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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 -
Current Opinion in Oncology Mar 2005The present review reports findings in the field of epithelial tumors originating from the thymus from the past year and discusses these findings in the context of the... (Review)
Review
PURPOSE OF REVIEW
The present review reports findings in the field of epithelial tumors originating from the thymus from the past year and discusses these findings in the context of the literature.
RECENT FINDINGS
Epithelial tumors of the thymus are relatively common tumors of the anterior superior mediastinum. Thymomas are usually slowly growing tumors, and their prognosis depends on the macroscopic and microscopic invasion of surrounding tissues. Thymic carcinomas are more aggressive and less common tumors than thymomas and have been increasing in frequency in recent years. Surgery is the mainstay treatment of thymic malignancies, and complete resection represents the best prognostic factor in this disease. Postoperative radiotherapy may be indicated in tumors with invasion of surrounding tissues, but it is controversial in early-stage thymomas. Combination chemotherapy has been employed in several small studies and in advanced disease has been demonstrated to produce a 50-80% objective response rate. Neoadjuvant chemotherapy or external beam radiotherapy have been used with success in patients with tumors that are not readily resectable. Novel antiproliferative systemic agents are being investigated, based on a better understanding of the biology of these tumors.
SUMMARY
A better understanding of the clinical behavior of thymomas versus thymic carcinomas and systemic therapies targeted to biologically validated targets in these diseases will help improve efficacy of treatment.
Topics: Combined Modality Therapy; Humans; Myasthenia Gravis; Thymoma; Thymus Neoplasms
PubMed: 15725919
DOI: 10.1097/01.cco.0000152628.43867.8e -
Current Opinion in Oncology Mar 2012Thymomas are the most common tumors of the anterior mediastinum. Although surgery remains the only curative treatment, the use of multimodality therapy for primary... (Review)
Review
PURPOSE OF REVIEW
Thymomas are the most common tumors of the anterior mediastinum. Although surgery remains the only curative treatment, the use of multimodality therapy for primary unresectable thymomas has led to change the clinical management of these tumors.
RECENT FINDINGS
Nowadays Masaoka stage, WHO, and radical surgical resection are considered by many authors as independent prognostic factors for long-term survival. Radiotherapy may be useful as adjuvant therapy in cases of incomplete surgical resection with microscopic or macroscopic residual disease, or for those patients with locally advanced or metastatic unresectable disease. Chemotherapy is considered a valid option in selected patients with residual disease after local treatments or as a neoadjuvant approach to improve resectability in Masaoka stages III or IV-a thymomas. Currently, no standardized regimen for chemotherapy or agreed timing exists.
SUMMARY
So far, multimodality treatment has been related to low morbidity and long survival rate, but there are still many concerns regarding a different regimen of therapy and the correct timing.
Topics: Antineoplastic Combined Chemotherapy Protocols; Combined Modality Therapy; Humans; Neoadjuvant Therapy; Neoplasm Staging; Radiotherapy Dosage; Survival Rate; Thymectomy; Thymoma; Thymus Neoplasms
PubMed: 22143371
DOI: 10.1097/CCO.0b013e32834ea6bb -
Cancer Jul 1992Sixty-one patients underwent operations for malignant thymomas between 1961 and 1989. Twenty-three patients had associated myasthenia gravis (MG), an incidence of 37.7%....
Sixty-one patients underwent operations for malignant thymomas between 1961 and 1989. Twenty-three patients had associated myasthenia gravis (MG), an incidence of 37.7%. Upon being admitted to the hospital, the patients' most common symptoms included chest pain, MG, cough, and dyspnea. Only 7 of 61 (11.5%) patients had no symptom. Tumor staging of 58 patients with invasive thymomas was performed according to Masaoka classification. The patients were classified as follows: Stage II disease, 5; Stage III, 41; Stage IVa, 8; and Stage IVb, 4. In addition, thymic carcinoma was present in three patients. The series had a resection rate of 55.7%. The incidence of operative complications was 16.3%. Only one patient died of myocardial infarction; the incidence of operative mortality was 1.6%. The patients with MG had a higher rate of resection (69.6%) and a higher incidence of complete thymectomy (14 of 23 patients; 60.9%). Mixed lymphoepithelial tumors and epithelial cell predominant tumors were the most frequent histologic patterns (45.9% and 34.4%, respectively). Fifty-two patients had postoperative radiation therapy, and 10 patients had chemotherapy. The overall cumulative survival rates in the series were 59% and 34% at 5 and 10 years, respectively. The results demonstrated that the factors affecting the prognosis may include resectability, postoperative irradiation or chemotherapy, MG, and tumor staging. The influence of histologic variation on survival rates could not be clearly defined in the series. Surgical resection, particularly complete thymectomy, followed by irradiation is the primary option of therapeutic management for malignant thymoma.
Topics: Adolescent; Adult; Aged; Chi-Square Distribution; Child; Child, Preschool; Combined Modality Therapy; Female; Humans; Incidence; Male; Middle Aged; Myasthenia Gravis; Neoplasm Invasiveness; Neoplasm Staging; Prognosis; Survival Rate; Thymectomy; Thymoma; Thymus Neoplasms
PubMed: 1617594
DOI: 10.1002/1097-0142(19920715)70:2<443::aid-cncr2820700212>3.0.co;2-t -
Seminars in Arthritis and Rheumatism Oct 1998To determine whether malignant thymoma is associated with high rates of concomitantly occurring autoimmune diseases. (Review)
Review
OBJECTIVES
To determine whether malignant thymoma is associated with high rates of concomitantly occurring autoimmune diseases.
