Pictorial Review of Pleural Disease: Multimodality Imaging and Differential Diagnosis.

The pleura is a thin, smooth, soft-tissue structure that lines the pleural cavity and separates the lungs from the chest wall, consisting of the visceral and parietal pleurae and physiologic pleural fluid. There is a broad spectrum of normal variations and abnormalities in the pleura, including pneumothorax, pleural effusion, and pleural thickening. Pneumothorax is associated with pulmonary diseases and is caused by iatrogenic or traumatic factors. Chest radiography and US help detect pneumothorax with various signs, and CT can also help assess the causes. Pleural effusion occurs in a wide spectrum of diseases, such as heart failure, cirrhosis, asbestos-related diseases, infections, chylothorax, and malignancies. Chest US allows detection of a small pleural effusion and evaluation of echogenicity or septa in pleural effusion. Pleural thickening may manifest as unilateral or bilateral and as focal, multifocal, or diffuse. Various diseases can demonstrate pleural thickening, such as asbestos-related diseases, neoplasms, and systemic diseases. CT, MRI, and fluorodeoxyglucose (FDG) PET/CT can help differentiate between benign and malignant lesions. Knowledge of these features can aid radiologists in suggesting diagnoses and recommending further examinations with other imaging modalities. The authors provide a comprehensive review of the clinical and multimodality imaging findings of pleural diseases and their differential diagnoses. ©RSNA, 2024 Test Your Knowledge questions for this article are available in the supplemental material.

A Rare Presentation of Minimally Invasive Mesothelioma as a Large Tension Pneumothorax.

Development of mesothelioma is associated with asbestos exposure. Common presentations are with pleural-based plaques invading the chest wall and/or pleural effusion on chest imaging. The intent of this case report is to describe a rare presentation of mesothelioma, which presented atypically as a large tension pneumothorax. A 93-year-old male presented with a history of dyspnea that started after a coughing episode. On physical examination he was hemodynamically stable, but was hypoxic requiring 2L of supplemental oxygen. Computed tomography of the chest revealed a large right tension pneumothorax. A chest tube was placed and connected to suction (-20cmH20), but he continued to have an unresolving air leak over the following 2-week period. Upon video-assisted thoracotomy there were no blebs or adhesions seen. Right apical wedge resection and talc pleurodesis were performed. Pathologic examination revealed an atypical mesothelial cell proliferation with minimal, focal invasion into the pulmonary parenchyma. Tumor spread along the visceral pleura was thought to be the underlying cause of the pneumothorax. The surgical margins were uninvolved by the tumor, and the patient was later discharged home in stable condition. This was a rare presentation of what could best be described as minimally invasive mesothelioma arising in a background of probable mesothelioma in situ, which presented atypically as a large tension pneumothorax. This case highlighted the importance of establishing a pathologic diagnosis from pleural effusion cytology and/or pleural biopsy in persons presenting with spontaneous pneumothorax, and the difficulty in confirming a pathologic diagnosis of early mesothelial neoplasia.

Psychosocial well-being among patients with malignant pleural mesothelioma.

OBJECTIVES: The investigators conducted a psychosocial needs assessment of mesothelioma patients through self-report measures of quality of life (QOL), coping, depression, and social support. METHODS: Patients with malignant pleural mesothelioma (MPM) (N = 67) completed a battery of assessments at a single timepoint after being approached during routine medical oncology clinic appointments or by letter. RESULTS: Participants were predominately male (70.0%; n = 47) and ranged in age from 35 to 83 years old (M = 65.61, SD = 9.71). Most participants were white (88.0%; n = 59), and 10.0% (n = 7) were identified as Hispanic. The majority were married or living with a partner (93.0%; n = 62) and had some college or more education (64.0%; n = 43). Fourteen percent of participants (n = 11) endorsed significantly elevated depression symptoms. No significant demographic or clinical differences in depressed compared to nondepressed participants were observed, with a trend toward those identifying as Hispanic and those who were divorced as being more likely to be depressed. For the total sample, the most frequently endorsed coping strategies were active coping, emotional support, and acceptance. SIGNIFICANCE OF RESULTS: The present study did not identify any clear correlates of depression or QOL among patients with MPM. This research contributes to the small literature on psychosocial functioning in patients with MPM and provides putative directions for future larger studies and the development of interventions to provide appropriate support to diverse patients with MPM.

