Unilateral laparoscopic adrenalectomy following partial transsphenoidal adenomectomy of pituitary macroadenoma - life-saving procedure in a patient with ACTH-dependent Cushing's syndrome.

ABSTRACT

INTRODUCTION: Cushing's disease is the most common cause of endogenous hypercortisolemia, in 90% of cases due to microadenoma. Macroadenoma can lead to atypical hormonal test results and complete removal of the tumour is unlikely. CASE REPORT A 77-year-old woman with diabetes and hypertension was admitted because of fatigue, proximal muscles weakness, lower extremities oedema, and worsening of glycaemic and hypertension control. Physical examination revealed central obesity, 'moon'-like face, supraclavicular pads, proximal muscle atrophy, and skin hyperpigmentation. Biochemical and hormonal results were as follows K 2.3 mmol/L (3.6-5), cortisol 8.00 86 µg/dL (6.2-19.4) 23.00 76 µg/dL, ACTH 8.00 194 pg/mL (7.2-63.3) 23.00 200 pg/mL, DHEAS 330 µg/dL (12-154). CRH stimulation test showed lack of ACTH stimulation > 35%, overnight high dose DST revealed no suppression of cortisol. Chest and abdominal CT as well as somatostatin receptor scan didn't show ectopic tumour responsible for ACTH oversecretion. MRI revealed a pituitary macroadenoma (28 × 20 × 17 mm) extending towards the left cavernous sinus. After partial transsphenoidal adenomectomy, serum cortisol of 40 µg/dL was obtained. The patient's condition was still very poor, so unilateral adrenalectomy was performed. After surgery, serum morning cortisol level dropped to 20 µg/dL and the patient's condition improved significantly. 26 months after the operations, the patient remains in remission. Because her life expectancy exceeds the prognosed duration of remission with the presence of pituitary tumour remnants and intact left adrenal gland, the patient was qualified to radiosurgery with a Gamma Knife. CONCLUSION: In selected cases, unilateral, laparascopic adrenalectomy may serve as a life-saving procedure in a patient with ACTHdependent Cushing's syndrome.