RESUMO
Ranked high in worldwide growing health issues, pleural diseases affect approximately one million people globally per year and are often correlated with a poor prognosis. Among these pleural diseases, malignant pleural mesothelioma (PM), a neoplastic disease mainly due to asbestos exposure, still remains a diagnostic challenge. Timely diagnosis is imperative to define the most suitable therapeutic approach for the patient, but the choice of diagnostic modalities depends on operator experience and local facilities while bearing in mind the yield of each diagnostic procedure. Since the analysis of pleural fluid cytology is not sufficient in differentiating historical features in PM, histopathological and morphological features obtained via tissue biopsies are fundamental. The quality of biopsy samples is crucial and often requires highly qualified expertise. Since adequate tissue biopsy is essential, medical or video-assisted thoracoscopy (MT or VATS) is proposed as the most suitable approach, with the former being a physician-led procedure. Indeed, MT is the diagnostic gold standard for malignant pleural pathologies. Moreover, this medical or surgical approach can allow diagnostic and therapeutic procedures: it provides the possibility of video-assisted biopsies, the drainage of high volumes of pleural fluid and the administration of sterile calibrated talcum powder under visual control in order to achieve pleurodesis, placement of indwelling pleural catheters if required and in a near future potential intrapleural therapy. In this context, dedicated diagnostic pathways remain a crucial need, especially to quickly and properly diagnose PM. Lastly, the interdisciplinary approach and multidisciplinary collaboration should always be implemented in order to direct the patient to the best customised diagnostic and therapeutic pathway. At the present time, the diagnosis of PM remains an unsolved problem despite MDT (multidisciplinary team) meetings, mainly because of the lack of standardised diagnostic work-up. This review aims to provide an overview of diagnostic procedures in order to propose a clear strategy.
Assuntos
Mesotelioma Maligno , Neoplasias Pleurais , Humanos , Neoplasias Pleurais/diagnóstico , Neoplasias Pleurais/terapia , Mesotelioma Maligno/diagnóstico , Mesotelioma Maligno/terapia , Mesotelioma Maligno/patologia , Mesotelioma/diagnóstico , Mesotelioma/terapia , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/terapia , Biópsia/métodos , Cirurgia Torácica Vídeoassistida/métodosRESUMO
Mesotheliomas are neoplasia developed from the mesothelium, a layer covering the viscera (visceral layer) and the cavity where the organs are (parietal layer). The best known, and the most frequently encountered is the pleural mesothelioma. This disease has a close link with exposure to asbestos, a mineral fibre now banned in several countries. However, other exposure factors have also been incriminated, including another one recognised as a certain carcinogenic agent for several years now : erionite. We present the case of a patient with pleural mesothelioma whose exposure to erionite could be demonstrated. The presentation of this clinical case will be complemented by a literature review on this less known and mostly environmental exposure, contrary to asbestos which is mostly professional.
Les mésothéliomes sont des néoplasies se développant à partir du mésothélium, feuillet recouvrant, d'une part, les viscères (feuillet viscéral) et, d'autre part, la cavité où se trouvent les organes (feuillet pariétal). Le plus connu, et le plus fréquemment rencontré, est le mésothéliome pleural. Cette maladie a un lien étroit avec l'exposition à l'amiante, fibre minérale maintenant interdite dans plusieurs pays. Cependant, d'autres facteurs expositionnels ont également été incriminés, dont un autre reconnu comme cancérogène certain depuis plusieurs années : l'érionite. Nous présentons le cas d'un patient atteint d'un mésothéliome pleural pour lequel une exposition à l'érionite a pu être étayée. La présentation du cas clinique sera complétée d'une revue de la littérature sur cette exposition particulière moins connue et majoritairement environnementale, contrairement à l'amiante dont l'exposition est majoritairement professionnelle.