The Western Australian Mesothelioma Registry: Analysis of 60 years of cases.

BACKGROUND AND OBJECTIVE: Australia introduced a partial ban on asbestos consumption in 1984. There is continuing concern about exposure to asbestos in the built environment and non-occupational exposures. The aim of this study was to describe epidemiological trends of mesothelioma in Western Australia (WA) over the 60 years since the first case was recorded. METHODS: Every case of mesothelioma notified to the WA Cancer Registry is reviewed by an expert panel. Data include demographic and clinical variables including principal mode of asbestos exposure and age at first exposure. Trends over time for survival, latency and pathological subtype of mesothelioma where analysed. Incidence rates for cases exposed during home renovation where calculated. RESULTS: Two thousand seven hundred ninety-six cases of mesothelioma were identified with males comprising the majority (n = 2368, 84.7%). The median (IQR) age at diagnosis was 70 (62-78) years, and median latency of 47 (38-55) years. Pleural mesothelioma was recorded in 2620 (93.7%) cases with the epithelioid subtype most prevalent (n = 1730, 61.9%). Overall, median survival was 298 (128-585) days and latency 46 (37-54) years, both effectively doubling over the study period. Non-occupational exposures were proportionally higher in females (52.6%), compared with males (9.5%). Home renovation was the primary exposure in 227 (8.1%) cases, with number of cases and incidence rate ratio peaking in 2005/09 but subsequently decreasing. CONCLUSION: The annual number of cases of mesothelioma in WA may have hit a plateau. The majority of females have non-occupational exposures and incidence rates from home renovation exposure may have peaked, suggesting the ban on asbestos has been effective.

[Reconocimiento del mesotelioma de pleura como enfermedad profesional en la Comunidad Valenciana de 2012 a 2018.] / Recognition of pleural mesothelioma as an occupational disease in the Valencian Community from 2012 to 2018.

OBJECTIVE: Pleural mesothelioma is a neoplasm almost exclusively attributed to occupational exposure to asbestos and is legally considered an occupational disease. Nevertheless, only a few cases achieve that official recognition. The objective of this work was to describe and analyse this issue, and to identify the major obstacles to its recognition. METHODS: A descriptive and retrospective epidemiological study of data was carried out, including figures and some characteristics, of all patients with pleural mesothelioma registered in the official health and labor registries of the Valencian Community from 2012 to 2018, using frequencies, proportions, and incidence rates. RESULTS: There were large differences between the two sets of data collected in the different registries, especially regarding the number of cases. During the seven years of data examined, 590 pleural mesotheliomas were diagnosed in the Valencian public health system. Of these, the number of cases that were related to occupational exposure was at least 437. Despite the legal duty of doctors to report such cases, only 31 were reported as suspected occupational disease (7.09%), of which only 13 were ultimately officially recognized as such. It was estimated that the annual economic overcost to the public system of unrecognised patients with this occupational disease by was 2,2270,520 euros. CONCLUSIONS: Only a small proportion of occupational mesotheliomas are officially recognized as such. This has important health care and economic repercussions for the individuals involved as well as for the public health system.

