cells with large round nuclei and prominent nucleoli, arranged in a tubular papillary pattern. Finally, the patient was diagnosed with primary malignant pericardial mesothelioma of epithelioid type. The patient died 6 weeks after admission. This diagnosis must be considered in patients having unexplained massive pericardial effusion. Furthermore, we should consider prompt cytological analysis and FDG PET to arrive rapidly at a definitive diagnosis to administer combination chemotherapy that may provide clinical benefit. < Primary malignant pericardial mesothelioma is a rare disease. Malignant disease of the pericardium is an infrequent cause of cardiac tamponade. Hence, cardiac tamponade in the context of primary malignant mesothelioma of the pericardium is an uncommon clinical scenario. However, this diagnosis must be considered in patients having unexplained massive pericardial effusion. Furthermore, we should consider prompt cytological analysis and fluorodeoxyglucose positron emission tomography to arrive rapidly at a definitive diagnosis to administer combination chemotherapy that may provide clinical benefit.>.
PubMed: 35059046
DOI: 10.1016/j.jccase.2021.03.002 -
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 -
Cancer Cytopathology Aug 2019The cytomorphologic evaluation of serous cavity specimens can quickly determine the cause of an effusion as well as the presence or absence of a neoplastic process.... (Review)
Review
The cytomorphologic evaluation of serous cavity specimens can quickly determine the cause of an effusion as well as the presence or absence of a neoplastic process. Ancillary tests such as immunohistochemistry and fluorescence in situ hybridization are frequently used to help to definitively identify malignant cells, determine a site of origin, and distinguish between malignant and reactive mesothelial proliferations. In recent years, microRNAs (miRNAs) have been increasingly evaluated in cytologic specimens, including those from serous cavities. The examination of miRNA is attractive because of the stability of miRNA in such specimens and data suggesting that miRNA can provide prognostic and therapeutic information in addition to its role in diagnosis. Furthermore, miRNAs exist within extracellular exosomes, and this allows for their analysis in specimens that contain only rare malignant cells, degenerated cells, or obscuring components. This review discusses the technical aspects of specimen processing for miRNA analysis, recent studies of miRNA expression in malignant serous cavity specimens, and the potential importance of miRNA expression analysis for serous cavity specimens in the near future.
Topics: Ascitic Fluid; Biomarkers, Tumor; Diagnosis, Differential; Exosomes; Heart Neoplasms; Humans; MicroRNAs; Pericardial Effusion; Peritoneal Neoplasms; Pleural Effusion, Malignant; RNA Stability
PubMed: 31154675
DOI: 10.1002/cncy.22143 -
The Pan African Medical Journal 2020Metastases to the heart and pericardium are much more common than primary malignant neoplasms. Primary malignant pericardial mesothelioma is a rare tumor that arises...
Metastases to the heart and pericardium are much more common than primary malignant neoplasms. Primary malignant pericardial mesothelioma is a rare tumor that arises from the mesothelial cells of the pericardium. It is usually characterized by a delayed diagnosis, a low response to treatment, and a poor prognosis with an overall survival up to six months after the onset of symptoms. We report a rare case of a 32-year-old woman with primary pericardial malignant mesothelioma that was diagnosed 4 months after the onset of pericardial effusion as the first clinical manifestation.
Topics: Adult; Delayed Diagnosis; Diagnosis, Differential; Female; Heart Neoplasms; Humans; Mesothelioma, Malignant; Pericardial Effusion; Pericarditis; Pericardium; Radiography, Thoracic; Tachycardia
PubMed: 33235652
DOI: 10.11604/pamj.2020.36.375.25336 -
Journal For Immunotherapy of Cancer Feb 2021CD40 agonist immunotherapy can potentially license antigen-presenting cells to promote antitumor T-cell activation and re-educate macrophages to destroy tumor stroma....