Assuntos
Mesotelioma , Neoplasias Pleurais , Humanos , Neoplasias Pleurais/etiologia , Neoplasias Pleurais/diagnóstico , Mesotelioma/etiologia , Mesotelioma/induzido quimicamente , Masculino , Zeolitas/efeitos adversos , Exposição Ambiental/efeitos adversos , Mesotelioma Maligno/patologiaAssuntos
Alveolite Alérgica Extrínseca , Lipoma , Neoplasias Pleurais , Humanos , Lipoma/complicações , Lipoma/patologia , Neoplasias Pleurais/complicações , Neoplasias Pleurais/diagnóstico , Neoplasias Pleurais/patologia , Alveolite Alérgica Extrínseca/diagnóstico , Doença Crônica , Masculino , Feminino , Pessoa de Meia-IdadeRESUMO
INTRODUCTION: Histological confirmation of a lung tumor is the prerequisite for treatment planning. It has been suspected that CT-guided needle biopsy (CTGNB) exposes the patient to a higher risk of pleural recurrence. However, the distance between tumor and pleura has largely been neglected as a possible confounder when comparing CTGNB to bronchoscopy. METHODS: All patients with lung cancer histologically confirmed by bronchoscopy or CTGNB between 2010 and 2020 were enrolled and studied. Patients' medical histories, radiologic and pathologic findings and surgical records were reviewed. Pleural recurrence was diagnosed by pleural biopsy, fluid cytology, or by CT chest imaging showing progressive pleural nodules. RESULTS: In this retrospective unicenter analysis, 844 patients underwent curative resection for early-stage lung cancer between 2010 and 2020. Median follow-up was 47.5 months (3-137). 27 patients (3.2 %) with ipsilateral pleural recurrence (IPR) were identified. The distance of the tumor to the pleura was significantly smaller in patients who underwent CTGNB. A tendency of increased risk of IPR was observed in tumors located in the lower lobe (HR: 2.18 [±0.43], p = 0.068), but only microscopic pleural invasion was a significant independent predictive factor for increased risk of IPR (HR: 5.33 [± 0.51], p = 0.001) by multivariate cox analysis. Biopsy by CTGNB did not affect IPR (HR: 1.298 [± 0.39], p = 0.504). CONCLUSION: CTGNB is safe and not associated with an increased incidence of IPR in our cohort of patients. This observation remains to be validated in a larger multicenter patient cohort.
Assuntos
Biópsia Guiada por Imagem , Neoplasias Pulmonares , Neoplasias Pleurais , Tomografia Computadorizada por Raios X , Humanos , Masculino , Feminino , Neoplasias Pleurais/secundário , Neoplasias Pleurais/patologia , Neoplasias Pleurais/diagnóstico por imagem , Neoplasias Pleurais/diagnóstico , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/diagnóstico , Estudos Retrospectivos , Idoso , Tomografia Computadorizada por Raios X/métodos , Biópsia Guiada por Imagem/métodos , Pessoa de Meia-Idade , Pleura/patologia , Pleura/diagnóstico por imagem , Recidiva Local de Neoplasia/patologia , Seguimentos , Idoso de 80 Anos ou mais , Biópsia por Agulha/métodos , AdultoRESUMO
BACKGROUND: The diagnostic complexities that arise in radiographic distinction between ectopic pleural thymoma and other thoracic neoplasms are substantial, with instances of co-occurring T-cell lymphocytosis and osseous metastasis being exceedingly rare. CASE PRESENTATION: A 51-year-old woman was admitted to our hospital with dyspnea and chest pain. Upon imaging examination, she was found to have diffuse and nodular pleural thickening on the left side, collapse of the left lung and a compression in the second thoracic vertebrae. All lesions showed significant 18F-FDG uptake on 18F-FDG PET/CT examination. Furthermore, she exhibited T-cell lymphocytosis in her peripheral blood, lymph nodes, and bone marrow. After ruling out malignant pleural mesothelioma (MPM), lung cancer with pleural metastasis, and T-cell lymphoma, the definitive diagnosis asserted was ectopic pleural thymoma with T-cell lymphocytosis and bone metastasis. CONCLUSION: Physicians need to expand their knowledge of the imaging features of ectopic pleural thymoma. Cases with T-cell lymphocytosis may exhibit increased aggressiveness and prone to bone metastasis.
