Thymomas and Thymic Carcinomas: A Review on Pathology, Presentation, Staging, Treatment, and Novel Systemic Therapies

Abstract

Although thymomas and thymic carcinomas only represent 0.2–1.5% of all malignancies, they are the most common tumour found in the anterior mediastinum. Recently, the World Health Organization (WHO) classification of thymic epithelial tumours was revised and a new tumour, node, and metastasis (TNM) staging system is currently being developed. Nearly a third of patients with thymoma present with paraneoplastic syndromes, most commonly myasthenia gravis. Thymic carcinomas are rarely associated with paraneoplastic syndromes, with patients often presenting with local symptoms. Recommendations for the management of these tumours are primarily based on small prospective studies, meta-analyses, and expert guidelines. The development of novel therapies to treat thymomas and thymic carcinomas is an area of robust research.