METHODS
Sheba Medical Center computer records from 1966 to 1995 were reviewed to identify patients with malignant thymoma, either type I (invasive thymoma) or type II (thymic carcinoma). All patients who had malignant thymoma and autoimmune phenomena were analyzed. The diagnosis of thymic neoplasm was confirmed by two independent pathologists. The diagnosis of autoimmune diseases was based on both clinical and serological findings.
RESULTS
Six of 22 (27%) cases of malignant thymoma had an autoimmune disease. Five patients had type I malignant thymoma and either myasthenia gravis (four patients) or Graves' disease (one patient). Only one patient had type II malignant thymoma with Sjögren's syndrome. The diagnosis of autoimmune disease preceded the diagnosis of thymic neoplasm in four cases, and was diagnosed simultaneously in two.
CONCLUSIONS
Malignant thymomas are highly associated with autoimmune diseases, as are benign thymomas. To our knowledge, we report the first documented cases of a patient with thymic carcinoma and Sjögren's syndrome, and another with invasive thymoma and Graves' disease.
Topics: Adult; Aged; Autoimmune Diseases; Female; Humans; Male; Middle Aged; Myasthenia Gravis; Retrospective Studies; Thymoma; Thymus Neoplasms
PubMed: 9806367
DOI: 10.1016/s0049-0172(98)80039-5 -
Cancer Treatment Reviews Apr 2000Malignant thymomas are rare indolent tumours of the anterior superior mediastinum. Despite a benign histologic appearance, some thymomas invade nearby structures or... (Review)
Review
Malignant thymomas are rare indolent tumours of the anterior superior mediastinum. Despite a benign histologic appearance, some thymomas invade nearby structures or metastasize. Patients are commonly asymptomatic, but some may present with unusual paraneoplastic syndromes such as myasthenia gravis, pure red cell aplasia, or hypogammaglobulinemia. Since tumour biopsy may potentially disrupt the thymic capsule, it is often not performed. Patients are therefore diagnosed and staged at the time of definitive surgery. Thymomas can generally be categorized into two stages: non-invasive and invasive. Prognosis closely parallels the disease stage. Surgery is the principal treatment and is curative in early stage disease. Radiation therapy, either alone or in combination with chemotherapy, is an option for both incompletely or completely resected disease. Chemotherapy is offered to patients with locally advanced, recurrent, or metastatic thymoma, with excellent responses and prolonged survival. Multicentre co-operative group clinical trials are required to assess novel thymoma therapies to maximize patient resources in this uncommon tumour.
Topics: Combined Modality Therapy; Forecasting; Humans; Neoplasm Staging; Salvage Therapy; Thymoma; Thymus Neoplasms
PubMed: 10772969
DOI: 10.1053/ctrv.1999.0159 -
Oncology (Williston Park, N.Y.) Jul 1998Thymomas are rare, slow-growing neoplasms that are considered to be malignant because of their potential invasiveness. The most widely used staging system is that of... (Review)
Review
Thymomas are rare, slow-growing neoplasms that are considered to be malignant because of their potential invasiveness. The most widely used staging system is that of Masaoka and colleagues, which takes into account the extent of clinical and histopathologically determined disease involvement. However, recent data suggest that the staging system of the French Study Group on Thymic Tumors (GETT system), which is based on the surgical and pathologic features of the tumor, may be superior to the Masaoka system. Total resection followed by radiation therapy is the treatment of choice for all thymomas, except stage IA tumors, which can be treated with surgery alone. Chemotherapy can improve the outcome of invasive Masaoka stage III and IV thymomas or recurrent thymomas. Only platinum-containing regimens show consistent efficacy.
Topics: Antineoplastic Agents; Antineoplastic Combined Chemotherapy Protocols; Chemotherapy, Adjuvant; Humans; Neoplasm Invasiveness; Neoplasm Recurrence, Local; Neoplasm Staging; Platinum; Radiotherapy, Adjuvant; Thymectomy; Thymoma; Thymus Neoplasms
PubMed: 9684271
DOI: No ID Found -
Cancer Treatment Reviews Aug 2012Thymomas are the most common tumors of the anterior mediastinum. Classification, treatment options and understanding of the pathophysiology of thymoma have changed over... (Review)
Review
Thymomas are the most common tumors of the anterior mediastinum. Classification, treatment options and understanding of the pathophysiology of thymoma have changed over the past years. It is hoped that novel therapeutic strategies will lead to a survival benefit in these patients. It has turned out that patients with thymoma are best treated with multimodality therapy. In this review, a pathologist, an immunologist, a surgeon, a radiotherapist, a pneumologist and oncologists discuss the current status of classification and strategies for the treatment of thymoma patients.
Topics: Chemotherapy, Adjuvant; Combined Modality Therapy; Diagnosis, Differential; Humans; Neoadjuvant Therapy; Thymoma; Thymus Neoplasms
PubMed: 22244677
DOI: 10.1016/j.ctrv.2011.11.010 -
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 -
Current Opinion in Oncology Sep 2000Thymomas are fascinating tumors because of their multifaceted clinical presentation, including an unrivaled frequency of associated paraneoplastic autoimmune diseases... (Review)
Review
Thymomas are fascinating tumors because of their multifaceted clinical presentation, including an unrivaled frequency of associated paraneoplastic autoimmune diseases and an astounding histologic heterogeneity. Thus, thymomas challenge the clinician, pathologist, and immunologist alike with complex diagnostic and therapeutic problems in almost all fields of medicine, radiology, nuclear medicine, surgery, radiooncology, and pathology. This review highlights recent progress in these fields, including the new World Health Organization's classification of thymomas, and reports on the beginning discovery of the genetic basis of thymoma oncogenesis and the cellular and molecular immunopathology of thymoma-associated paraneoplastic autoimmunity.
Topics: Animals; Humans; Thymoma; Thymus Neoplasms
PubMed: 10975549
DOI: 10.1097/00001622-200009000-00007