Pituitary irradiation is an effective and well-tolerated treatment for patients with Cushing's disease in whom transsphenoidal surgery is unsuccessful.

We present the ACROQOL questionnaire, developed and preliminarily validated to specifically assess HRQOL in patients with acromegaly.

Our report illustrates the high incidence of endocrine deficits in patients with craniopharyngioma. Additional hypothalamic-pituitary dysfunction usually occurs following surgical treatment of these tumours.

A series of 35 patients with non-functioning pituitary adenomas undergoing transsphenoidal surgery is presented. In most cases, the presenting symptoms were related to the mass effect of the tumor. There was no operative mortality. Before surgery, visual field defects were documented in 21 patients (60%). After surgery, excluding 3 patients with preoperative blindness, 28% regained normal vision and 67% showed variable improvement. Preoperatively, 24 patients (69%) had abnormal pituitary function, 24 (69%) had hypogonadism, 7 (20%) adrenal insufficiency, 8 (23%) hypothyroidism and 2 (6%) panhypopituitarism. After pituitary surgery, all but one patient with normal preoperative function retained it. Of the patients with hypopituitarism, 11 (46%) had variable improvement and 13 (54%) had persistent deficits. After surgery, 4 patients (57%) with adrenal insufficiency recovered normal adrenal function, 7 patients (29%) with hypogonadism recovered gonadal function and 1 patient (13%) with hypothyroidism recovered thyroid function. Prior to surgery, the presence of a normal or slightly elevated PRL and a rise in TSH after TRH and in LH after GnRH stimulation were of value in predicting possible recovery of pituitary function after surgery. These observations suggest the presence of viable pituitary tissue in these cases and point out that, in some instances, the mechanism of hypopituitarism may be compression of the portal circulation, rather than destruction of the normal pituitary gland.

Context: Pegvisomant is an effective treatment for somatostatin analogue-resistant acromegaly, but the determinants defining the response to this treatment are largely unknown. Objective: To investigate the efficacy of pegvisomant treatment in resistant acromegalic patients (e.g. serum IGF1 at least 1.25!upper normal limit) in a clinical setting and the factors conditioning this response. Design and setting: A retrospective cross-sectional study performed in six Spanish University hospitals from 2004 to 2007. Patients: Forty-four acromegalic patients (61.4% female, mean age: 49G14), 95% of whom had undergone pituitary surgery and 61% having received pituitary radiotherapy. The mean follow-up was 22.7G11.2 months. Main outcome measures: IGF1 levels reflected treatment efficacy, and the influence of gender, age, weight, previous radiotherapy and duration of treatment was assessed. Results: IGF1 normalisation was achieved in 84% of the patients. Male gender (P!0.05), previous irradiation (P!0.05) and the treatment duration (rZ0.364, P!0.02) were associated with a better response to pegvisomant therapy. There was a significant decrease in HbA1c (P!0.001) and in the mean insulin dose (P!0.01) in acromegalic diabetic patients. Although 25% of patients experienced mild adverse events, pegvisomant was only withdrawn in four patients due to side effects (two cases of tumour growth, one liver dysfunction and one headache). Conclusions: Long-term pegvisomant is a very effective therapy in resistant acromegaly. Male gender and prior radiotherapy influence the therapeutic response rate.

A short administration of preoperative lanreotide 30 mg induced a statistically significant shrinkage of GH-secreting pituitary adenomas where this reduction was > 20% of the pretreatment value in 29% of the whole population. Among the factors considered was the fact that positive hormone response to preoperative lanreotide 30 mg was the sole predictive factor of this significant tumour volume reduction.

Only when a significant IPS:P ACTH ratio is present can Cushing's disease be established by IPS sampling. The absence of a significant IPS: P ACTH ratio does not necessarily imply ectopic secretion of ACTH, nor does it exclude Cushing's disease. The results of lateralization by IPS sampling do not remove the need for a thorough transsphenoidal examination of the contents of the sella turcica.

Objective: ACTH-independent macronodular adrenal hyperplasia is a rare cause of Cushing's syndrome. Bilateral adrenalectomy is considered the treatment of choice, but the patient is obliged to receive lifetime steroid replacement therapy and is susceptible to adrenal insufficiency crisis. New therapeutic alternatives are being proposed as new etiopathological features of the disease are known. Unilateral adrenalectomy of the largest gland can be a safe and effective alternative, but only short-term follow-up is reported in the literature. We present four consecutive patients with ACTH-independent macronodular hyperplasia and long-term remission of Cushing's syndrome after unilateral adrenalectomy. Subjects: Four consecutive patients (two males and two females, mean age 50.3 years) with Cushing's syndrome due to ACTH-independent macronodular adrenal hyperplasia underwent unilateral adrenalectomy of the largest gland. Results: The weight of the resected glands ranged from 26.8 to 210 g. Two patients suffered transient post-surgical adrenal insufficiency and had steroid replacement therapy for 60 and 14 months respectively. After a mean follow-up of 78.8 months (range 30-137 months) all the patients persist without any evidence of Cushing's syndrome. Urinary free cortisol and serum cortisol, after the adrenal insufficiency stage, have always stayed within their normal ranges, but cortisol circadian rhythm and suppressibility after dexamethasone have never normalized. No further enlargement of the contralateral gland has been documented 62 to 126 months after surgery in three of the four patients. Conclusions: Unilateral adrenalectomy can be an effective and safe alternative treatment for ACTH-independent macronodular adrenal hyperplasia, and can achieve long-term remission of Cushing's syndrome.