SAT-018 - Recurrent Cushing Disease Complicated by Gadolinium Allergy and Panhypopituitarism Following Repeat Transsphenodial Surgery

The primary treatment for Cushing's Disease is transsphenoidal resection (TSS), with a cure rate of about 80% following initial surgery. Higher remission rates are seen in patients with microadenomas (83%) versus macroadenomas (68%). Additional factors predicting remission include adenoma visibility on preoperative imaging, histopathology confirming adenoma, absence of cavernous sinus invasion, and low postoperative cortisol levels. Rate of recurrent disease is up to 18% following initial surgery, with highest incidence in the first five years. Revision TSS is associated with higher recurrence rate, up to 28%, with lower remission rates in patients with persistent disease (54%) versus recurrence (80%). At the same time, risk of structural and endocrinological complications of TSS rise with subsequent surgeries, including diabetes insipidus (DI) and hypopituitarism. An alternative to surgery is medical management, such as with osilodrostat, to reduce cortisol levels without incurring operative risks. However, it requires extensive lab monitoring for dose titration and poses risks of adverse effects including adrenal insufficiency and arrhythmia.
We present a patient who developed recurrence of Cushing disease after primary TSS and persistence of disease after revision TSS, complicated by panhypopituitarism. Her management is also complicated by severe allergy to gadolinium limiting pre- and postoperative imaging. Through robust interdisciplinary communication between medical endocrinology, neurosurgery, radiology, and centered around the patient's interests, a decision was reached to hold off on further surgical intervention and initiate osilodrostat therapy for medical management.
In summary, a 36-year old female patient initially presented with amenorrhea and was diagnosed with pituitary macroadenoma associated with hyperprolactinemia, which did not improve on cabergoline. She underwent TSS with postoperative adrenal insufficiency, and pathology revealed corticotroph adenoma with aggressive features. Adrenal insufficiency resolved after one year, and symptoms recurred with amenorrhea, migraines and leg edema. However, repeat non-contrasted MRI and cortisol levels remained stable, despite elevated ACTH. Symptoms progressed, and she ultimately had radiologic and biochemical evidence of recurrent disease, prompting second TSS 3.5 years after the primary surgery. Cortisol level improved to normal range, but she developed diabetes insipidus, central hypothyroidism, and ongoing amenorrhea. After four months, patient had new biochemical evidence of hypercortisolemia, without clear change on non-contrast imaging. Risks and benefits of a third surgery with suboptimal preoperative imaging were weighed with potential complications of medical therapy, and the patient ultimately elected to pursue medical management with osilodrostat.

*Unless otherwise noted, all abstracts presented at ENDO must not be released to the press or the public until the date and time of presentation. For oral presentations, the abstracts are embargoed until the session begins. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO.*