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  • Author: Richard G. Pestell x
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Richard G. Pestell, Frank P. Alford and James D. Best

Abstract.

Five family members over 3 generations had isolated functional pituitary adenomas diagnosed. In four cases acromegaly was diagnosed, and in the fifth galactorrhoea from prolactin excess was the presenting feature. A prominent feature of the affected members tumours were histological finding of either atypical mixed cell or undifferentiated cell type. Given the low incidence of acromegaly and the demonstration of vertical transmission over 3 generations it is suggested this family represents an inherited pituitary syndrome, distinct from Multiple Endocrine Neoplasia (MEN) type 1.

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Richard D Arnott, Richard G Pestell, Penelope A McKelvie, J Keith Henderson, Peter M McNeill and Frank P Alford

Abstract.

Twenty-eight patients underwent transsphenoidal pituitary surgery for Cushing's disease. Selective surgical procedures were performed in 26. In 19 patients pituitary adenoma was confirmed histologically and 5 had clumps or clusters of ACTH-staining cells. At follow-up after 22.3 (range 5-56) months 21 (75%) patients remained in clinical and biochemical remission. The 24-h urinary free cortisol performed between 1 and 4 weeks postoperatively while patients took replacement doses of dexamethasone (0.5 to 0.75 mg per day) was found to predict outcome. All patients with suppressed urinary free cortisol excretion have remained in remission, 3 with levels in the normal range have suffered late relapse after initial remission, and 4 with elevated urinary free cortisol excretion were regarded as having failed to remit. Furthermore, all patients with unsuccessful outcomes had no pituitary adenoma and 3 had features consistent with corticotrope nodular hyperplasia at histological examination compared to only 2 and 1, respectively, of patients still in remission. We conclude that 24-h urinary free cortisol performed at 1 to 4 weeks postoperatively whilst on low-dose dexamethasone and the histological findings may predict outcome following transsphenoidal pituitary surgery in Cushing's disease.

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Patricia A. Crock, Richard G. Pestell, Anthony J. Calenti, Eric J. Gilford, J. Keith Henderson, James D. Best and Frank P. Alford

Abstract. Pre-operative bilateral simultaneous inferior petrosal sinus sampling with assessment of ACTH levels in the left and right sinuses and the periphery was performed in 9 patients with pituitary dependent Cushing's disease who were subsequently found at surgery to have basophil microadenomata. The novel observation of this study was the pattern of secretion of other pituitary hormones so that significant inter-sinus gradients ≥ 1.4:1 were seen for β-endorphin (2.8 ± 1.3, mean ± sem), PRL (4.2 ± 1.3) and GH (6.9 ± 2.4) as well as for ACTH (5.1 ± 1.1). There was no inter-sinus gradient for LH, FSH and TSH. In these 9 patients with adenomata, the correlations between the inter-sinus gradients for ACTH and β-endorphin were r = 0.95 (P <0.01), ACTH and PRL r = 0.90 (P < 0.01) and for ACTH and GH r = 0.89 (P <0.05). This close association between the gradients for ACTH and other anterior pituitary hormones could be due either to cosecretion of β-endorphin, PRL and GH by the ACTH-producing pituitary adenomata or to a paracrine effect of β-endorphin from the tumours on adjacent pituitary tissue. By reflecting the central pituitary hormone milieu, petrosal sinus sampling can give information about pituitary function unobtainable from peripheral hormone levels.