OBJETIVO: El mesotelioma de pleura es un cáncer atribuido casi en exclusiva a la exposición laboral al amianto y que tiene la consideración legal de enfermedad profesional, aunque pocos casos consiguen ese reconocimiento oficial. Describir y analizar este problema y los obstáculos para su reconocimiento fue el objetivo de este trabajo. METODOS: Se realizó un estudio epidemiológico descriptivo y retrospectivo de las cifras y algunas características de todos los pacientes de mesotelioma de pleura recogidos en los principales registros oficiales, sanitarios y laborales, de la Comunidad Valenciana, desde 2012 a 2018, utilizando frecuencias, fracciones y tasas de incidencia. RESULTADOS: Hubo grandes diferencias en el número de casos recogidos en los distintos registros. En los siete años estudiados, los mesoteliomas de pleura diagnosticados en el sistema sanitario público valenciano fueron 590. De ellos, aplicando la fracción atribuible al trabajo con amianto, al menos 437 fueron atribuibles al trabajo. Los facultativos comunicaron 31 casos como sospechas de enfermedad profesional, el 7,09% del total, y, finalmente, 13 casos se reconocieron oficialmente como enfermedad profesional. El coste económico estimado de su atención sanitaria para el sistema público valenciano fue de 2.270.520 euros. CONCLUSIONES: Una mínima parte de los mesoteliomas obtienen el reconocimiento de enfermedad profesional. Este hecho conlleva importantes repercusiones asistenciales y económicas para las personas afectadas y para el sistema sanitario público.

Pleural lymphoma combined with pleural sarcoidosis.

A patient with sarcoidosis was found to have a massive left pleural effusion. Her chest computed tomography showed small nodules in the lung parenchyma and swelling of the hilar lymph nodes, with normal visceral and parietal pleura. Thoracoscopy showed white nodules on the visceral pleura and normal parietal pleura, which were resected. Epithelioid granulomas were seen in the visceral pleura and lung parenchyma. Surprisingly, in the parietal pleura, abnormal cells that were positive for the leukocyte common antigen, CD20, and CD79a were found, leading to the diagnosis of malignant B-cell lymphoma.

Clinically Occult Diffuse Pleural Mesothelioma in Patients Presenting With Spontaneous Pneumothorax.

OBJECTIVES: To report histologic features of unsuspected diffuse pleural mesothelioma (DPM) in surgical specimens for pneumothorax and demonstrate how ancillary markers support a diagnosis of malignancy in this context. We explored whether pneumothorax may be a clinical manifestation of mesothelioma in situ (MIS). METHODS: A single-institution database search identified patients who underwent surgical resection for spontaneous pneumothorax (n = 229) and/or were diagnosed with DPM (n = 88) from 2000 to 2020. RESULTS: Spontaneous pneumothorax without clinical, radiologic, or intraoperative suspicion of mesothelioma was the initial presentation in 2 (2.3%) of 88 patients diagnosed with DPM. This represented 0.9% (2/229) of all patients undergoing surgical management of pneumothorax but accounted for a larger proportion of older patients (12.5% older than 70 years). Immunohistochemistry for BAP-1 and/or MTAP confirmed the diagnosis of DPM in 2 cases. Mesothelioma in situ was identified retrospectively by immunohistochemistry in 1 case of spontaneous pneumothorax from a 77-year-old man who developed invasive DPM 25 months later. No additional cases of MIS were identified in 19 surgical lung resections for spontaneous pneumothorax. CONCLUSIONS: Histologic examination of bleb resections with ancillary testing for cases with ambiguous features is essential for detection of early DPM. It is uncertain whether spontaneous pneumothorax may represent a clinical manifestation of MIS.

A Narrative Review-Management of Malignant Pleural Effusion Related to Malignant Pleural Mesothelioma.

Malignant pleural mesothelioma (MPM) is an aggressive, almost universally fatal cancer with limited therapeutic options. Despite efforts, a real breakthrough in treatment and outcomes has been elusive. Pleural effusion with significant breathlessness and pain is the most typical presentation of individuals with MPM. Although thoracentesis provides relief of breathlessness, most such pleural effusions recur rapidly, and a definitive procedure is often required to prevent a recurrence. Unfortunately, the optimal treatment modality for individuals with recurrent MPM-related effusion is unclear, and considerable variation exists in practice. In addition, non-expandable lung is common in pleural effusions due to MPM and makes effective palliation of symptoms more difficult. This review delves into the latest advances in the available management options (both surgical and non-surgical) for dealing with pleural effusion and non-expandable lung related to MPM. We discuss factors that determine the choice of definitive procedures that need to be tailored to the individual patient.

