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Critical Reviews in Oncology/hematology Mar 2016Thymomas (Ts) and thymic carcinomas (TCs) are rare tumours of the mediastinum with an incidence rate of 1.7/million per year in Europe. Histological classification is... (Review)
Review
Thymomas (Ts) and thymic carcinomas (TCs) are rare tumours of the mediastinum with an incidence rate of 1.7/million per year in Europe. Histological classification is based on rate of non-malignant-appearing thymic epithelial cells and proportions of lymphocytes (A, AB, B1, B2, B3, and C), while staging system concerns localisation of the involved areas. Surgery is the mainstay of treatment with a 10-year survival of 80%, 78%, 75%, and 42% for stages I, II, III and IV, respectively, with an R0 resection. Radiotherapy has a role in selected cases (stage III patients or R1-2 residual) and platinum-based chemotherapy remains the standard of care for patients with advanced disease. A multimodality approach would be advisable when surgery is not recommended. Since molecular aberrations are poorly understood and few responses are reported, targeted therapies are yet being studied. In this review, we describe key aspects of clinical management for Ts and TCs.
Topics: Humans; Thymoma; Thymus Neoplasms
PubMed: 26818050
DOI: 10.1016/j.critrevonc.2016.01.012 -
Expert Review of Anticancer Therapy Apr 2022Curative-intent surgery is the best therapeutic option for thymic malignancies. However, patients with advanced or recurrent thymic malignancies often require... (Review)
Review
INTRODUCTION
Curative-intent surgery is the best therapeutic option for thymic malignancies. However, patients with advanced or recurrent thymic malignancies often require palliative-intent chemotherapy or radiotherapy. Since thymic malignancies are rare cancers, the efficacy and safety of treatments have been verified based on small Phase 2 trials or retrospective studies.
AREA COVERED
We comprehensively reviewed the treatment strategies for thymic malignancies, including surgery, radiotherapy, and pharmacotherapy, including cytotoxic chemotherapy, molecular-targeted therapy, and immunotherapy. Additionally, we reviewed specific situations, such as pleural dissemination, central nervous system metastasis, and paraneoplastic syndrome.
EXPERT OPINION
Cytotoxic chemotherapy remains the standard option in pharmacotherapy. However, multikinase inhibitors, such as sunitinib and lenvatinib, and immune checkpoint inhibitors including pembrolizumab have been developed to treat thymic carcinomas. Now, a Phase 2 study is evaluating whether lenvatinib plus pembrolizumab benefits patients with type B3 thymoma or thymic carcinoma. Phosphatidylinositol 3-kinase/AKT/mammalian target of rapamycin inhibitors may contribute to disease control and octreotide scan is only applicable to somatostatin analogues. Although the genomic characteristics of thymic malignancies have been analyzed, few actionable mutations have been detected in general. The development of a treatment strategy using combination pharmacotherapy is anticipated.
Topics: Carcinoma; Clinical Trials, Phase II as Topic; Humans; Neoplasm Recurrence, Local; Retrospective Studies; Thymoma; Thymus Neoplasms
PubMed: 35266421
DOI: 10.1080/14737140.2022.2052278 -
International Journal of Biological... 2023As the most common malignancy from mediastinum, the metabolic reprogramming of thymoma is important in its development. Nevertheless, the connection between the...
As the most common malignancy from mediastinum, the metabolic reprogramming of thymoma is important in its development. Nevertheless, the connection between the metabolic map and thymoma development is yet to be discovered. Thymoma was categorized into three subcategories by unsupervised clustering of molecular markers for metabolic pathway presentation in the TCGA dataset. Different genes and functions enriched were demonstrated through the utilization of metabolic Gene Ontology (GO) analysis. To identify the main contributors in the development of thymic malignancy, we utilized Gene Set Enrichment Analysis (GSEA), Gene Set Variation Analysis (GSVA), and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway enrichment analysis. The prognosis of thymoma was evaluated by screening the essential pathways and genes using GSVA scores and machine learning classifiers. Furthermore, we integrated the transcriptomics findings with spectrum metabolomics investigation, detected through LC-MS/MS, in order to establish the essential controller network of metabolic reprogramming during thymoma progression. The thymoma prognosis is related to glycosphingolipid biosynthesis-lacto and neolacto series pathway, of what high B3GNT5 indicate poor survival. The investigation revealed that glycosphingolipid charts have a significant impact on metabolic dysfunction and could potentially serve as crucial targets in the clinical advancement of metabolic therapy.
