Category: Adrenal Disorders

Type: e-Poster

Monitor: 21

21 - CO-SECRETING ADRENAL INCIDENTALOMA: A RARE CASE OF PHEOCHROMOCYTOMA WITH ACTH-INDEPENDENT CUSHING'S SYNDROME

Thursday, Apr 25
12:00 PM – 12:30 PM

Objective : An adrenal incidentaloma is a mass, greater than 1 cm, found on imaging studies during evaluation unrelated to adrenal disease. Of these masses, 10-15% are reported to be functioning masses. Co-secreting tumors are a rare entity of adrenal masses. We present a rare case of adrenal incidentaloma with coexistence of both pheochromocytoma and adrenocorticotropic hormone (ACTH) independent Cushing’s syndrome.


Methods : N/A


Results : N/A


Discussion : Our patient is a 63-year-old female who was initially admitted to the medical intensive care unit for management of sepsis secondary to urinary tract infection. A CT scan abdomen and pelvis showed an incidental finding of a 6.5 cm left adrenal mass. Subsequently, MRI abdomen with and without contrast revealed a 5.3 x 6.3 x 5.5 cm circumscribed, cystic, multiseptated, T2-hyperintense mass arising from the left adrenal gland. This was suspicious for pheochromocytoma. Serum metanephrines, urine metanephrines, and urine catecholamine studies returned elevated, consistent with a diagnosis of pheochromocytoma. Despite absence of clinical stigmata, screening test for Cushings came back positive with abnormal 1 mg dexamethasone suppression test. Cushing’s syndrome was confirmed with elevated two midnight salivary cortisol and 24 hour free urine cortisol level. ACTH level was <5 pg/mL consistent with ACTH independent Cushing’s syndrome. The remaining hormonal studies returned negative. She was started on an alpha-blocker followed by a beta blocker to prepare her for surgery. She underwent successful robotic assisted laparoscopic left adrenalectomy. Grossly, the tumor had cystic spaces and necrosis. The final pathology report is pending. She received perioperative hydrocortisone and was discharged home on oral maintenance steroids.


Conclusion :

The discovery of adrenal incidentalomas is rising with the increased use of high-resolution imaging. Recent studies report that pheochromocytomas account for approximately 3% of these masses. Co-secreting pheochromocytomas represent an even smaller percentage of cases with only a few published reports describing ectopic ACTH secretion from adrenal pheochromocytomas leading to Cushing’s syndrome. Our patient had a low ACTH level suggesting ACTH-independent cortisol secretion from the mass, which has been rarely reported. Although the mechanism is not clear, prior literature suggests the paracrine effect of catecholamines may stimulate steroidogenesis. Our case illustrates that it is prudent to consider testing for co-secreting adrenal masses even in the absence of typical clinical signs and symptoms.

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Jennifer Do

Resident Physician
Advocate Lutheran General Hospital

Currently PGY2 in Internal Medicine Residency at Advocate Lutheran General Hospital in Park Ridge, IL.

Meghana Doniparthi

Resident Physician
Advocate Lutheran General Hospital

Currently a PGY3 in Internal Medicine Residency at Advocate Lutheran General Hospital in Park Ridge, IL.

Vinita Singh

Endocrinologist
Advocate Lutheran General Hospital

Attending Physician, Department of Endocrinology at Advocate Lutheran General Hospital in Park Ridge, IL.

Jennifer Do

Resident Physician
Advocate Lutheran General Hospital

Currently PGY2 in Internal Medicine Residency at Advocate Lutheran General Hospital in Park Ridge, IL.