# Hypercortisolism Due to Paraganglioma Secreting Adrenocorticotropin and Catecholamines

## TL;DR

A patient with a paraganglioma tumor had both high cortisol and catecholamines, leading to Cushing syndrome, and was successfully treated with surgery and medication.

## Contribution

This case highlights a rare paraganglioma that secretes both ACTH and catecholamines, causing ectopic Cushing syndrome.

## Key findings

- The patient's symptoms resolved after surgical removal of the ACTH- and catecholamine-secreting paraganglioma.
- Pathology confirmed the tumor produced ACTH, confirming ectopic Cushing syndrome.
- Osilodrostat reduced cortisol levels before surgery, aiding in management.

## Abstract

A paraganglioma is a neuroendocrine tumor classically associated with catecholamine production. We describe a 71-year-old woman with an incidentally identified para-aortic mass who later developed hyperglycemia, hypertension, hypokalemia, and leukocytosis. Work-up ultimately revealed significantly elevated adrenocorticotropin (ACTH), cortisol, and metanephrines, and biopsy of the mass suggested paraganglioma cosecretion of both ACTH and catecholamines. Using osilodrostat to decrease her excess cortisol production, she underwent successful surgical paraganglioma resection. Pathology of the mass demonstrated a paraganglioma with ACTH-producing cells, confirming the diagnosis of ectopic Cushing syndrome (CS). Following resection, the patient had resolution of hypertension and hyperglycemia and normalization of the hypothalamic-pituitary-adrenal axis. We describe the work-up and important perioperative and long-term management considerations for patients with hypercortisolism from ectopic CS and catecholamine excess.