A rare case of primary gastric Burkitt's lymphoma associated with malignant pleural mesothelioma.

BACKGROUND: Primary gastric Burkitt lymphoma (PG BL) and malignant pleural mesothelioma (MPM) are rare and aggressive tumors with poor prognosis. HIV and EBV infection have a link in the aetiology of PG BL, while MPM is usually associated with asbestos exposure. Endoluminal bleeding from massive solid tumor, and dyspnea usually due to pleural effusion, are the typical clinical manifestations respectively of PG BL and MPM. In most patients just palliative treatment is indicated. CASE REPORT: A caucasian elderly male, negative for the proven risk factors, presenting respiratory failure due to massive left pleural effusion with severe mediastinal shift. Contrast enhanced - Computed Tomography (CE-CT) showed a large mass causing circumferential thickening of the gastric fundus, infiltrating the left diaphragmatic dome and the ipsilateral crus. Macroscopically, on endoscopy the gastric fundus appeared completely occupied by an ulcerated large mass protunding in the gastric lumen. Histopathological examination from biopsy specimens taken during esophagogastroduodenoscopy and thoracoscopy allowed to make diagnosis of PG BL and MPM. The patient first underwent a placement of a chest tube drainage for the pleural effusion and then a thoracoscopic talc insufflation (TTI) in the left hemithorax. A surgical treatment of the gastric lesion was planned, due to the rapid growth and the high risk of bleeding. The patient died because of fatal cardiac arrhythmia, before undergoig abdominal surgery. CONCLUSIONS: This report presents an unique case of PG BL associated with MPM and highlights the real challenge for the physicians to identify them in early stage, especially in patients without the proved risk factors. The onset symptoms make it a very singular case, characterized by severe dyspnea up to respiratory failure, due to massive left pleural effusion and contralateral mediastinal fluttering, without an active bleeding from the gastric mass, while CE-CT findings were instead negative for pleural thickening and positive for circumferential thickening of the gastric fundus. KEY WORDS: Burkitt Lymphoma, Case Report, Gastric, Pleural Mesothelioma, Pleural Effusion, Respiratory Failure.

Current causes of mesothelioma: how has the asbestos ban changed the perspective?

The association of mesothelioma, a lethal lung disease, with asbestos has led to an absolute ban on asbestos in at least 55 countries worldwide. The purpose of this paper is to review residual exposure to asbestos as well as other emerging causes of mesothelioma outside asbestos. The review provides detailed description of asbestos minerals, their geographical locations, mesothelioma in these areas, as well as contemporary possible sources of asbestos exposure. Second, we examine other emerging causes of mesothelioma including: ionizing radiation as the second most important risk factor after asbestos, particularly relevant to patients undergoing radiotherapy, third, carbon nanotubes which are under investigation and fourth, Simian virus 40. In the case of asbestos per se, the greatest risk is from occupational exposure during mining and subsequent processing. Of the non-occupational exposures, environmental exposure is most serious, followed by exposure from indoor asbestos minerals and secondary familial exposure. Overall, asbestos is still a major risk factor, but alternative causes should not be neglected, especially in young people, in women and those with a history of radiotherapy or living in high-risk locations.

Pleural mesothelioma metastasis of the scalp.

Mesothelioma is a locally aggressive malignant tumor that arises on the mesothelial surfaces of the pleura, peritoneum and tunica vaginalis. There are three histologic subtypes of mesothelioma: epithelioid, biphasic, and sarcomatoid. Pleural mesothelioma is usually characterized by diffuse pleural thickening. Disease progression is characterized by local invasion of the chest wall and lung. Lymphatic metastasis is rare and hematogenous metastasis is much rarer. The purpose of these case reports is to emphasize that pleural mesothelioma metastases can occur in unexpected places and to contribute to the literature.

