-
Frontiers in Oncology 2022Mesothelioma is a rare malignant neoplasm with poor survival. It mainly affects the pleura (90%) but can arise in all serous cavities: peritoneum (5-10%), pericardium... (Review)
Review
Mesothelioma is a rare malignant neoplasm with poor survival. It mainly affects the pleura (90%) but can arise in all serous cavities: peritoneum (5-10%), pericardium and tunica vaginalis testis (<1%). The onset of pleural mesothelioma is strictly related to asbestos exposure with a long latency time. The causal link with asbestos has also been suggested for peritoneal mesothelioma, while the importance of exposure in the onset of pericardial and tunica vaginalis testis mesotheliomas is not well known. Mesothelioma remains an aggressive and fatal disease with a five-year mortality rate higher than 95%. However, new therapeutic approaches based on molecular-targeted and immunomodulatory therapies are being explored but have conflicting results. In this context, the identification of critical targets appears mandatory. Awareness of the molecular and physiological changes leading to the neoplastic degeneration of mesothelial cells and the identification of gene mutations, epigenetic alterations, gene expression profiles and altered pathways could be helpful for selecting targetable mechanisms and molecules. In this review, we aimed to report recent research in the last 20 years focusing on the molecular pathways and prognostic factors in peritoneal mesothelioma and their possible diagnostic and therapeutic implications.
PubMed: 35223506
DOI: 10.3389/fonc.2022.823839 -
Interactive Cardiovascular and Thoracic... Jun 2022Mediastinal malignant pleural mesothelioma with signs of tamponade is rare. Indication for reoperation for recurrent malignant pleural mesothelioma is a controversial...
Mediastinal malignant pleural mesothelioma with signs of tamponade is rare. Indication for reoperation for recurrent malignant pleural mesothelioma is a controversial but viable option in selected patients. We report a case of a 68-year-old man presenting with epithelioid malignant pleural mesothelioma who underwent a total of three debulking surgeries (pleurectomy/decortication) combined with hyperthermic intrathoracic chemotherapy. Five years after the first procedure, a third urgent operation was performed for recurrence of a large mediastinal mesothelioma causing acute symptoms of pericardial constriction and tamponade. The patient was alive for eight years since the first treatment and 36 months after the second reoperation.
Topics: Aged; Combined Modality Therapy; Cytoreduction Surgical Procedures; Humans; Male; Mediastinum; Mesothelioma; Mesothelioma, Malignant; Neoplasm Recurrence, Local; Pleural Neoplasms; Treatment Outcome
PubMed: 35218349
DOI: 10.1093/icvts/ivac034 -
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 Thoracic Oncology : Official... May 2022Substantial changes in the 2021 WHO Classification of Tumors of the Pleura and Pericardium since the 2015 WHO Classification include the following: (1) pleural and... (Review)
Review
Substantial changes in the 2021 WHO Classification of Tumors of the Pleura and Pericardium since the 2015 WHO Classification include the following: (1) pleural and pericardial tumors have been combined in one chapter whereas in the 2015 WHO, pericardial tumors were classified with cardiac tumors; (2) well-differentiated papillary mesothelioma has been renamed well-differentiated papillary mesothelial tumor given growing evidence that these tumors exhibit relatively indolent behavior; (3) localized and diffuse mesothelioma no longer include the term "malignant" as a prefix; (4) mesothelioma in situ has been added to the 2021 classification because these lesions can now be recognized by loss of BAP1 and/or MTAP by immunohistochemistry and/or CDKN2A homozygous deletion by fluorescence in situ hybridization; (5) the three main histologic subtypes (i.e., epithelioid, biphasic, and sarcomatoid) remain the same but architectural patterns and cytologic and stromal features are more formally incorporated into the 2021 classification on the basis of their prognostic significance; (6) nuclear grading for epithelioid diffuse mesothelioma is introduced, and it is recommended to record this and other histologically prognostic features in pathology reports; (7) BAP1, EZH2, and MTAP immunohistochemistry have been found to be useful in separating benign mesothelial proliferations from mesothelioma; (8) biphasic mesothelioma can be diagnosed in small biopsies having both epithelioid and sarcomatoid components even if the amount of one component is less than 10%; and (9) the most frequently altered genes in diffuse pleural mesothelioma include BAP1, CDKN2A, NF2, TP53, SETD2, and SETDB1.