BACKGROUND
CD40 agonist immunotherapy can potentially license antigen-presenting cells to promote antitumor T-cell activation and re-educate macrophages to destroy tumor stroma. Systemic administration of CD40 agonists has historically been associated with considerable toxicity, providing the rationale for development of tumor-targeted immunomodulators to improve clinical safety and efficacy. This phase I study assessed the safety, tolerability, preliminary antitumor activity, and preliminary biomarkers of ABBV-428, a first-in-class, mesothelin-targeted, bispecific antibody designed for tumor microenvironment-dependent CD40 activation with limited systemic toxicity.
METHODS
ABBV-428 was administered intravenously every 2 weeks to patients with advanced solid tumors. An accelerated titration (starting at a 0.01 mg/kg dose) and a 3+3 dose escalation scheme were used, followed by recommended phase II dose cohort expansions in ovarian cancer and mesothelioma, tumor types associated with high mesothelin expression.
RESULTS
Fifty-nine patients were treated at doses between 0.01 and 3.6 mg/kg. The maximum tolerated dose was not reached, and 3.6 mg/kg was selected as the recommended phase II dose. Seven patients (12%) reported infusion-related reactions. Treatment-related grade ≥3 treatment-emergent adverse events were pericardial effusion, colitis, infusion-related reaction, and pleural effusion (n=1 each, 2%), with no cytokine release syndrome reported. The pharmacokinetic profile demonstrated roughly dose-proportional increases in exposure from 0.4 to 3.6 mg/kg. Best response was stable disease in 9/25 patients (36%) treated at the recommended phase II dose. CD40 receptor occupancy >90% was observed on peripheral B-cells starting from 0.8 mg/kg; however, no consistent changes from baseline in intratumoral CD8+ T-cells, programmed death ligand-1 (PD-L1+) cells, or immune-related gene expression were detected post-ABBV-428 treatment (cycle 2, day 1). Mesothelin membrane staining showed greater correlation with progression-free survival in ovarian cancer and mesothelioma than in the broader dose escalation population.
CONCLUSIONS
ABBV-428 monotherapy exhibited dose-proportional pharmacokinetics and an acceptable safety profile, particularly for toxicities characteristic of CD40 agonism, illustrating that utilization of a tumor-targeted, bispecific antibody can improve the safety of CD40 agonism as a therapeutic approach. ABBV-428 monotherapy had minimal clinical activity in dose escalation and in a small expansion cohort of patients with advanced mesothelioma or ovarian cancer.
TRIAL REGISTRATION NUMBER
NCT02955251.
Topics: Adult; Aged; Aged, 80 and over; Antibodies, Bispecific; Antineoplastic Agents, Immunological; CD40 Antigens; Female; France; Humans; Male; Maximum Tolerated Dose; Mesothelin; Middle Aged; Neoplasms; Progression-Free Survival; Time Factors; Tumor Microenvironment; United States
PubMed: 33608377
DOI: 10.1136/jitc-2020-002015 -
Frontiers in Cardiovascular Medicine 2023Primary pericardial mesothelioma (PPM) is an exceedingly rare malignant cancer and has a poor prognosis, which has been partly attributed to its frequently delayed...
BACKGROUND
Primary pericardial mesothelioma (PPM) is an exceedingly rare malignant cancer and has a poor prognosis, which has been partly attributed to its frequently delayed diagnosis due to its nonspecific syndromes, its similar presentation to benign pericardial diseases, and its non-definitive etiology. In many PPM cases, the time from presentation to definite diagnosis may last for several months or even over one year. Unlike pleural mesothelioma, the relationship between PPM and asbestos exposure remains unsettled. To date, there is no consensus on the treatment of PPM.