Assuntos
Neoplasias Ósseas , Linfocitose , Neoplasias Pleurais , Timoma , Humanos , Feminino , Pessoa de Meia-Idade , Timoma/patologia , Timoma/diagnóstico por imagem , Timoma/complicações , Timoma/diagnóstico , Linfocitose/patologia , Linfocitose/diagnóstico , Neoplasias Pleurais/secundário , Neoplasias Pleurais/patologia , Neoplasias Pleurais/complicações , Neoplasias Pleurais/diagnóstico , Neoplasias Ósseas/secundário , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Neoplasias do Timo/patologia , Neoplasias do Timo/complicações , Neoplasias do Timo/diagnóstico , Linfócitos T/patologia , Fluordesoxiglucose F18 , Diagnóstico Diferencial , Pleura/patologia , Pleura/diagnóstico por imagemRESUMO
Pleural mesothelioma (PM) is a highly aggressive tumor that is caused by asbestos exposure and lacks effective therapeutic regimens. Current procedures for PM diagnosis are invasive and can take a long time to reach a definitive result. Small extracellular vesicles (sEVs) have been identified as important communicators between tumor cells and their microenvironment via their cargo including circular RNAs (circRNAs). CircRNAs are thermodynamically stable, highly conserved, and have been found to be dysregulated in cancer. This study aimed to identify potential biomarkers for PM diagnosis by investigating the expression of specific circRNA gene pattern (hsa_circ_0007386) in cells and sEVs using digital polymerase chain reaction (dPCR). For this reason, 5 PM, 14 non-PM, and one normal mesothelial cell line were cultured. The sEV was isolated from the cells using the gold standard ultracentrifuge method. The RNA was extracted from both cells and sEVs, cDNA was synthesized, and dPCR was run. Results showed that hsa_circ_0007386 was significantly overexpressed in PM cell lines and sEVs compared to non-PM and normal mesothelial cell lines (p < 0.0001). The upregulation of hsa_circ_0007386 in PM highlights its potential as a diagnostic biomarker. This study underscores the importance and potential of circRNAs and sEVs as cancer diagnostic tools.
Assuntos
Biomarcadores Tumorais , Vesículas Extracelulares , Mesotelioma , RNA Circular , Humanos , RNA Circular/genética , RNA Circular/metabolismo , Vesículas Extracelulares/metabolismo , Vesículas Extracelulares/genética , Biomarcadores Tumorais/genética , Biomarcadores Tumorais/metabolismo , Mesotelioma/genética , Mesotelioma/diagnóstico , Linhagem Celular Tumoral , Neoplasias Pleurais/genética , Neoplasias Pleurais/diagnóstico , Regulação Neoplásica da Expressão Gênica , Mesotelioma Maligno/genética , Mesotelioma Maligno/diagnósticoRESUMO
We developed a composite symptom score (CSS) representing disease-related symptom burden over time in patients with malignant pleural mesothelioma (MPM). Longitudinal data were collected from an open-label Phase IIB study in which 239 patients completed the validated MD Anderson Symptom Inventory for MPM (MDASI-MPM). A blinded, independent review committee of external patient-reported outcomes experts advised on MDASI-MPM symptoms to include in the CSS. Through iterative analyses of potential symptom-item combinations, 5 MPM symptoms (pain, fatigue, shortness of breath, muscle weakness, coughing) were selected. The CSS correlated strongly with the full MDASI-MPM symptom set (0.92-0.94) and the Lung Cancer Symptom Scale-Mesothelioma (0.79-0.87) at each co-administration of the scales. The CSS also had good sensitivity to worsening disease and global quality-of-life ratings. The MDASI-MPM CSS can be used as an outcome in MPM clinical trials, including in responder analyses and at the individual patient level. It is brief enough to administer frequently, including electronically, to better capture symptom trajectories during and after a trial and in clinical practice. As a single score, the CSS addresses multiplicity issues that can arise when several symptoms increase due to worsening disease. Our process can be adapted to produce a CSS for other advanced-cancer trials.