Topics: Biomarkers, Tumor; Homozygote; Humans; In Situ Hybridization, Fluorescence; Lung Neoplasms; Mesothelioma; Mesothelioma, Malignant; Pleura; Pleural Neoplasms; Sequence Deletion; Tumor Suppressor Proteins; Ubiquitin Thiolesterase; World Health Organization
PubMed: 35026477
DOI: 10.1016/j.jtho.2021.12.014 -
Journal of Thoracic Disease Nov 2021This observational study evaluates retrospectively the long-term outcomes after pleurectomy/decortication for pleural mesothelioma, with and without the...
BACKGROUND
This observational study evaluates retrospectively the long-term outcomes after pleurectomy/decortication for pleural mesothelioma, with and without the resection/reconstruction of diaphragm and pericardium.
METHODS
Data from 155 consecutive patients undergoing lung-sparing surgery for epithelial pleural mesothelioma were reviewed. Selection criteria for surgery were cT1-3, cN0-1, good performance status, age <80 years. Perioperative Pemetrexed-Platinum regimen was administered as induction in 101 cases (65.2%) and as adjuvant treatment in 54 cases (34.8%). Extended pleurectomy/decortication was performed in 87 cases (56.12%). In 68 patients (43.87%) standard pleurectomy/decortication was performed without resection/reconstruction of diaphragm and pericardium, when tumour infiltration was deemed absent after intraoperative frozen section. The log-rank test and Cox regression model were used to assess the factors affecting overall survival and recurrence free survival.
RESULTS
Median follow-up was 20 months. The 2- and 5-year survival rate was 60.9% and 29.2% with a median survival of 34 months. An improved survival was observed when standard pleurectomy/decortication was carried out (P=0.007). A significant impact on survival was found comparing the TNM-stages (P=0.001), pT (P=0.002) and pN variables (P=0.001). Multivariate analysis identified the pN-status (P=0.003) and standard pleurectomy/decortication (P=0.017) as predictive for longer survival. The recurrence-free survival >12 months was strongly related to the overall survival (P<0.001). The macroscopic complete resection (P=0.001), TNM-stage (P=0.003) and pT-status (P=0.001) are related to relapse.
CONCLUSIONS
Within multimodal management of pleural mesothelioma, lung-sparing surgery is a valid option even with more conservative technique. A benefit for a longer survival was observed in the early stage of disease, with pN0 and when pleurectomy/decortication is carried out, preserving diaphragm and pericardium. Recurrence is not affected by the type of surgery, and a recurrence-free interval >12 months is predictive of an increased survival when the macroscopic complete resection is achieved.
PubMed: 34992808
DOI: 10.21037/jtd-21-691 -
Cureus Nov 2021Primary pericardial mesothelioma (PPM) is an extremely rare malignancy with a very poor prognosis. It poses a diagnostic challenge given its often late and non-specific...
Primary pericardial mesothelioma (PPM) is an extremely rare malignancy with a very poor prognosis. It poses a diagnostic challenge given its often late and non-specific presentation. This report describes a 74-year-old man who presented with central pleuritic chest pain and mild breathlessness. The patient was febrile and mildly tachycardic with crepitations in the right lung base. Blood tests revealed raised inflammatory markers and chest X-ray showed no acute pathology. Following admission, CT pulmonary angiogram showed a large left-sided mediastinal mass (approximately 110 x 70 x 85 mm) centered on the pericardium. Further post venous phase CT imaging identified possible myocardial invasion alongside suspicious liver nodules. Later, outpatient fluorodeoxyglucose (FDG) positron emission tomography (PET) imaging highlighted further FDG avid pleural and liver lesions. CT-guided biopsy of the pericardial lesion was undertaken, with histology and immunohistochemistry indicating epitheliod-type mesothelioma. A significant malignant pericardial effusion was also identified, which ultimately required pericardial window formation. Immunotherapy was commenced utilizing dual nivolumab and ipilimumab, a novel regime for the treatment of mesothelioma. Palliative radiotherapy to the pericardial lesion will also be performed. Here, we demonstrate the diagnostic challenge of this vanishingly rare condition, which is usually diagnosed upon the development of associated complications. Early recognition gives the best chance of improved mortality, however, diagnosis requires a high index of clinical suspicion alongside prompt investigation, primarily involving cross-sectional imaging.
PubMed: 34984126
DOI: 10.7759/cureus.19966 -
Frontiers in Cardiovascular Medicine 2021Primary pericardial mesothelioma is an extremely rare tumor, and early identification and accurate diagnosis may improve its clinical outcome. In this study, we...
