Recurrence of Ectopic Cushing's Syndrome 10 Years after Bilateral Adrenalectomy

Presentation Number: MON 411
Date of Presentation: April 3rd, 2017

Sablaa Ali* and Yusef Hazimeh
Arnot Ogden Medical Center, Elmira, NY


Background: Ectopic Cushing’s syndrome occurs in 10-15% of ACTH-dependent Cushing's syndrome. The treatment is usually resection of the ACTH producing tumor. In certain cases bilateral adrenalectomy is done as a final resort to treatment. We present a patient who had recurrence of ectopic Cushing’s syndrome 10 years after bilateral adrenalectomy.

Clinical Case: A 38 year old male was diagnosed with Cushing's syndrome in 2003. His initial workup included a pituitary evaluation and petrosal sinus sampling. Ultimately he was found to have ectopic ACTH production secondary to a lung tumor. He underwent left lung lobectomy, however, this did not normalize his ACTH and cortisol levels, and he underwent a bilateral adrenalectomy in 2006.

He moved to our practice in 2016 and was noted to have easy bruising, flushing, weight gain and central obesity. Workup for recurrence of his Cushing's revealed a 24-hour urine cortisol of 900 mcg/24hr (normal 3.5-45). Morning cortisol level post 1 mg dexamethasone suppression was also significantly elevated at 14.2 ug/dL (normal <1.8). This was in the setting of an elevated ACTH level of 187 pg/mL (normal 10-60). MRI of the pituitary did not reveal any tumor. An octreotide scan was consistent with hilar carcinoid tumor, showing two new foci. However, CT chest did not reveal any tumors. CT scan with adrenal protocol was performed and confirmed bilateral adrenal hyperplasia. He was evaluated by surgery and deemed a poor candidate for a second adrenalectomy. Evaluation with ultrasound-guided endobronchial biospy of a pulmonary lymph node was consistent with metastasis from carcinoid tumor. Surgical resection was not possible due to the small size of these lymph nodes and the inability to identify them on CT scan. He was therefore referred for radiation therapy for these lymph nodes.

Conclusion: Patients with ectopic Cushing's syndrome are prone to have recurrence, despite bilateral adrenalectomy. We postulate that the continuous presence of ACTH production was able to stimulate regrowth of any remnant adrenal gland tissue, and precipitated the disease again. This case shows the need for continuous monitoring of these patients.


Nothing to Disclose: SA, YH