Carcinoma of the Adrenal Cortex

Adrenal cortical carcinoma is a rare tumor that affects all ages and sexes and has a dismal prognosis. Half are functional, half are not. Early stages are uncommon and are detected by their endocrine effects, usually in females, as incidental findings at surgery or on computed tomography scans. Arteriography, venography, and CT-scan define the local disease. Endocrine evaluations may delay surgery and do not affect the treatment. Radical local surgery with wide exposure is indicated in all adrenal tumors except the radiologically defined small tumor of pure primary hyperaldosteronism. Thoracoabdominal incisions, extra-fascial dissection, nephrectomy, splenectomy, and partial pancreatectomy may be used. Adjunctive chemotherapy or radiation have not been tested. Cures are recorded only in widely-excised, low stage tumors without evidence of metastases (T 1-2 NOMO). Ortho-p-DDD is useful in less than half the cases, functional or not. Phase II studies and collaborative combined chemotherapy treatment studies are badly needed.