This should be conducted in a specialist centre.
Surgery is the treatment of choice - the prime aim being to render serum cortisol undetectable. Post-operative radiotherapy will be required if the prime aim is not achieved. A trans-sphenoidal approach, in general, is used. A transfrontal approach is the first-line treatment for tumours with large extrasellar extensions (compression of the optic chiasm implies a suprasellar extension of at least 10 mm). Remission is achieved in 75-80% of cases. Bilateral adrenalectomy is less commonly performed but may occasionally be required. Nelson's syndrome is a potential complication and pituitary irradiation is recommended as prophylaxis although not always sucessful.
Pituitary irradiation is now reserved for patients in whom surgery was not entirely successful or was contraindicated or unacceptable. Treatment may be necessary for several years but remission occurs in about 60% of cases.
Metyrapone or ketoconazole may used to lower serum cortisol pre-operatively or as an adjuvant therapy. Metyrapone blocks cortisol synthesis and is effective within 2 hours. It may worsen hirsutism in women. Ketoconazole acts on the adrenal to inhibit cortisol and androgen secretion. Occasionally, bromocriptine may be used.
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