Complete surgical removal of all tumor is the only known way to cure adrenal cancer. The best opportunity for complete removal is at the first operation. Although laparoscopic removal is preferable for most small benign tumors, laparoscopic removal of ACC is very controversial with several noted endocrince surgeons being strongly opposed to this approach. These surgeons believe that all ACC should be removed with an open operation if it is known pre-operatively that the tumor is cancer. These surgeons have published data suggesting that patients undergoing laparoscopic resection of their ACC have a higher and earlier rate of tumor recurrence. A recent study from Europe, however, suggests that for tumors smaller than 10 cm, the results of laparoscopic surgery are equivalent to open.
After surgery to remove the adrenal gland and surrounding tissue, patients may receive radiation or chemotherapy to reduce the risk that the ACC may come back. Mitotane, a drug that is a chemical relative of the pesticide DDT, has been shown to reduce the risk of tumor recurrence.
For advanced or metastatic ACC, the current standard chemotherapy regimen is a combination of drugs including doxorubicin, cisplatin, etoposide and mitotane. The most recent clinical trial comparing this combination to streptozotocin and mitotane (FIRM-ACT study) has been completed and results are pending. Results from a previous smaller study showed a response rate of 48%. For patients whose tumors progress despite standard chemotherapy, there are no proven second-line options. A multi-site phase III clinical trial using OSI-906, a blocker of insulin growth factor receptor type 1 has been completed and the results are pending.
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