Endocrine Abstracts

A 52-year-old woman presented in 1998 at age 26 with hirsutism, obesity, and purple striae. She had history of learning difficulties and hyperphagia since age7. Physical examination revealed a supraclavicular pad of fat and hypertension. Biochemical analysis indicated a non-suppressed low-dose dexamethasone suppression test and an ACTH level of 55 ng/l, with all other pituitary hormones normal. MRI findings showed a midline hypodense area anterior to the posterior lobe, with deviation of the stalk to the right. CT scans of the adrenals appeared normal, prompting further investigation with an IPSS revealing a left sided pituitary source. Subsequent transsphenoidal surgery in 2000 successfully excised the adenoma, followed by a postoperative rise in cortisol levels necessitating treatment with metyrapone. In June 2003, she experienced severe headache and dysconjugate movements suggestive of pituitary apoplexy, leading to secondary adrenal failure persisting despite cessation of metyrapone. Hydrocortisone therapy was initiated at a dosage of 20/10/10 and then subsequently reduced to 15/5/5 mg. On further assessment low-dose dexamethasone suppression test results (353 to 45 nmol/l) and an ACTH level of 45.5 ng/l to 14.3 ng/l, off hydrocortisone. From 2014 to 2016, she received escalating doses of metyrapone, followed by a second transsphenoidal surgery in June 2017, excising a large pituitary adenoma with postoperative ACTH levels measuring 186 ng/l. Histology revealed positive staining for ACTH and GH, with a KI67 index exceeding 10%. Despite consideration of conventional radiotherapy, the patient’s inability to tolerate the treatment led to the resumption of metyrapone. A new diagnosis of type 2 diabetes necessitated treatment with Metformin and Trulicity. Bilateral adrenalectomy was deliberated by the multidisciplinary team (MDT) but deemed high-risk due to concerns regarding hydrocortisone compliance. An application for Pasireotide to NHS England was rejected. In 2022, discontinuation of metyrapone due to taste intolerance led to an increase in ACTH levels to 228 ng/l. Subsequent clinical deterioration and non-compliance with oral metyrapone necessitated a renewed successful application for Pasireotide. Despite developing DKA while on Pasireotide, insulin was initiated, achieving moderate biochemical control with Pasireotide, maintaining ACTH levels at 112 ng/l, hydrocortisone day curve of 600-700 nmol/l, serum testosterone reduced from 3.7 to 0.3 nmol/l. This case underscores the complex challenges physicians encounter when managing patients with learning difficulties. Pasireotide was chosen due to its subcutaneous administration, facilitating ease of delivery by healthcare providers. Additionally, her inability to tolerate radiotherapy further complicated the decision-making process. Pasireotide emerged as the more practical choice given the patient’s circumstances.