-
Current Opinion in Oncology Jan 2021Malignant pleural mesothelioma (MPM) is a rare, but aggressive tumor with still poor prognosis. In this article, we focus on recent developments in the management of MPM... (Review)
Review
PURPOSE OF REVIEW
Malignant pleural mesothelioma (MPM) is a rare, but aggressive tumor with still poor prognosis. In this article, we focus on recent developments in the management of MPM including diagnosis, staging, biomarkers, and treatment strategies.
RECENT FINDINGS
Molecular markers such as programmed death-ligand 1 (PDL-1), Breast Cancer gene 1-associated protein gene, and cyclin-dependent kinase inhibitor 2A (CDKN2A) have prognostic impact and should be considered for assessment in patient samples. In addition to histological subtype and tumor pattern, tumor volumetry plays an increasing important role in staging, assessment of treatment response, and prediction of survival. Several new blood-based biomarkers have been recently reported including peripheral blood DNA methylation, microRNAs, fibulin, and high-mobility group box 1, but have not been established in clinical routine use yet. Regarding treatment, targeted therapies, immunotherapy, and vaccination are considered as new promising strategies. Moreover, extended pleurectomy/decortication is favored over extrapleural pneumonectomy (EPP) and intensity-modulated radiotherapy represents a possible approach in combination with EPP and pleurectomy/decortication. Intracavitary treatment options are promising and deserve further investigations.
SUMMARY
Overall, there has not been a real breakthrough in the treatment of MPM. Further research and clinical trials are needed to evaluate outcome and to identify new potential treatment candidates.
Topics: Clinical Trials, Phase II as Topic; Clinical Trials, Phase III as Topic; Combined Modality Therapy; Humans; Mesothelioma, Malignant; Neoplasm Staging; Pleural Neoplasms; Randomized Controlled Trials as Topic
PubMed: 33186182
DOI: 10.1097/CCO.0000000000000697 -
DICER1 tumor predisposition syndrome: an evolving story initiated with the pleuropulmonary blastoma.Modern Pathology : An Official Journal... Jan 2022DICER1 syndrome (OMIM 606241, 601200) is a rare autosomal dominant familial tumor predisposition disorder with a heterozygous DICER1 germline mutation. The most common... (Review)
Review
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.
Topics: Causality; Germ-Line Mutation; Humans; Lung Neoplasms; Pleural Neoplasms; Pulmonary Blastoma; Ribonuclease III; Syndrome
PubMed: 34599283
DOI: 10.1038/s41379-021-00905-8 -
The New England Journal of Medicine Sep 2021
Review
Topics: Antineoplastic Agents; Combined Modality Therapy; Epigenetic Repression; Humans; Immunotherapy; Mesothelioma, Malignant; Pleura; Pleural Neoplasms; Radiotherapy
PubMed: 34551230
DOI: 10.1056/NEJMra1912719 -
Advances in Anatomic Pathology Jul 2023Primary pericardial mesothelioma (PM) is a rare tumor arising from the mesothelial cells of the pericardium. It has an incidence of <0.05% and comprises <2% of all...
Primary pericardial mesothelioma (PM) is a rare tumor arising from the mesothelial cells of the pericardium. It has an incidence of <0.05% and comprises <2% of all mesotheliomas; however, it is the most common primary malignancy of the pericardium. PM should be distinguished from secondary involvement by the spread of pleural mesothelioma or metastases, which are more common. Although data are controversial, the association between asbestos exposure and PM is less documented than that with other mesotheliomas. Late clinical presentation is common. Symptoms may be nonspecific but are usually related to pericardial constriction or cardiac tamponade, and diagnosis can be challenging usually requiring multiple imaging modalities. Echocardiography, computed tomography, and cardiac magnetic resonance demonstrate heterogeneously enhancing thickened pericardium, usually encasing the heart, with findings of constrictive physiology. Tissue sampling is essential for diagnosis. Histologically, similar to mesotheliomas elsewhere in the body, PM is classified as epithelioid, sarcomatoid, or biphasic, with the biphasic type being the most common. Combined with morphologic assessment, the use of immunohistochemistry and other ancillary studies is helpful for distinguishing mesotheliomas from benign proliferative processes and other neoplastic processes. The prognosis of PM is poor with about 22% 1-year survival. Unfortunately, the rarity of PM poses limitations for comprehensive and prospective studies to gain further insight into the pathobiology, diagnosis, and treatment of PM.