Primary pericardial mesothelioma is an extremely rare tumor, and early identification and accurate diagnosis may improve its clinical outcome. In this study, we reported a case of a 70-year-old woman who presented with dyspnea. Conventional transthoracic echocardiography showed massive pericardial effusion. Contrast-enhanced ultrasonography revealed a hyper-enhancing mass in the pericardium. Further imaging methods, including cardiac MRI and positron emission tomography/computed tomography, showed invasion of the pericardial mass into the adjacent tissues and distant metastases. Pathologic examination of a puncture biopsy specimen finally confirmed the diagnosis of PPM. Pericardial masses are difficult to detect when a large amount of pericardial effusion is present and the mass is small. The combination of multiple modalities plays a meaningful role in identifying PPM.
PubMed: 34869673
DOI: 10.3389/fcvm.2021.758988 -
Thoracic Cancer Jan 2022A 70-year-old man diagnosed with right-sided malignant epithelial pleural mesothelioma, underwent pleurectomy/decortication after three courses of neoadjuvant...
A 70-year-old man diagnosed with right-sided malignant epithelial pleural mesothelioma, underwent pleurectomy/decortication after three courses of neoadjuvant chemotherapy. He had a history of mitral valve replacement and maze procedure with median sternotomy, and the procedures resulted in strong adhesion from the apex to the mediastinal side. In particular, the peeling of the area where the tumor invaded the pericardium required the most attention; however, the involved pericardium could be partially resected without damaging the right atrium. Finally, en bloc macroscopic complete resection with the entire pleura was successfully performed without conversion to extrapleural pneumonectomy.
Topics: Aged; Humans; Male; Mesothelioma, Malignant; Neoadjuvant Therapy; Pleural Neoplasms; Sternotomy; Thoracic Surgical Procedures
PubMed: 34799989
DOI: 10.1111/1759-7714.14231 -
Toxicologic Pathology Feb 2022Spontaneous primary pleural mesotheliomas in Fischer 344 (F344) or other rat strains have rarely been reported. The objectives of this retrospective study were to...
Spontaneous primary pleural mesotheliomas in Fischer 344 (F344) or other rat strains have rarely been reported. The objectives of this retrospective study were to develop historical incidence data and better characterize the light-microscopic morphology of these naturally occurring neoplasms in a large cohort of rats of several strains. A retrospective review was performed of National Toxicology Program (NTP) studies in rats conducted between 1980 and 2019 and comprising a total of 104,029 rats (51,326 males, 52,703 females), predominantly (90%) of the F344 strain. Of the 94,062 F344 rats surveyed, there were 30 cases of primary pleural mesotheliomas (22 males, 8 females). Of the 2998 Wistar Han rats surveyed, primary pleural mesotheliomas were present in 2 male rats. No primary pleural mesotheliomas were noted in male and female rats of other strains (6669 Sprague Dawley; 300 Osborne-Mendel). All primary pleural mesotheliomas in control and treated F344 and Wistar Han rats were considered spontaneous and unrelated to treatment. Based on light-microscopic evaluation of paraffin-embedded hematoxylin and eosin stained sections, only epithelioid and biphasic histologic subtypes were observed: 18 and 12 in F344 rats, respectively, and one each in Wistar Han rats. No sarcomatoid subtype cases were noted in any strain of rat.
Topics: Animals; Female; Humans; Male; Mesothelioma; Rats; Rats, Inbred F344; Rats, Sprague-Dawley; Rats, Wistar; Retrospective Studies
PubMed: 34727809
DOI: 10.1177/01926233211053631 -
Cureus Sep 2021Malignant mesotheliomas (MM), as described are rare tumors that are mostly associated with occupational exposure to asbestos. They most commonly occur in the pleura....
Malignant mesotheliomas (MM), as described are rare tumors that are mostly associated with occupational exposure to asbestos. They most commonly occur in the pleura. Other unfamiliar sites where they can occur are the peritoneum, pericardium, and tunica vaginalis. There is no significant correlation between the amount and duration of asbestos exposure to mesothelioma development as reported by various studies over the years. Apart from the environmental exposure, the development of malignant mesothelioma has been linked to a mutation in the BAP1 gene, which can predispose the patient to develop other malignancies associated with BAP1 mutation. We report a case of a 43-year-old man without any significant risk factors, who presented with a complaint of abdominal discomfort and was found to have malignant peritoneal mesothelioma (MPM). With a known familial history of mesothelioma and melanoma, our patient underwent genetic testing which revealed a mutation in BAP1, affirming the strong association with the development of MPM. Young patients who develop malignant mesothelioma without risk factors for MM should have germline testing for BAP1. This case report is unique and highlights a familial variant of mesothelioma, even rare with peritoneal mesothelioma in our patient.
PubMed: 34725624
DOI: 10.7759/cureus.18380