Beneficial gamma-knife radiosurgery in a patient with Nelson's syndrome.

Department of Endocrinology, University Hospital Maastricht, The Netherlands. bwo@sint.azm.nl A 50-year-old female patient with Cushing's disease had undergone transsphenoidal removal of the pituitary adenoma and conventional radiotherapy in 1988. Since no remission was achieved, she underwent bilateral adrenalectomy in May 1989. During out-patient follow-up, she developed signs and symptoms due to invasive Adrenocorticotrophic hormone (ACTH)-producing macroadenoma (Nelson's syndrome) in 1994. ACTH levels at that time were 3400 ng/l. Near-total surgical resection of a 2.0 x 2.5 pituitary tumour which slightly extended laterally into the cavernous sinus was achieved by subfrontal approach in June 1994. However, she rapidly developed a recurrence of her complaints, with a visual field defect inferior-nasal of the left eye, and a second operation was carried out in November 1994. Culture of the tumour's cells revealed significant inhibition of ACTH production by bromocriptine. Adjuvant treatment with this drug therefore was started in November 1994. Because of the rapid recurrence it was decided to treat her with gamma-knife radiosurgery. The dose that was given in January 1995 was 12 Gy to the border and 40 Gy into the centre of the tumour. During a follow-up of more than 2 years, no recurrence, but even a minor reduction of tumour mass was observed by magnetic resonance imaging (MRI). Plasma ACTH levels decreased gradually to levels between 200 and 400 ng/l, and ophthalmologic complaints disappeared. It is concluded that gamma-knife radiosurgery may be a good alternative for patients with Nelson's syndrome who have rapidly recurring disease