Assuntos
Mesotelioma Maligno , Neoplasias Pleurais , Qualidade de Vida , Humanos , Mesotelioma Maligno/tratamento farmacológico , Mesotelioma Maligno/patologia , Mesotelioma Maligno/diagnóstico , Masculino , Feminino , Neoplasias Pleurais/diagnóstico , Idoso , Pessoa de Meia-Idade , Neoplasias Pulmonares/diagnóstico , Mesotelioma/diagnóstico , Medidas de Resultados Relatados pelo Paciente , Fadiga , Avaliação de Sintomas , Estudos Longitudinais , Índice de Gravidade de Doença , Carga de SintomasRESUMO
Imaging continues to gain a greater role in the assessment and clinical management of patients with mesothelioma. This communication summarizes the oral presentations from the imaging session at the 2023 International Conference of the International Mesothelioma Interest Group (iMig), which was held in Lille, France from June 26 to 28, 2023. Topics at this session included an overview of best practices for clinical imaging of mesothelioma as reported by an iMig consensus panel, emerging imaging techniques for surgical planning, radiologic assessment of malignant pleural effusion, a radiomics-based transfer learning model to predict patient response to treatment, automated assessment of early contrast enhancement, and tumor thickness for response assessment in peritoneal mesothelioma.
Assuntos
Mesotelioma , Neoplasias Pleurais , Humanos , Mesotelioma/diagnóstico , Mesotelioma/diagnóstico por imagem , Mesotelioma/patologia , Neoplasias Pleurais/diagnóstico , Neoplasias Pleurais/diagnóstico por imagem , Neoplasias Pleurais/patologia , Mesotelioma Maligno/patologia , Mesotelioma Maligno/diagnóstico , Mesotelioma Maligno/diagnóstico por imagem , Diagnóstico por Imagem/métodos , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/diagnóstico por imagem , Neoplasias Pulmonares/patologiaRESUMO
BACKGROUND: Thoracoscopy is useful for diagnosing unexplained pleural effusions. A sufficient specimen volume is often difficult to obtain using forceps biopsies (FBs) but can be obtained with pleural cryobiopsies (CBs). This study aimed to assess the utility and safety of CB during thoracoscopy in the Japanese population. METHODS: Patients who underwent thoracoscopic CBs at the Japanese Red Cross Medical Center between January 2017 and August 2023 were included in the study. Data were retrospectively analyzed, including clinical data, thoracoscopic findings, specimen size, diagnostic yield, and complications. The number of collected specimens and the freezing time were left to the discretion of the attending physician. RESULTS: Twenty-six patients underwent thoracoscopic CB. Specimens obtained by CB were larger than those obtained by FB. Primary lung cancer was the most common cause of pleural effusion, followed by malignant pleural mesothelioma. CB contributed to the diagnosis in 24 of 26 cases (92.3%) and FB contributed to the diagnosis in 11 of 18 cases (61.1%). Severe fibrosis could be diagnosed in all 3 cases by CB, but not by FB. The common complications of CB included bleeding at the biopsy site and atelectasis, but no severe complications occurred. CONCLUSIONS: The utility and safety of thoracoscopic CB for diagnosing pleural effusions in Japan were verified. The diagnostic yield, specimen size, and safety profile of CB support the diagnostic utility of this method.
Assuntos
Neoplasias Pulmonares , Derrame Pleural , Toracoscopia , Humanos , Estudos Retrospectivos , Toracoscopia/métodos , Toracoscopia/efeitos adversos , Masculino , Feminino , Idoso , Biópsia/métodos , Biópsia/efeitos adversos , Japão , Pessoa de Meia-Idade , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/patologia , Derrame Pleural/etiologia , Derrame Pleural/patologia , Idoso de 80 Anos ou mais , Neoplasias Pleurais/diagnóstico , Neoplasias Pleurais/patologia , Mesotelioma Maligno/diagnóstico , Mesotelioma Maligno/patologia , AdultoRESUMO
CASE PRESENTATION: An 80-year-old woman presented with complaints of weakness and dizziness. She had a medical history of subacute cerebral ischemia, vertigo, hypertension, and thalassemia minor. The patient was born and raised in Turkey and has lived in Switzerland for 50 years. Her sister died of a mesothelioma caused by asbestos exposure at the age of 60 years but had lived in Turkey until her death. The patient had neither a history of TB nor B symptoms. She has never smoked.