Topics: Humans; Prospective Studies; Mesothelioma, Malignant; Mesothelioma; Pleural Neoplasms; Sarcoma; Heart Neoplasms
PubMed: 37104724
DOI: 10.1097/PAP.0000000000000399 -
Topics in Magnetic Resonance Imaging :... Apr 2018The pleura may be affected by primary tumors or metastatic spread of intrathoracic or extrathoracic neoplasms. Primary pleural neoplasms represent ∼10% of all pleural... (Review)
Review
The pleura may be affected by primary tumors or metastatic spread of intrathoracic or extrathoracic neoplasms. Primary pleural neoplasms represent ∼10% of all pleural tumors, and malignant lesions are more common than benign lesions. The most common primary tumors include malignant pleural mesothelioma and solitary fibrous tumor. Although pleural neoplasms may initially be evaluated with computed tomography (CT) and/or fluorodeoxyglucose positron emission tomography (PET)/CT, magnetic resonance (MR) imaging is complementary to these other imaging modalities for disease staging and evaluation of patients. In this article, we discuss the etiology, clinical presentation, and imaging of pleural neoplasms, with specific attention given to the role of MR imaging.
Topics: Fluorodeoxyglucose F18; Humans; Lung Neoplasms; Magnetic Resonance Imaging; Mesothelioma; Mesothelioma, Malignant; Neoplasm Staging; Pleural Neoplasms; Positron-Emission Tomography; Tomography, X-Ray Computed
PubMed: 29613962
DOI: 10.1097/RMR.0000000000000162 -
Clinical Radiology Dec 2018There are various neoplasms and tumour-like conditions of the pleura. Mesothelioma is perhaps the most widely recognised; however, there are many others that are more... (Review)
Review
There are various neoplasms and tumour-like conditions of the pleura. Mesothelioma is perhaps the most widely recognised; however, there are many others that are more common and should be considered. Understanding the similarities and differences can be helpful in managing the patient with a newly found pleural lesion. We will discuss clinical symptoms at presentation and describe the imaging findings associated with these tumours, starting with conventional radiology, and correlating with computed tomography and combined positron-emission tomography (PET)/computed tomography (CT). Finally, imaging characteristics that help differentiation between the benign and malignant varieties will be reviewed.
Topics: Diagnosis, Differential; Fluorodeoxyglucose F18; Humans; Lung Neoplasms; Mesothelioma; Mesothelioma, Malignant; Neoplasm Staging; Pleura; Pleural Neoplasms; Positron-Emission Tomography; Radiopharmaceuticals; Tomography, X-Ray Computed
PubMed: 30064697
DOI: 10.1016/j.crad.2018.07.093 -
Seminars in Respiratory and Critical... Jun 2019Malignant pleural mesothelioma is a rare cancer associated with asbestos exposure and portends a dismal prognosis. Its worldwide incidence has been increasing, and... (Review)
Review
Malignant pleural mesothelioma is a rare cancer associated with asbestos exposure and portends a dismal prognosis. Its worldwide incidence has been increasing, and treatment options are currently suboptimal and noncurative. However, since the turn of the century, several encouraging steps have been made toward improving outcomes for mesothelioma patients. An increased understanding of disease pathophysiology has led to more accurate diagnosis and staging, and the establishment of the standard of care first-line pemetrexed/platin doublet chemotherapy regimen in 2003 initially revolutionized treatment. While significant debate remains regarding the preferred approach to surgical and radiation therapy in the context of multimodal therapy, recent breakthroughs in immunotherapy offer hope for another paradigm shift in the near future. This review will summarize the current clinical approach to diagnosis, staging, and treatment of malignant pleural mesothelioma.