Near Missed Case of Occupational Pleural Malignant Mesothelioma, a Case Report and Latest Therapeutic Options.

Asbestos use started to be gradually banned in Europe from 1991 onwards, and there are currently strict occupational exposure limits for asbestos. However, malignant mesothelioma has a long latency time (in some cases up to 50-60 years), so the risks related to asbestos exposure should not be forgotten. Considering the increased risk of lung cancer following the inhalation of asbestos fibers, lifetime health monitoring should be considered in people occupationally exposed to asbestos, with an emphasis on the respiratory system. An assessment of their occupational history should be performed rigorously, especially in the areas with a history of asbestos production/use, as this is a key element for an early diagnosis and appropriate treatment. This case report presents a near-missed case of occupational pleural malignant mesothelioma. The latency time between the first asbestos exposure and the diagnosis of occupational pleural malignant mesothelioma was 49 years. The accurate diagnosis was made two years after the first symptoms appeared.

Spatial Analysis of Shared Risk Factors between Pleural and Ovarian Cancer Mortality in Lombardy (Italy).

BACKGROUND: Asbestos exposure is a recognized risk factor for ovarian cancer and malignant mesothelioma. There are reports in the literature of geographical ecological associations between the occurrence of these two diseases. Our aim was to further explore this association by applying advanced Bayesian techniques to a large population (10 million people). METHODS: We specified a series of Bayesian hierarchical shared models to the bivariate spatial distribution of ovarian and pleural cancer mortality by municipality in the Lombardy Region (Italy) in 2000-2018. RESULTS: Pleural cancer showed a strongly clustered spatial distribution, while ovarian cancer showed a less structured spatial pattern. The most supported Bayesian models by predictive accuracy (widely applicable or Watanabe-Akaike information criterion, WAIC) provided evidence of a shared component between the two diseases. Among five municipalities with significant high standardized mortality ratios of ovarian cancer, three also had high pleural cancer rates. Wide uncertainty was present when addressing the risk of ovarian cancer associated with pleural cancer in areas at low background risk of ovarian cancer. CONCLUSIONS: We found evidence of a shared risk factor between ovarian and pleural cancer at the small geographical level. The impact of the shared risk factor can be relevant and can go unnoticed when the prevalence of other risk factors for ovarian cancer is low. Bayesian modelling provides useful information to tailor epidemiological surveillance.

DOEGE-POTTER SYNDROME BY PLEURAL SOLITARY FIBROUS TUMOR.

INTRODUCTION: Solitary fibrous tumor of the pleura (SFTP) is a rare neoplasm that accounts for less than 5% of all pleural tumors. We present the case of a 73-year-old man with a history of recurrent episodes of severe hypoglycemia secondary to a large malignant SFTP. This paraneoplastic manifestation of SFTP occurs in less than 5% of cases and is referred to as Doege-Potter syndrome. Although rare, this is an important and reversible cause of hypoglycemia, which is resolved by complete surgical resection of the tumor. We describe the pathogenesis, diagnosis, and treatment of Doege-Potter syndrome. Key imaging findings and pathologic correlation are shown.

DICER1 tumor predisposition syndrome: an evolving story initiated with the pleuropulmonary blastoma.