Assuntos
Tomografia Computadorizada por Raios X , Humanos , Feminino , Idoso de 80 Anos ou mais , Derrame Pleural/etiologia , Derrame Pleural/diagnóstico , Diagnóstico Diferencial , Neoplasias Pleurais/complicações , Neoplasias Pleurais/diagnósticoRESUMO
Spindle cell lesions of the pleura and pericardium are rare. Distinction from sarcomatoid mesothelioma, which has a range of morphologic patterns, can be difficult, but accurate diagnosis matters. This article provides practical guidance for the diagnosis of pleural spindle cell neoplasms, focusing on primary lesions.
Assuntos
Pericárdio , Neoplasias Pleurais , Humanos , Pericárdio/patologia , Neoplasias Pleurais/patologia , Neoplasias Pleurais/diagnóstico , Diagnóstico Diferencial , Neoplasias Cardíacas/patologia , Neoplasias Cardíacas/diagnóstico , Mesotelioma/patologia , Mesotelioma/diagnóstico , Sarcoma/patologia , Sarcoma/diagnóstico , Biomarcadores Tumorais/análise , Pleura/patologiaRESUMO
17-ß-estradiol, involved in mesothelioma pathogenesis, and its precursors were explored as potential biomarkers for the early diagnosis of mesothelioma. Using enzyme-linked immunosorbent assay(ELISA) for 17-ß-estradiol and ultra-high performance liquid chromatography/tandem mass spectrometry(UHPLC-MS/MS) for 19 17-ß-estradiol precursors, a comprehensive analysis of 20steroid hormones was conducted in the serum of mesothelioma patients(n=67), asbestos-exposed healthy subjects(n=39), and non-asbestos-exposed healthy subjects(n=35). Bioinformatics analysis explored three potential serum biomarkers: 17-ß-estradiol, DHEA-S, and androstenedione. The results revealed significant differences in 17-ß-estradiol levels between mesothelioma patients and both non-asbestos-exposed and asbestos-exposed healthy subjects. No significant variations in serum 17-ß-estradiol levels were observed among mesothelioma patients at different stages, suggesting its potential as an early diagnostic marker. 17-ß-estradiol levels were similar in mesothelioma patients with environmental and occupational asbestos exposure, while males with occupational asbestos exposure exhibited significantly higher levels of 17-ß-estradiol compared to females. Significant reduction in androstenedione and an increase in DHEA-S were observed in asbestos-exposed individuals compared to non-asbestos-exposed individuals. The analysis of DHEA-S-androstenedione-17-ß-estradiol signature score showed an increase in asbestos-exposed individuals and mesothelioma patients compared to non-asbestos-exposed individuals, and this score effectively distinguished between the groups. The Cancer Genome Atlas data was utilized to analyze the expression of 5-α-reductase1 and hydroxysteroid-17ß-dehydrogenase2 genes. The findings indicated that mesothelioma patients with elevated gene values for 5-α-reductase1 and hydroxysteroid-17ß-dehydrogenase2 have a worse or better prognosis on overall survival, respectively. In conclusion, this study suggests 17-ß-estradiol, DHEA-S, and androstenedione as biomarkers for mesothelioma risk and early diagnosis of mesothelioma in asbestos-exposed individuals, aiding timely intervention and improved care.
Assuntos
Androstenodiona , Amianto , Biomarcadores Tumorais , Estradiol , Neoplasias Pulmonares , Mesotelioma Maligno , Exposição Ocupacional , Humanos , Estradiol/sangue , Masculino , Biomarcadores Tumorais/sangue , Androstenodiona/sangue , Amianto/toxicidade , Amianto/efeitos adversos , Feminino , Pessoa de Meia-Idade , Exposição Ocupacional/efeitos adversos , Idoso , Mesotelioma Maligno/sangue , Mesotelioma Maligno/diagnóstico , Neoplasias Pulmonares/sangue , Neoplasias Pulmonares/diagnóstico , Mesotelioma/sangue , Mesotelioma/diagnóstico , Mesotelioma/induzido quimicamente , Neoplasias Pleurais/sangue , Neoplasias Pleurais/diagnóstico , Neoplasias Pleurais/induzido quimicamente , Desidroepiandrosterona/sangue , Estudos de Casos e Controles , Detecção Precoce de Câncer/métodosRESUMO
OBJECTIVE: To review the pathological diagnosis of possible cases and/or hidden cases of malignant mesothelioma (MM) between 2000 and 2012 using the Hospital-Based Cancer Registry database in the state of São Paulo, Brazil. METHODS: Possible cases were retrieved by assessing the database. Inclusion criteria were being older than 30 years of age and having ICD-O-3 topography and morphology codes related to MM. A board of expert pathologists reviewed the pathology reports and requested paraffin blocks in cases that demanded revision. After staining with calretinin, D2-40, WT-1 (as positive MM markers) and Ber-EP4 and MOC31 (as negative MM markers), cases were divided and studied independently by a pair of pathologists to confirm or discard the diagnosis of MM. RESULTS: Our sample comprised 482 cases from 25 hospitals, and 130 needed further histological revision. We received 73 paraffin blocks with adequate material. After board analysis, there were 9 cases with a definitive diagnosis of MM, improving the diagnostic rate in 12%. Two cases of previously diagnosed MM were discarded by review. CONCLUSIONS: Our results confirm that part of MM underdiagnosis and underreporting in Brazil is due to incomplete or mistaken pathological diagnosis.