Topics: Humans; Thymoma; Chromatography, Liquid; Tandem Mass Spectrometry; Thymus Neoplasms; Cluster Analysis
PubMed: 37781041
DOI: 10.7150/ijbs.83468 -
Der Pathologe Sep 2016Thymic carcinomas (TC) are approximately 10 times less prevalent than thymomas but of high clinical relevance because they are more aggressive, less frequently... (Review)
Review
Thymic carcinomas (TC) are approximately 10 times less prevalent than thymomas but of high clinical relevance because they are more aggressive, less frequently resectable than thymomas and usually refractory to classical and targeted long-term treatment approaches. Furthermore, in children and adolescents TC are more frequent than thymomas and particularly in this age group, germ cell tumors need to be a differential diagnostic consideration. In diagnostic terms pathologists face two challenges: a), the distinction between thymic carcinomas and thymomas with a similar appearance and b), the distinction between TC and histologically similar metastases and tumor extensions from other primary tumors. Overcoming these diagnostic challenges is the focus of the new WHO classification of thymic epithelial tumors. The objectives of this review are to highlight novel aspects of the WHO classification of thymic carcinomas and to address therapeutically relevant diagnostic pitfalls.
Topics: Adolescent; Child; Diagnosis, Differential; Humans; Immunohistochemistry; Neoplasms, Germ Cell and Embryonal; Thymoma; Thymus Gland; Thymus Neoplasms; World Health Organization
PubMed: 27538748
DOI: 10.1007/s00292-016-0194-4 -
Zhongguo Fei Ai Za Zhi = Chinese... Mar 2020Thymoma is a potential malignant disease with a recurrence rate of 10% to 30% after complete resection. There is no agreement on the treatment strategy and standard for... (Review)
Review
Thymoma is a potential malignant disease with a recurrence rate of 10% to 30% after complete resection. There is no agreement on the treatment strategy and standard for recurrent thymoma. The treatment methods include reoperation, chemotherapy, radiotherapy, targeted therapy and immunotherapy. In this article, we review the previous literature and summarize the indications, efficacy and prognosis of different treatments for recurrent thymoma, so as to provide some reference for treatment criteria for recurrent thymoma.
Topics: Humans; Prognosis; Thymoma
PubMed: 32102138
DOI: 10.3779/j.issn.1009-3419.2020.03.11 -
APMIS : Acta Pathologica,... Dec 2023Good's syndrome, an infrequent adult-onset immunodeficiency is characterized by the triad of thymoma, hypogammaglobulinemia, and increased susceptibility to recurrent... (Review)
Review
Good's syndrome, an infrequent adult-onset immunodeficiency is characterized by the triad of thymoma, hypogammaglobulinemia, and increased susceptibility to recurrent infections. The clinical presentation is highly variable, with a spectrum ranging from recurrent bacterial and opportunistic infections to concomitant autoimmune diseases and, sometimes malignant pathologies. Due to heterogeneous clinical phenotypes and the lack of adequate diagnostic criteria, its recognition is often challenging, even delaying it by years. It is one of the most unusual, less studied form of the immune deficiency syndromes with a still unknown pathophysiology. It was initially considered a thymoma-associated variant of primary antibody deficiencies with a reduced or absent number of mature B cells, but it later emerged that significant defects of T cell-mediated immune functions are the underlying cause of opportunistic infections. On the basis of current evidence, Good's syndrome is evaluated as a distinct acquired form of combined immunodeficiency states and classified as a phenocopy of primary immunodeficiency diseases. Epigenetic and acquired genetic factors can play an ultimate role in its evolution.
Topics: Adult; Humans; Thymoma; Immunologic Deficiency Syndromes; Thymus Neoplasms; Primary Immunodeficiency Diseases; Opportunistic Infections
PubMed: 37729389
DOI: 10.1111/apm.13351 -
La Revue Du Praticien Apr 2017Thymic tumors. Thymic epithelial tumors are rare malignancies, that may be aggressive and difficult to treat, with variable prognosis. The histopathological...
