Endocrine Abstracts

BackgroundPPAR-gamma agonists have been proposed as therapy to lower plasma ACTH in Cushing's disease. However, cyclical secretion may explain some of the 'responses' seen. In contrast, patients with Nelson's syndrome have continual high ACTH levels, and can present with pituitary mass effects and pigmentation. Since no established medical therapy exists, we assessed whether prolonged high-dose rosiglitazone therapy reduces circulating ACTH levels in Nel...

BackgroundPPAR-gamma agonists have been proposed as therapy to lower plasma ACTH in Cushing's disease. However, cyclical secretion may explain some of the 'responses' seen. In contrast, patients with Nelson's syndrome have continual high ACTH levels, and can present with pituitary mass effects and pigmentation. Since no established medical therapy exists, we assessed whether prolonged high-dose rosiglitazone therapy reduces circulating ACTH levels in Nel...

ObjectiveTo assess the results of bilateral adrenalectomy and further assess the relevance of Nelson's syndrome as a complication of bilateral adrenalectomy.Patients33 patients treated by bilateral adrenalectomy at the Hammersmith Hospital were identified (earliest operation performed in 1958). Although all bilateral adrenalectomies in recent years were included, it was not possible to confirm that all bilate...

Nelson syndrome (N S) is the set of symptoms related to a pituitary macroadenoma secreting ACTH developed following a bilateral adrenalectomy (BA). Its frequency is thought to account for up to 40% of adult cushing diseases (CD) who have undergone BA. We report the case of a patient with macroinvasive NS revealed by pituitary apoplexy. Mr M.L aged 35 years, followed for CD with negative imaging evolving for ten years, treated with BA. The evaluation at 03 months post-surgery n...

Introduction: Atypical pituitary adenomas are often characterized by rapid growth and massive invasion of the surrounding structures. Usually, they are associated with poor prognosis and high recurrence rate due to resistance to conventional therapies. When surgery and radiation are ineffective, alternative therapies remain the last line treatment. Lately, a novel chemotherapy with capecitabine and temozolomide was proposed as a highly effective and extending the life of the p...

Introduction: Nelson’s syndrome (NS) is an exceptionally rare condition diagnosed sometimes after bilateral suprarenalectomy for Cushing’s disease (CS) involving rapid enlargement of a pre-existing ACTH-secreting pituitary tumor. The clinical picture varies from hyperpigmentation, headache and visual disturbance to diabetes insipidus and hypopituitarism if the hypothalamic-pituitary portal system is disrupted or normal pituitary tissue is destroyed by the adenoma. Th...

Nelson’s syndrome (NS) is a rare clinical syndrome of an enlarging, aggresive corticotroph pituitary adenoma that can occur following bilateral adrenalectomy performed in the treatment of CD.The aim of this work is the evaluation of the early and long-term results of the microsurgery in a single surgeon’s series of patients with NSDuring the period from January 2000 to December 2005 - ten patients with NS have been operat...

Background: Mutations in succinate dehydrogense-B (SDH-B) or von Hippel Lindau (VHL) genes can result in chromaffin tumours.Objective: To compare the clinical phenotypes of subjects developing chromaffin tumours as a result of SDH-B or VHL mutations.Subjects: Thirty-one subjects with chromaffin tumours. Sixteen subjects had SDH-B gene mutations and 15 subjects had a diagnosis of VHL.<p ...

Introduction: Nelson’s syndrome is a rare complication of Cushing’s disease treated with bilateral adrenalectomy. There is no effective medical treatment yet. Nelson’s patients respond to hypothalamic stimuli distinctly from patients with Cushing’s disease and those with Addison’s disease. We describe the responses to standard ACTH stimulation tests in a patient with Nelson’s syndrome.Case report: A 42-year-old woman present...

Pituitary apoplexy in patients with Nelson’s syndrome has rarely been described. We report the case of a 33-year-old woman with a history of bilateral adrenalectomy for Cushing’s disease three years previously. Following adrenalectomy, despite adequate replacement therapy with prednisone and fludrocortisone, she became hyperpigmented, morning serum ACTH level rose from 185 to over 1250 pg/ml (normal range 10–60) and the pituitary tumour increased from 0.8/0.3 to...