The Management of Thymoma: A Systematic Review and Practice Guideline

Introduction: Thymoma is a rare turner for which there is little randomized evidence to guide treatment. Because of the lack of high-quality evidence, a formal consensus-based approach was used to develop recommendations on treatment. Methods: A systematic refview of the literature was performed. Recommendations were formed from available evidence and developed through a two-round modified Delphi consensus approach. Results: The treatment recommendations are summarized as follows: Stage I-complete resection of the entire thymus without neoadjuvant or adjuvant therapy. Stage II-complete resection of the entire thymus with consideration of adjuvant radiation for high-risk tumors. Stage IIIA-surgery either initially or after neoadjuvant therapy, or surgery followed by adjuvant therapy. Stage IIIB-treatment may include a combination of chemotherapy, radiation, and/or surgery, or if technically possible, surgery in combination with chemoradiotherapy (concurrent cisplatin based). For bulky tumors, consideration should be given to sequential chemotherapy followed by radiation. Stage IVA-as per stage III, with surgery only if metastases can be resected. Stage IVB-treatrnent on an individual case basis (no generic recommendations). Recurrent disease-consider surgery, radiation, and/or chemoradiation. Chemoradiation should be considered in all medically inoperable and technically inoperable patients. Conclusion: Consensus was achieved on these recommendations, which serve to provide practical guidance to the physician treating this rare disease.