Abstract
Thymic epithelial tumours (TET) represent a heterogeneous group of rare malignancies that include thymomas and thymic carcinoma. Treatment of TET is based on the resectability of the tumour. If this is considered achievable upfront, surgical resection is the cornerstone of treatment. Platinum-based chemotherapy is the standard regimen for advanced TET. Due to the rarity of this disease, treatment decisions should be discussed in specific multidisciplinary tumour boards, and there are few prospective clinical studies with new strategies. However, several pathways involved in TET have been explored as potential targets for new therapies in previously treated patients, such as multi-tyrosine kinase inhibitors with antiangiogenic properties and immune checkpoint inhibitors (ICI). One third of patient with thymoma present an autoimmune disorders, increasing the risk of immune-related adverse events and autoimmune flares under ICIs. In these guidelines, we summarize the current evidence for the therapeutic approach in patients with TET and define levels of evidence for these decisions.
Highlights
–Postoperative radiotherapy is recommended in stage III, thymic carcinoma and ≥ R1 resection [IV, B] –Post-operative radiotherapy should start within 3 months of complete resection [V, B] –Definitive radiotherapy is recommended as part of a sequential chemoradiotherapy strategy for patients not suitable for surgery or if complete resection is not feasible [III, A]
It is strongly recommended that the treatment of patients with Thymic epithelial tumours (TET) be discussed in multidisciplinary tumour boards (MTB)
For those patients with induction chemotherapy followed by surgery, postoperative RT should be applied in case of thymic carcinomas (TC), R1 or R2 resection [IV, B] and T with stage III [IV, B] or IIB if B2/B3 subtype [IV, C]
Summary
These guidelines are based on leading studies published in peer review journals. The Infectious Diseases Society of America grading system was used to assign levels of evidence and grades of recommendation [1]. In the European Union, the overall annual incidence of TET is 0.18 per 100.000 (T: 0.14/100.000, and TC: 0.01/100.000) [3]. The overall incidence in Spain remains unknown, but as an example, in Madrid 18 new cases were reported in 2019 [4]. There are no identified risk factors for developing TETs. a higher incidence of TETs has been reported in multiple endocrine neoplasia type 1 [6]. In patients with TET, several studies have documented rates of second malignancies ranging from 8 to 31%, thymomas [7]. The prognosis of TET correlates with the histological subtype, with a 5-year overall survival (OS) of ~ 80% and ~ 45% for T and TC, respectively [8]. Due to the rarity of this disease, there are few prospective clinical studies, and most recommendations stem from retrospective cohort studies or expert opinions. (Table 1)
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
