Treatment Options for Stage III Adrenocortical Carcinoma (ACC)

Complete surgical removal of the tumor, with or without regional lymph node dissection, is the treatment of choice for patients with stage III ACC. The treatment of patients who have tumors with local invasion, but without clinically enlarged regional lymph nodes, is complete surgical removal, as for stage I and stage II tumors. For those with enlarged regional lymph nodes, a lymph node dissection should be included in the procedure. These patients are at high risk of disease recurrence and should consider enrolling in a clinical trial.

Radiation therapy (approximately 50–70 Gy over a period of 4 weeks) may be given to patients with localized but unresectable tumors (under clinical evaluation).[1]

Chemotherapy with mitotane in doses as high as 10 to 12 g/day to achieve a blood level of 14 to 20 mg/L should be considered for patients unable to undergo complete resection (under clinical evaluation). This adrenolytic drug produces useful clinical responses in about 20% to 30% of patients with measurable tumor burden.[2,3]The role of mitotane as adjuvant therapy after complete tumor resection is still unclear but should be discussed with the patient. For patients who undergo a complete resection, the role of adjuvant mitotane and radiation therapy is the same as for patients with stage I and stage II ACC.

Chemotherapy with mitotane plus streptozotocin or mitotane plus etoposide, doxorubicin, and cisplatin may be effective and has been compared in a phase III clinical trial (NCT00924144) (under clinical evaluation).[2]