A Second Look at Cushing Disease: Hypercortisolism Recurrence From Another Gland

Area of Science:

• Endocrinology
• Oncology
• Neurosurgery

Background:

• Cushing disease (CD) is the most common cause of ACTH-dependent Cushing syndrome (CS).
• Unilateral adrenal adenoma is the most common cause of ACTH-independent CS.
• Concurrent or sequential occurrence of different Cushing syndrome subtypes in a single patient is exceptionally rare.

Observation:

• A 44-year-old woman with a history of left adrenalectomy and thyroidectomy presented with sequential CS subtypes.
• Initially diagnosed with CD, she achieved remission post-pituitary surgery.
• Subsequently, she developed adrenal CS due to a right adrenal adenoma.

Findings:

• The patient opted for medical management over a second adrenalectomy for the right adrenal adenoma.
• After 7 years, the adenoma remained stable, and she achieved biochemical control with low-dose osilodrostat.
• This demonstrates successful long-term management of a distinct secondary CS after treated CD.

Implications:

• Highlights the importance of recognizing rare sequential or coexisting CS subtypes.
• Emphasizes the need for individualized treatment strategies based on patient preference and clinical course.
• Suggests medical management with agents like osilodrostat can be effective for adrenal CS in select cases.