Background:
Hypertension and diabetes are common diseases in the adult population. Hypertension is found in about 40% of patients with newly diagnosed type 2 diabetes mellitus. Five to 10% of hypertension cases are secondary to an endocrine hypersecretory disorder including pheochromocytoma, and Cushing's syndrome.
Results:
A 51 year‐old African american female presented with a draining abscess on her right buttock. She gave a history of hypertension and poorly controlled diabetes mellitus. Additionally she reported oligomenorrhea, infertility and hirsutism for many years. Physical examination findings included a BP of 148/93 mmHg, facial hirsutism, male pattern baldness, well‐developed muscles, breast atrophy, and central obesity. Her skin was of normal thickness and was without striae.
Initial laboratory studies showed a hypokalemic metabolic alkalosis and a hemoglobin A1c of 7.2%. Further evaluation revealed normal renin and aldosterone, but an elevated random cortisol level of 34.1 mcg/dL (normal < 25). A CT scan of the abdomen ordered for further evaluation of her gluteal abscess revealed a soft tissue mass in the right posterior abdomen. Endocrine testing showed normal 24 hour urinary catecholamines, but elevated 24 hour urinary free cortisol (1316 mcg/dL ‐ normal < 100) and 24 hour urinary hydroxycorticosteroid (62.5 mcg/mg creatinine ‐ normal < 8). Her serum ACTH was suppressed at < 5 pg/dL. She had a normal serum DHEA (dehydroepiandrosterone) of 218 ng/dL, but her serum total testosterone was elevated at 246 mcg/dL (normal female < 60). An MRI of the abdomen revealed the soft tissue mass in the right upper posterior abdomen to be an 8 cm heterogeneous adrenal mass.
She was diagnosed with adrenocortical carcinoma and underwent a right adrenalectomy. The diagnosis was confirmed histologically with immunoperoxidase stains. Postoperatively her hypokalemic alkalosis resolved, her blood pressure and diabetes were better controlled, and a repeat 24‐hour urinary free cortisol was 91 mcg/dL. She was discharged on mitotane chemotherapy and replacement prednisone.
Conclusion:
Adrenocortical carcinoma is a rare tumor with approximately two new cases per million population occurring each year. There is a bimodal age distribution with the first peak occurring before age 5 and the second in the forth to fifth decade. Females account for 65 to 90% of the cases reported. Cushing's syndrome is seen in 30 to 40 percent of cases, and Cushing's with virilization is seen in 10 to 30%.
This case underscores the importance of evaluation for secondary causes of poorly controlled diabetes and hypertension especially in the setting of hirsutism, hypokalemia, and metabolic alkalosis.
Author Disclosure Block:
N.E. Sanchez‐Crespo, None; I. Orija, None; R. Osburne, None.