Topics: Antineoplastic Agents, Immunological; Antineoplastic Combined Chemotherapy Protocols; Asbestos; Biomarkers, Tumor; Biopsy; CTLA-4 Antigen; Catheters, Indwelling; Combined Modality Therapy; Humans; Immunohistochemistry; Lung Neoplasms; Mesothelioma; Mesothelioma, Malignant; Neoplasm Staging; Pleural Neoplasms; Pneumonectomy; Prognosis; Programmed Cell Death 1 Receptor; Radiotherapy, Adjuvant; Thoracoscopy
PubMed: 31525810
DOI: 10.1055/s-0039-1693406 -
European Respiratory Review : An... Mar 2021Pleural infection and malignancy are among the most common causes of pleural disease and form the mainstay of pleural practice. There has been significant research and...
Pleural infection and malignancy are among the most common causes of pleural disease and form the mainstay of pleural practice. There has been significant research and increase in scientific understanding in these areas in the past decade. With regard to pleural infection, the rising incidence remains worrying. An increased awareness allowing earlier diagnosis, earlier escalation of therapy and the use of validated risk stratification measures may improve outcomes. In pleural malignancy, research has enabled clinicians to streamline patient pathways with focus on reducing time to diagnosis, definitive management of malignant pleural effusion and achieving these with the minimum number of pleural interventions. Trials comparing treatment modalities of malignant pleural effusion continue to highlight the importance of patient choice in clinical decision-making. This article aims to summarise some of the most recent literature informing current practice in these two areas.
Topics: Humans; Pleural Diseases; Pleural Effusion, Malignant; Pleural Neoplasms; Thoracic Surgical Procedures
PubMed: 33650525
DOI: 10.1183/16000617.0002-2020 -
European Respiratory Review : An... Mar 2021Malignant pleural mesothelioma is an aggressive, incurable cancer that is usually caused by asbestos exposure several decades before symptoms arise. Despite widespread... (Review)
Review
Malignant pleural mesothelioma is an aggressive, incurable cancer that is usually caused by asbestos exposure several decades before symptoms arise. Despite widespread prohibition of asbestos production and supply, its incidence continues to increase. It is heterogeneous in its presentation and behaviour, and diagnosis can be notoriously difficult. Identification of actionable gene mutations has proven challenging and current treatment options are largely ineffective, with a median survival of 10-12 months.However, the past few years have witnessed major advances in our understanding of the biology and pathogenesis of mesothelioma. This has also revealed the limitations of existing diagnostic algorithms and identified new treatment targets.Recent clinical trials have re-examined the role of surgery, provided new options for the management of associated pleural effusions and heralded the addition of targeted therapies. The increasing complexity of mesothelioma management, along with a desperate need for further research, means that a multidisciplinary team framework is essential for the delivery of contemporary mesothelioma care.This review provides a synthesised overview of the current state of knowledge and an update on the latest research in the field.
Topics: Asbestos; Biology; Humans; Mesothelioma; Mesothelioma, Malignant; Pleural Neoplasms
PubMed: 33472960
DOI: 10.1183/16000617.0226-2020 -
Histopathology Jan 2024Mesothelioma is a rare disease with an historically poor prognosis. Over the past decade, a grading system has been developed that is a powerful prognostic tool in... (Review)
Review
Mesothelioma is a rare disease with an historically poor prognosis. Over the past decade, a grading system has been developed that is a powerful prognostic tool in epithelioid mesothelioma. Grading of epithelioid mesothelioma is now required or strongly recommended by expert consensus, the College of American Pathologists, the World Health Organization, and the International Mesothelioma Interest Group. The original nuclear grading system for epithelioid mesothelioma, developed in the United States, split epithelioid mesotheliomas into three prognostic groups with marked differences in survival. Now, this three-tiered nuclear grading system has been combined with the presence or absence of necrosis to form the currently recommended two-tiered grading system of low- and high-grade epithelioid mesothelioma. This review will focus on the development of this grading system in mesothelioma, the grading system's shortcomings, and the application of the grading system to cytology specimens and other extra-pleural sites. Lastly, this review will briefly discuss alternative grading systems and future considerations.
Topics: Humans; Pleural Neoplasms; Lung Neoplasms; Neoplasm Grading; Mesothelioma, Malignant; Mesothelioma; Prognosis; Biomarkers, Tumor
PubMed: 37872123
DOI: 10.1111/his.15065