Thymic tumors. Thymic epithelial tumors are rare malignancies, that may be aggressive and difficult to treat, with variable prognosis. The histopathological classification distinguishes two major tumor types: thymomas and thymic carcinomas. Autoimmune manifestations are observed in nearly one third of patients at diagnosis; myasthenia gravis is the most common, followed by pure red cell aplasia and hypogammaglobulinemia. Assessing the resectability of the tumor represents the first stage of the therapeutic strategy, as complete resection is the most significant prognostic factor on patient survival. For unresectable thymic tumors, treatment is then based on induction chemotherapy followed by surgical resection or radiotherapy. Following a call of the French National Cancer Institute, a network of expert centers for the management of thymic malignancies started in in 2012: RYTHMIC.
Topics: Humans; Myasthenia Gravis; Neoplasm Staging; Neoplasms, Glandular and Epithelial; Prognosis; Thymoma; Thymus Neoplasms
PubMed: 30512890
DOI: No ID Found -
Advances in Anatomic Pathology Sep 2021The interpretation of biopsy specimens in the diagnosis of thymoma is a subject that is generally not addressed in the literature. Even though the diagnosis of thymoma... (Review)
Review
The interpretation of biopsy specimens in the diagnosis of thymoma is a subject that is generally not addressed in the literature. Even though the diagnosis of thymoma may seem to be an easy step in the assessment of these tumors, in reality, it is the biopsy specimen interpretation that will be use to determine course of action in any particular patient. It may determine whether a patient is a surgical candidate or on the contrary whether a patient may be benefited the most by medical therapy. In addition, there may be conditions in which all that is required is surgical resection without any further treatment, and that the evaluation of those conditions does not necessarily required the careful pathologic staging that thymomas need. In addition, it is important to highlight that in small biopsies, there are limitations not only in terms of the cellularity and other features that may not be present in such biopsy but also the limitation in term of immunohistochemical interpretation. Herein we have attempted to highlight numerous tumoral conditions that are frequently encountered in the daily practice of mediastinal pathology, some of them pose significant problems in separating them from thymomas. Needles to say, the entire spectrum of mediastinal pathology that may at any given time mimic thymoma is well beyond the scope of this review. Furthermore, we also herein emphasize the need for proper clinical and radiologic information and correlation in order to lead to a better interpretation of the biopsy specimen. The emphasis in this review is on thymoma and their possible pitfall and shortcomings while evaluating small biopsy specimens.
Topics: Biopsy; Humans; Thymoma; Thymus Neoplasms
PubMed: 34139743
DOI: 10.1097/PAP.0000000000000310 -
Critical Reviews in Immunology 2016Acquired T-cell immunodeficiency can occur in thymoma patients with or without hypogammaglobulinemia (Good's syndrome), but it has received little attention to date. It... (Review)
Review
Acquired T-cell immunodeficiency can occur in thymoma patients with or without hypogammaglobulinemia (Good's syndrome), but it has received little attention to date. It appears predominantly associated with lymphocyte-rich (i.e., cortical or mixed) thymomas and frequently coexists with autoimmune manifestations. The main abnormalities are an increase in circulating naive T cells, cutaneous T-cell anergy, TCR hyporesponsiveness in vitro as well as a numerical and functional impairment of regulatory T cells. All of these probably result from an abnormal T-cell maturation in the neoplastic thymic microenvironment. A better understanding of thymoma-related acquired T-cell immunodeficiency will be important for immunotherapy of this orphan disease as well as for the prevention and treatment of opportunistic infections, autoimmune complications and secondary malignancies that contribute to the morbidity and mortality of thymoma patients.
Topics: Autoimmunity; Humans; Immunologic Deficiency Syndromes; T-Lymphocytes; Thymoma
PubMed: 28322136
DOI: 10.1615/CritRevImmunol.2017018916 -
European Respiratory Review : An... Dec 2021Thymic tumours are rare thoracic malignancies, that may be aggressive and difficult to treat. The pillars of the management include pathological review, consideration of... (Review)
Review
Thymic tumours are rare thoracic malignancies, that may be aggressive and difficult to treat. The pillars of the management include pathological review, consideration of differential diagnoses, staging and multidisciplinary discussion. Assessment of resectability is key to drive the treatment sequencing. Association with autoimmune diseases, especially myasthenia gravis, is observed, which impacts the oncological management. Networks are being built at the national and international levels. This article provides an overview of the most recent findings in the diagnosis, staging, histology, and management strategies of thymic tumours.
Topics: Diagnosis, Differential; Humans; Myasthenia Gravis; Retrospective Studies; Thymoma; Thymus Neoplasms
PubMed: 34670805
DOI: 10.1183/16000617.0394-2020