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Thymoma is unusual but nonetheless the most common tumor of the anterior mediastinum. This tumor is unique in its indolent growth and its frequent association with paraneoplastic syndromes, hematologic syndromes, and autoimmune disorders. By far the most common of these paraneoplastic syndromes is myasthenia gravis, present in 30% to 40% of patients with thymoma.
The diagnosis of thymoma is usually made clinically. Approximately one third of patients with thymoma present without symptoms, typically with a mass detected on chest x-ray obtained for other reasons. Another one third of patients present with vague nonspecific symptoms, such as pressure in the chest or nonspecific pains, or with local symptoms, such as dyspnea, dysphagia, cough, hoarseness, and respiratory infection. Less commonly, patients exhibit superior vena cava syndrome, tracheal stenosis, or bronchial stenosis. The remainder of patients present with signs and symptoms of myasthenia gravis or other paraneoplastic disorders.
Despite contradictory viewpoints regarding histology, pathology, staging, and nonsurgical management, the most important therapeutic modality for thymoma is surgical resection.
Operative mortality of this procedure has been nearly eliminated, and perioperative survival is excellent, with more than 95% of patients discharged from the hospital. Aggressive preoperative and postoperative management of any associated myasthenia gravis has essentially eliminated the problem of postoperative respiratory failure.
The literature clearly indicates that surgery (particularly complete resection) is an important aspect of a multimodality approach to therapy.
The actual strategies used in our institutions for locally advanced thymomas are multidisciplinary with complete surgical resection when feasible. Neoadjuvant chemotherapy has replaced preoperative radiation therapy in the management of presumed invasive thymomas.
The 10-year actuarial overall survival is 60% to 70%. The overall likelihood of recurrence of a completely resected thymoma ranges from 10% to 30%.
Most authors agree that prolonged follow-up is needed for patients who undergo thymoma resection, because recurrence may occur several years after the operation. Some authors have pointed out that tumor recurrence can happen in most stages and in most histologic types, but most commonly in stage III or IV epithelial thymomas. Recurrences often appear as a local or regional rather than a hematogenous spread. Considering the encouraging results of the aggressive surgical approach, some authors recommend re-resection in resectable recurrent thymomas.
New chemotherapy regimens are leading to multimodality therapies, and data suggest that the response rate to multidrug, cisplatin-based therapy ranges from 50% to 90%. Nonetheless, it will remain difficult to determine their benefits, considering this tumor's rarity, heterogeneity, and indolent natural history.
Thymoma: A multivariate analysis of factors predicting survival.