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Work-up
CT scan of the thorax
Extrathoracic disease such as metastases to the kidney, bone, liver, and brain are rare. Consequently, an extensive work-up for disease outside the thorax is not indicated in the absence of symptoms
Pleural or pericardial effusion represents the most common form of metastatic involvement
Proper intrathoracic staging is surgical
Ströbel P et al. Blood 2002;100:159–166
Thomas CR Jr et al. J Clin Oncol 1999;17:2280–2289
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Despite their rarity as a group of cancers, thymic epithelial tumors are amongst the most common cancers of the anterior mediastinum in adults
The clinical course can vary from relatively indolent in the case of some thymomas to highly aggressive in the case of thymic carcinomas
Thymomas are often associated with a variety of autoimmune conditions; myasthenia gravis is the most common
Complete surgical resection should be attempted whenever feasible
Multimodality treatment is frequently required for locally advanced thymoma and thymic carcinoma
Chemotherapy is offered to patients with advanced stages III to IV thymomas. The evidence for these recommendations is derived from small phase II studies in either the neoadjuvant or refractory/recurrent disease setting
Platinum-based combination chemotherapy is the standard of care for unresectable, advanced disease. A combination of cisplatin, doxorubicin and cyclophosphamide (PAC) or cisplatin and etoposide (EP) are usually used as first-line regimens [Table]
The molecular pathogenesis of thymic epithelial tumors and associated autoimmune conditions is gradually being unraveled and targeted therapy is being investigated for the management of advanced disease
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