Category: Adrenal Disorders

Monitor: 18

18 - THE COMPLEX CLINICAL MANAGEMENT OF AN ACTH-SECRETING PHEOCHROMOCYTOMA

Saturday, Apr 27
10:00 AM – 10:30 AM

Objective : We present a case of an ACTH-secreting pheochromocytoma resulting in hypertension (HTN) and hypercortisolism-induced hyperglycemia. This case was also complicated by ketoconazole-induced liver dysfunction.


Methods : A 47-year-old female was referred for suspected pheochromocytoma.


Results :

Biochemical testing revealed elevated labs including norepinephrine 1887 pg/mL (112-750), epinephrine 288 pg/mL (0-50), dopamine 31 pg/mL (0-29), normetanephrine 366 pg/mL (18-112), metanephrine 195 pg/mL (12-61), cortisol 84.7 mcg/dL (5-25), ACTH 417 pg/mL (5-46), glucose 270 mg/dL, HbA1c 6%. Imaging was significant for a 2.6cm right adrenal mass on Ga-68 DOTATATE, F-DOPA. PET, CT, and MRI showed a right adrenal pheochromocytoma, as well as a left pelvic mass which was suspected to be a fibroid. Vital signs included blood pressure 122-172/68-100, heart rate 63-99. Medications included ketoconazole, spironolactone, phenoxybenzamine, labetalol, detemir, lispro, potassium chloride, and sulfamethoxazole/trimethoprim.
As ketoconazole was increased to 400 mg Q6h to block cortisol excess (cortisol 84.8--> 22.6 mcg/dL), the patient developed elevated liver enzymes. Ketoconazole was decreased, resulting in a concomitant increase in cortisol to 108 mcg/dL. HTN was difficult to control on α-adrenoceptor blockade with phenoxybenzamine 10mg BID, β-blockade labetalol 250mg BID, and spironolactone 100mg QHS. The patient was transferred to the ICU for HTN and diabetes control. The patient underwent surgical resection of the right adrenal mass. Pathology was significant for pheochromocytoma, and positive for synaptophysin, chromogranin, S-100 protein, and ACTH. The patient had complete resolution of hyperglycemia so insulin was discontinued. Blood pressure remained elevated, requiring amlodipine 10mg and atenolol 12.5mg BID.


Discussion : Cushing syndrome in the setting of an ACTH-secreting pheochromocytoma is rare (1,2,3). Simultaneous treatment of hypercortisolism and catecholamine/metanephrine excess can be quite challenging. HTN control must be adequate with α and β-adrenoceptor blockers. Glucocorticoid secretion may contribute to catecholamine excess, thus increasing risk of hypertensive crisis (2). Patients may require IV anti-HTN therapy and ICU monitoring while awaiting surgery. Hypercortisolism-induced hyperglycemia likely warrants treatment with insulin due to glucocorticoid-induced insulin resistance (1).


Conclusion : In this case, we used detemir and lispro given the postprandial glucose excursion in hypercortisolemic patients. The complex pathophysiology in this case translated to simultaneous management of HTN and hyperglycemia control with an ultimately favorable outcome.

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Natalie Mora

Endocrinology Fellow
National Institutes of Health
Bethesda, Maryland

Dr.Mora is from San Antonio, Texas and is a graduate of SUNY Upstate Medical University. She trained in internal medicine at the University of Virginia and is a current endocrinology fellow at NIH. Dr.Mora's main interest is diabetes, nutrition, and health disparities.

Karel Pacak

Senior Investigator, Chief of Section on Medical Neuroendocrinology National Institute of Child Health and Human Development (NICHD)
National Institutes of Health

Dr. Karel Pacak graduated summa cum laude from Charles University, Prague, Czech Republic in 1984. In 1990 he began his postdoctoral fellowship at NINDS. Then in 1995 Dr. Pacak began his residency in internal medicine at the Washington Hospital Center under Dr. L. Wartofsky, followed by a fellowship in endocrinology, diabetes, and metabolism at NIH. He was Board certified in 1998 and 1999 in those disciplines. In 1998 he established a new Program for Neuroendocrine Tumors focusing on pheochromocytoma and paraganglioma at NICHD. He received his Ph.D. in1993 and his D.Sc. in 1998 in the field of neuroendocrinology from Charles University. In 2006 he was awarded a lifetime professorship in Internal Medicine at Charles University. Dr. Pacak is an internationally recognized expert in the diagnosis and treatment of neuroendocrine tumors, especially pheochromocytoma and paraganglioma. Dr. Pacak also established the International Symposia on Pheochromocytoma. He also helped co-found a new Asian Alliance for the Study of Neuroendocrine Tumors in 2010. He is a recipient of numerous awards including the Peter Heimann Memorial Award at Yale University, International Association of Endocrine Surgeons; NIH Director's Mentor Award, Award for Cure from Pheo & Para Alliance, NICHD Director's Award of Merit, Pincus Taft Memorial Lecture the Highest Award from Endocrine Society of Australia, and Jessenius Gold Medal from Slovak Academy of Sciences. Dr. Pacak is the author of more than 305 scientific peer-reviewed articles, 98 book chapters, and 5 books.

Natalie Mora

Endocrinology Fellow
National Institutes of Health
Bethesda, Maryland

Dr.Mora is from San Antonio, Texas and is a graduate of SUNY Upstate Medical University. She trained in internal medicine at the University of Virginia and is a current endocrinology fellow at NIH. Dr.Mora's main interest is diabetes, nutrition, and health disparities.