Assuntos
Neoplasias Pulmonares , Mesotelioma Maligno , Mesotelioma , Sistema de Registros , Humanos , Brasil/epidemiologia , Mesotelioma/patologia , Mesotelioma/epidemiologia , Mesotelioma/diagnóstico , Mesotelioma Maligno/patologia , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/epidemiologia , Adulto , Idoso de 80 Anos ou mais , Biomarcadores Tumorais/análise , Neoplasias Pleurais/patologia , Neoplasias Pleurais/epidemiologia , Neoplasias Pleurais/diagnósticoRESUMO
The nomenclature and diagnostic criteria of well-differentiated papillary mesothelial tumour (WDPMT) have been changed in the 2021 World Health Organization (WHO) classification of thoracic tumours, and a new entity, mesothelioma in situ (MIS), introduced. Histologically these two entities may be similar. However, MIS is regarded as a precursor to invasive mesothelioma and requires demonstration of loss of BAP1 and/or MTAP/CDKN2A for diagnosis, whereas performance of these ancillary tests is desirable but not essential for a diagnosis of WDPMT, in which the significance of BAP1 and/or MTAP/CDKN2A loss is not well understood or well defined. Against this backdrop, we undertook an investigation of 21 cases of WDPMT, identified from our case files and diagnosed according to 2021 WHO criteria, to explore the relationship between histology and BAP1 and MTAP/CDKN2A expression with clinical features including asbestos exposure, focality of tumours and clinical outcome. There were 18 women and three men, with ages ranging from 23-77 years (median 62 years), in which six had a history of asbestos exposure, two had no exposure, and in 13 exposure history was unavailable. Of 20 peritoneal tumours and one pleural tumour, 13 were detected incidentally at the time of surgery for unrelated conditions and eight peritoneal tumours were multifocal at the time of diagnosis. BAP1 immunohistochemistry (IHC) was performed in all 21 tumours, with nine tumours showing BAP1 expression loss. MTAP/CDKN2A testing was performed in 14 tumours, comprising MTAP IHC in 12 and CDKN2A fluorescence in situ hybridisation (FISH) in two, with three tumours showing MTAP/CDKN2A expression loss. Two tumours with MTAP/CDKN2A loss also showed BAP1 expression loss. Four patients progressed to invasive mesothelioma, including one male with a pleural tumour and asbestos exposure, and three females with multifocal peritoneal tumours, two with asbestos exposure and one without exposure. BAP1 expression loss was seen in all tumours from the four patients who progressed to invasive mesothelioma, whilst two of these tumours showed retained MTAP IHC and two were not tested. There was one patient with a tumour with MTAP loss and retained BAP1 who died from unrelated causes 5 months after diagnosis. Eight patients received WDPMT-specific treatment in addition to the initial excision. Survival for all patients ranged from 4-218 months, with one patient dying of mesothelioma at 49 months. Based on our results in this series of 21 patients with WDPMT diagnosed according to 2021 WHO criteria, we propose that WDPMT with BAP1 expression loss may best be regarded as papillary MIS and that a history of asbestos exposure and the presence of multifocal tumours in patients diagnosed with WDPMT should prompt ancillary testing with BAP1 IHC. Further we propose that BAP1 IHC should be essential in the diagnosis of WDPMT, with the diagnosis restricted to those tumours which show retained BAP1 expression. However more studies in larger cohorts of patients are needed to explore the relationship between BAP1 expression and MTAP loss in WDPMT, which will help to define this entity and separate it more clearly from MIS and invasive mesothelioma.