CASE REPORT
The patient is a 57-year-old male who had nonspecific syndromes and inconclusive image findings. The occupational long-term asbestos exposure history of this patient raised our concerns regarding potential malignancy when confronted with unexplained pericardial effusion accompanied by cardiac tamponade. The heightened suspicion prompted us to perform pericardiocentesis and biopsy on the third day after admission to our department. An early diagnosis of PPM was established by the pathological and immunohistochemical evaluation of the biopsy specimen two weeks after admission. Positron emission tomography-computed tomography revealed that the lesion was localized at the anterior part of the mediastinum without distant metastasis. This patient refused to receive cardiac surgery. He subsequently underwent six cycles of chemotherapy (cisplatin plus pemetrexed) in combination with bevacizumab (a humanized anti-VEGF antibody) as the first-line treatment, resulting in complete relief of symptoms and satisfactory outcomes with no complications. Four months after the first course, the patient initiated a second course of chemotherapy with a similar regimen, but he opted to discontinue the medical treatment after the initiation of the second course. The patient was transferred to the hospice care unit and unfortunately expired one year after the initial presentation.
CONCLUSION
We present a case of an early multidisciplinary clinical approach to diagnose and manage PPM with consideration of occupational asbestos exposure history and clinical symptoms. Bevacizumab-based chemotherapy remains an option for the treatment of PPM.
PubMed: 38054089
DOI: 10.3389/fcvm.2023.1257373 -
International Journal of Surgery Case... 2020Primary malignant pericardial mesothelioma is a rare tumor that is very difficult to diagnose. Furthermore, it is a lethal disease, because patients usually have...
INTRODUCTION
Primary malignant pericardial mesothelioma is a rare tumor that is very difficult to diagnose. Furthermore, it is a lethal disease, because patients usually have progressed at the time of referral.
PRESENTATION OF THE CASE
We report a 44-year-old man with primary malignant pericardial mesothelioma. He was referred to our hospital for the diagnosis and treatment of a massive pericardial effusion and huge tumor. Pericardiocentesis was performed, but we could not obtain definitive diagnosis, and the cardiac tamponade continued along with the signs/symptoms. He required surgical intervention for the diagnosis and treatment. After surgery, his signs/symptoms improved. He received adjuvant therapy, although he died 7 months after surgery.
DISCUSSION
Primary malignant pericardial mesothelioma is a rare tumor. The most common signs and symptoms are related to constriction of the heart by the tumor and/or effusion. Even if the pericardial fluid specimen obtained by pericardiocentesis is negative for malignant cells, primary malignant pericardial mesothelioma should be included in the differential diagnosis. Because the malignancy is usually advanced at the time of diagnosis, it has been difficult to cure. Radiation and chemotherapy have been used in addition to surgery, but have been minimally effective.
CONCLUSION
The outcome of our patient with pericardial malignant mesothelioma was dismal. The indications for surgical intervention should be carefully considered except for critical cases requiring alleviation of immediate life-threating conditions.
PubMed: 32717680
DOI: 10.1016/j.ijscr.2020.07.054 -
Case Reports in Rheumatology 2022Pericardial effusion or the accumulation of fluid in the pericardial sac, can result from infectious, malignant, or autoimmune processes such as systemic lupus...
Pericardial effusion or the accumulation of fluid in the pericardial sac, can result from infectious, malignant, or autoimmune processes such as systemic lupus erythematous (SLE). However, pericardial effusion is infrequently the first presentation of SLE. Here, we describe the case of a 54-year-old African American woman who presented with hypertensive emergency and was found to have pericardial effusion on echocardiogram. Her hypertensive symptoms resolved with medical management and a work up were positive for serum markers of SLE and mesothelioma cell markers (calretinin, CK 5/6) and adenocarcinoma marker MOC31 in the pericardial fluid. Her effusion ultimately improved on high-dose steroid therapy and has not recurred in one year. Given normal pleura and pericardium on computed tomography (CT) imaging and long-term clinical improvement in SLE therapy, we hypothesize that she had false-positive mesothelioma markers in the setting of SLE.
PubMed: 36387931
DOI: 10.1155/2022/8081055 -
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 -
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