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Introduction: Cushing’s disease (CD), a disorder of elevated cortisol secondary to an ACTH-secreting pituitary tumor, is often first treated with transphenoidal resection (TSR). However, recurrences of CD can occur after TSR, and other treatment modalities such as radiotherapy and bilateral adrenalectomy (BLA) must often be utilized. Here we present an interesting case of recurrent CD and the treatment challenges faced. Clinical Case: A 24-year-old Caucasian female was diagnosed with CD and treated with TSR. Post-operatively, she was pan-hypopit with diabetes insipidus and placed on full replacement therapy. Six years after surgery, she developed the same Cushingoid symptoms that had been present prior to her surgery. In spite of holding her hydrocortisone for four months, she had an elevated serum cortisol level after a 1mg dexamethasone suppression test of 12.4 mcg/dL (<1.8mcg/dL), and elevated urinary free cortisol (UFC) studies ranging from 58-104.8 mcg/24h (4.0-50.0 mcg/24h). ACTH was 32 pg/mL (6-50 pg/mL). She underwent a second TSR at the age of 31 years. She was restarted on hydrocortisone which was discontinued after two months. Five months after surgery, she had an elevated 24-hour UFC of 105.6 mcg/24hr and ACTH of 25 pg/mL. She then underwent laparoscopic BLA one year after her second TSR. Adrenal pathology showed bilateral nodular adrenal hyperplasia. Steroid replacement therapy was resumed. Three years after BLA, she again developed CD symptoms despite weaning her hydrocortisone. An evening salivary cortisol was elevated at 1.48 mcg/dL (≤0.09 mcg/dL) and serum ACTH was 386 pg/mL (0-45 pg/mL). A CT abdomen without contrast showed a surgically absent right adrenal gland and a 9mm soft tissue density nodule in the left adrenal fossa suggestive of residual/ectopic adrenal tissue. An MRI brain with and without contrast showed a small focus of enhancing tissue in the right cavernous sinus measuring 7mm x 6mm x 8mm concerning for residual tumor. Findings were consistent with recurrent CD secondary to Nelson’s syndrome stimulating remnant adrenal tissue. She underwent a third TSR followed by pituitary radiosurgery (20 Gy) and stereotactic radiotherapy to the adrenal tissue (39 Gy in 3 fractions) six months post-op. Her serum morning cortisol dropped rapidly from 107.4 mcg/dL (5.0-23.0 mcg/dL) before radiation to 1.6 mcg/dL after. Cortisol replacement therapy was resumed, and the patient has remained cured of CD for over two years. Conclusion: In patients with Nelson’s syndrome, any remnant or ectopic adrenal tissue can produce cortisol. This is the first report of adrenal ablation by radiosurgery in this setting, leading to cure of CD.

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Background Traditionally, stimulated serum cortisol &lt; 18 mcg/dL at 30 or 60 minutes has been used as the cutoff for diagnosis of adrenal insufficiency (AI) during high dose cosyntropin stimulation testing (CST). Recent studies suggest that a cutoff of 14-15 mcg/dL is more accurate when using more specific monoclonal immunoassays (IAs). In February 2017, our institution switched from a polyclonal IA to a monoclonal IA. Despite this change, providers were diagnosing AI based on the 18 mcg/dL cutoff. Long term glucocorticoid use has several unwanted pleiotropic effects such as impaired glucose tolerance or diabetes mellitus (DM), hypertension (HTN), and increased risk of infection. Our study aims to evaluate patients with peak stimulated cortisol levels between 14-18 mcg/dL diagnosed with AI and placed on steroids. Methods We retrospectively reviewed charts of patients with peak cortisol levels 14-18 mcg/dL at 30 or 60 minutes on high dose CST with 250 mcg cosyntropin between February 2017 and August 2021. Cortisol levels were assessed using the monoclonal IA Roche Elecsys Cortisol II. Data regarding type and etiology of AI as well as patient demographics, comorbidities, steroid regimen, and follow up cortisol testing was collected. Worsening HTN and DM were defined as increase in blood pressure and HgbA1c respectively and/or intensification of medications between baseline at time of CST and last follow up while on steroids. Results 41 patients met inclusion criteria. 29 (71%) patients were female and mean age at CST was 52 years (range 21-86). Most patients were diagnosed with secondary (71%) or partial secondary (19%) AI by provider. Most common etiologies were pituitary disease (45%), chronic steroid use (24%), and unilateral adrenalectomy (19%). Mean follow up was 23 months (range 0.5-57). 33 (80%) patients were started or continued on glucocorticoid replacement. Remaining patients who were never started on steroids had a peak cortisol ≥ 15.6 mcg/dL. 21/33 (64%) of patients started on steroids had repeat adrenal function testing a mean of 18 months (range 2-42) after the initial CST. Based on testing or clinical judgement, 19/33 (58%) were taken off steroids. In patients treated with steroids, 64% developed weight gain, 42% worsening HTN, and 6% worsening DM. Additionally, 48% developed an infection, 24% insomnia, and 15% psychiatric illness. Conclusions The majority of patients with peak stimulated cortisol between 14-18 mcg/dL on high dose CST had secondary AI. Over 50% of patients started on steroids were later able to stop. Steroid-related complications were observed, but this may not differ significantly from the general population. This study underscores the importance of interpreting CST results in the context of the cortisol assay used and evaluating patients with secondary AI for recovery of the hypothalamic-pituitary-adrenal axis. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m., Saturday, June 11, 2022 1:06 p.m. - 1:11 p.m.

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