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The main therapy is represented by radical surgery conducted by an expert surgeon. Adjuvant mitotane has to be started in patients with high risk of recurrence. In patients with inoperable disease, the scheme etoposide, doxorubicin, cisplatin with mitotane (EDP-M) is the most employed. Few data are available on second-line and third-line chemotherapy in patients with disease progression after etoposide, doxorubicin, cisplatin with mitotane (EDP-M). Currently, the role of targeted therapies is under evaluation.
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