High-Grade Bacteremia in a Patient with Suspected Adrenocortical Carcinoma

Introduction:
Adrenocortical carcinoma (ACC) is a rare malignancy with poor prognosis, presenting more commonly in women and on the left side. Approximately 50-80% of functioning ACCs present with signs/symptoms of cortisol excess.

Case Description:
A 63-year-old woman was seen in clinic for severe osteoporosis. Her history included L5 compression fracture, multiple rib fractures, BMI 38, recurrent deep vein thrombosis on rivaroxaban, chronic pain and proximal muscle weakness limiting mobility, prediabetes, and hypertension. The patient had moon facies and a dorsocervical fat pad. Dexamethasone suppression test showed cortisol level of 29.6. A 24-hour urine cortisol was 1974. ACTH was <5. CT demonstrated a 5.9 x 5.3 x 4.2 cm left adrenal mass (20 Hounsfield units) with irregular soft tissue extending to the renal fascia. The patient was referred to surgical oncology and began ketoconazole. Osilodrostat, mitotane, and metyrapone were denied by insurance. Further workup showed normal plasma metanephrines, 17-hydroxyprogesterone, and androstenedione. DHEA-S was low at 5 and 11-deoxycortisol was elevated to 162.

Prior to her surgery clinic appointment, the patient presented to the hospital for generalized pain and fever. She was found to have high-grade Staphylococcus aureus bacteremia. Ketoconazole dosing was increased. High-grade bacteremia continued on repeat cultures. Lumbosacral MRI showed L4-S1 discitis/osteomyelitis and presacral abscess unamenable to drainage. PET/CT demonstrated FDG avid left adrenal mass highly suspicious for ACC with contiguous intensity likely representing local invasion but no distant metastatic disease. Five days later, the patient developed a pressor requirement. Morning cortisol was 5.9. Repeat 24-hour urine cortisol was 9.5. Stress dose steroids were started, and ketoconazole was decreased. Pressor requirements escalated despite these interventions. Ultimately, the patient opted to forgo further life-saving treatment.  She passed away 2.5 months after initial diagnosis of hypercortisolism. Unfortunately, the pathologic diagnosis of ACC was unable to be established.

Discussion:
This case signifies the life-threatening complications of Cushing syndrome secondary to suspected ACC. While the patient’s severe infection precluded pathologic diagnosis of ACC, the size of the adrenal mass and biochemical results, including elevated 24-hour urine cortisol and elevated 11-deoxycortisol, raised suspicion for ACC. Localized ACC would typically be treated with surgical resection. Adrenal insufficiency secondary to inhibition of steroidogenesis in this patient may have limited her ability to fight infection, hindering her opportunity to proceed with a surgical procedure.