Assuntos
Biomarcadores Tumorais , Inibidor p16 de Quinase Dependente de Ciclina , Mesotelioma , Proteínas Supressoras de Tumor , Ubiquitina Tiolesterase , Humanos , Ubiquitina Tiolesterase/metabolismo , Proteínas Supressoras de Tumor/metabolismo , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Adulto , Inibidor p16 de Quinase Dependente de Ciclina/metabolismo , Mesotelioma/patologia , Mesotelioma/metabolismo , Mesotelioma/diagnóstico , Biomarcadores Tumorais/metabolismo , Biomarcadores Tumorais/análise , Purina-Núcleosídeo Fosforilase/metabolismo , Adulto Jovem , Mesotelioma Maligno/patologia , Mesotelioma Maligno/diagnóstico , Mesotelioma Maligno/metabolismo , Neoplasias Mesoteliais/patologia , Neoplasias Mesoteliais/metabolismo , Neoplasias Mesoteliais/diagnóstico , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/metabolismo , Neoplasias Pulmonares/diagnóstico , Neoplasias Pleurais/patologia , Neoplasias Pleurais/metabolismo , Neoplasias Pleurais/diagnóstico , Imuno-HistoquímicaAssuntos
Tumor Fibroso Solitário Pleural , Humanos , Neoplasias Pleurais/cirurgia , Neoplasias Pleurais/patologia , Neoplasias Pleurais/diagnóstico por imagem , Neoplasias Pleurais/diagnóstico , Tumor Fibroso Solitário Pleural/cirurgia , Tumor Fibroso Solitário Pleural/patologia , Tumor Fibroso Solitário Pleural/diagnóstico por imagem , Tumor Fibroso Solitário Pleural/diagnóstico , Tomografia Computadorizada por Raios X , Resultado do TratamentoRESUMO
BACKGROUND: Extrapleural pneumonectomy (EPP) is a complex surgical procedure involving en-bloc resection of the parietal and visceral pleura, lung, pericardium, and ipsilateral diaphragm. Small case series of pleural-based sarcoma of predominantly pediatric patients suggest EPP may be a life-prolonging surgical option. We aimed to describe the characteristics and outcomes of adults who underwent EPP at a specialized sarcoma center. METHODS: Clinicopathologic variables, surgical details, and follow-up information were extracted for patients undergoing EPP for pleural-based sarcoma between August 2017 and December 2020. Primary outcomes were event-free survival (EFS) and overall survival (OS) from the date of EPP. Secondary outcomes were disease-free interval (DFI) prior to EPP, and early and late postoperative complications. RESULTS: Eight patients were identified, seven with soft tissue sarcoma and one with bone sarcoma. Patients had either localized disease with a primary thoracic sarcoma, sarcoma recurrent to the thorax, or de novo metastatic disease. All patients underwent resection of their pleural-based sarcoma by an experienced cardiothoracic surgeon, and some patients had pre or postoperative treatment. The perioperative morbidity was comparable with previously published reports of EPP performed in mesothelioma patients. At median follow-up of 22.5 months, median EFS was 6.0 months and OS was 20.7 months. Six patients (75%) had disease recurrence; five (62.5%) died of progressive disease. Two patients (25%) had not recurred: one died of a radiation-related esophageal rupture, and one was alive with no evidence of disease at 37.0 months. Characteristics of those with the longest EFS included low-grade histology and achieving a metabolic response to preoperative chemotherapy. CONCLUSIONS: In adults with pleural-based sarcoma, EPP is rarely curative but appears to be a feasible salvage procedure when performed at specialized centers. Patient selection is critical with strong consideration given to multimodal therapy to optimize patient outcomes. In the absence of a confirmed response to neoadjuvant treatment, long term survival is poor and EPP should not be recommended.