DICER1 syndrome (OMIM 606241, 601200) is a rare autosomal dominant familial tumor predisposition disorder with a heterozygous DICER1 germline mutation. The most common tumor seen clinically is the pleuropulmonary blastoma (PPB), a lung neoplasm of early childhood which is classified on its morphologic features into four types (IR, I, II and III) with tumor progression over time within the first 4-5 years of life from the prognostically favorable cystic type I to the unfavorable solid type III. Following the initial report of PPB, its association with other cystic neoplasms was demonstrated in family studies. The detection of the germline mutation in DICER1 provided the opportunity to identify and continue to recognize a number seemingly unrelated extrapulmonary neoplasms: Sertoli-Leydig cell tumor, gynandroblastoma, embryonal rhabdomyosarcomas of the cervix and other sites, multinodular goiter, differentiated and poorly differentiated thyroid carcinoma, cervical-thyroid teratoma, cystic nephroma-anaplastic sarcoma of kidney, nasal chondromesenchymal hamartoma, intestinal juvenile-like hamartomatous polyp, ciliary body medulloepithelioma, pituitary blastoma, pineoblastoma, primary central nervous system sarcoma, embryonal tumor with multilayered rosettes-like cerebellar tumor, PPB-like peritoneal sarcoma, DICER1-associated presacral malignant teratoid neoplasm and other non-neoplastic associations. Each of these neoplasms is characterized by a second somatic mutation in DICER1. In this review, we have summarized the salient clinicopathologic aspects of these tumors whose histopathologic features have several overlapping morphologic attributes particularly the primitive mesenchyme often with rhabdomyoblastic and chondroid differentiation and an uncommitted spindle cell pattern. Several of these tumors have an initial cystic stage from which there is progression to a high grade, complex patterned neoplasm. These pathologic findings in the appropriate clinical setting should serve to alert the pathologist to the possibility of a DICER1-associated neoplasm and initiate appropriate testing on the neoplasm and to alert the clinician about the concern for a DICER1 mutation.

Risk of asbestosis, mesothelioma, other lung disease or death among motor vehicle mechanics: a 45-year Danish cohort study.

INTRODUCTION: The risk of asbestosis, malignant mesothelioma and lung cancer among motor vehicle mechanics is of concern because of potential exposure to chrysotile asbestos during brake, clutch and gasket repair and maintenance. Asbestos has also been used in insulation and exhaust systems. METHODS: We examined the long-term risk of incident mesothelioma, lung cancer, asbestosis and other lung diseases and mortality due to mesothelioma, lung cancer, asbestosis and other lung diseases in a nationwide cohort of all men registered as motor vehicle mechanics since 1970 in Denmark. This was compared with the corresponding risk in a cohort of male workers matched 10:1 by age and calendar year, with similar socioeconomic status (instrument makers, dairymen, upholsterers, glaziers, butchers, bakers, drivers, farmers and workers in the food industry, trade or public services). RESULTS: Our study included 138 559 motor vehicle mechanics (median age 24 years; median follow-up 20 years (maximum 45 years)) and 1 385 590 comparison workers (median age 25 years; median follow-up 19 years (maximum 45 years)). Compared with other workers, vehicle mechanics had a lower risk of morbidity due to mesothelioma/pleural cancer (n=47 cases) (age-adjusted and calendar-year-adjusted HR=0.74 (95% CI 0.55 to 0.99)), a slightly increased risk of lung cancer (HR=1.09 (95% CI 1.03 to 1.14)), increased risk of asbestosis (HR=1.50 (95% CI 1.10 to 2.03)) and a chronic obstructive pulmonary disease risk close to unity (HR=1.02 (95% CI 0.99 to 1.05)). Corresponding HRs for mortality were 0.86 (95% CI 0.64 to 1.15) for mesothelioma/pleural cancer, 1.06 (95% CI 1.01 to 1.12) for lung cancer, 1.79 (95% CI 1.10 to 2.92) for asbestosis, 1.06 (95% CI 0.86 to 1.30) for other lung diseases caused by external agents and 1.00 (95% CI 0.98 to 1.01) for death due to all causes. CONCLUSIONS: We found that the risk of asbestosis was increased among vehicle mechanics. The risk of malignant mesothelioma/pleural cancers was not increased among vehicle mechanics.

[Giant Solitary Fibrous Tumour of the Pleura Compressing the Left Ventricle:Report of a Case].

A 71-year-old woman with chest pain and exertional dyspnea was referred to our hospital. Computed tomography revealed a huge intrathoracic tumor with left parietal pleural dissemination. Transthoracic echocardiography showed the left ventricular dysfunction due to external compression by the tumor. After excision of the tumor, marked improvement of the left ventricular dysfunction was obtained for eight years. But the patient died due to cardiac invasion of the tumor 9th year after surgery.