Early Detection of Recurrent Cushing's Disease: Proposal of a Novel Post-Surgical Monitoring Algorithm

Presentation Number: SAT-0648
Date of Presentation: June 21st, 2014

Alejandro Ayala*1 and Alex Manzano2
1University of Miami Miller School of Medicine, Miami, FL, 2Univ of Miami Miller Schl of Med, Coral Gables, FL


Cushing’s disease is a rare and devastating neuroendocrine disorder characterized by chronic hypercortisolism due to a hyperactive ACTH-secreting pituitary adenoma. Patients with Cushing’s disease often develop debilitating sequelae (eg, cardiovascular disease, glucose intolerance, cognitive impairment) (1), underscoring the urgent need for early diagnosis and treatment. Transsphenoidal surgery (TSS) is generally first-line treatment for Cushing’s disease. Remission rates following TSS are highly variable and disease recurrence, even several years after early remission, is not uncommon (2). Recognizing Cushing’s disease recurrence can be challenging, and although there is general acceptance among endocrinologists that surgically-treated patients need lifelong follow-up, there are currently no standardized guidelines for long-term monitoring following TSS. To begin addressing this need we have created a systematic monitoring algorithm to facilitate early detection of recurrent Cushing’s disease following TSS. We integrated information from PubMed-indexed articles that reported relapse rates in surgically-treated Cushing’s disease patients and our own clinical experiences to generate this novel algorithm. Reported rates of Cushing’s disease recurrence following surgery range from 3 to 47% (mean time to recurrence 16–49 months). We propose that patients with post-operative serum cortisol levels, measured 2–3 days after surgery, of <2 µg/dL (ie, patients in immediate remission) be monitored semiannually for 3 years and annually thereafter. Patients with postoperative cortisol levels of 2–5 µg/dL may experience subclinical Cushing’s disease or persistent, overt disease and should be evaluated every 2–3 months until biochemical control is achieved or additional treatment is initiated. Patients with postoperative cortisol levels of >5 µg/dL often have persistent disease, and second-line treatment, such as immediate repeat pituitary surgery, radiotherapy, and/or medical therapy may be considered. This follow-up algorithm is proposed with the aim to, (a) serve as a tool to enable early diagnosis and treatment of recurrent Cushing’s disease, thereby minimizing the detrimental effects of hypercortisolism, and (b) begin addressing the need for standardized guidelines for vigilant monitoring of Cushing’s disease patients treated by TSS, as is demonstrated by the reported rates of recurrence.


Disclosure: AA: Speaker, Pfizer, Inc.. Nothing to Disclose: AM