Endocrine Abstracts

Introduction: Ectopic adrenocorticotropic hormone (ACTH) secretion is a less common cause of Cushing syndrome(CS) and is seen in 5 to 10% of patients with endogenous hypercortisolism. The most common types are bronchial carcinoids and small cell lung carcinoma. However, in approximately 10–20% of the cases, overt tumor cannot be found. Here, we described two patients with catastrophic hypercorticolism associated with ectopic CS and who were treated with bilateral adrenalectomy.

Case 1: A 47-year-old female patient had a history of a total abdominal hysterectomy and bilateral salpingo-oopherectomy operation due to clear cell ovary carcinoma 7 months ago. She was treated with chemotherapy. After 3^rd dose chemotherapy, she had been hospitalized and treated due to sepsis associated with urinary tract infection. During the follow-up period in oncology, hypokalemia was detected and she was referred to our department due to severe muscle weakness in lower extremities and hypokalemia (serum K: 2.2 mEq/l). 24-h urinary free cortisol, ACTH measurement, dexamethasone suppression test (DST) demonstrated an ACTH dependent hypercortisolism. The inferior petrosal sinus sampling was indicative for an ectopic ACTH secretion. CT-scans of the thorax and abdomen, FDG-PET/CT scan and Ga68 DOTATATE PET/CT scans, were unable to demonstrate malignancy. Previous ovarian pathology was screened for ACTH secretion and evaluated as negative. Antifungal and metyrapone were started and then bilateral adrenalectomy was performed.

Case 2: A 71-year-old male patient who had a medical history of metastatic prostate carcinoma was referred to our department due to severe hypopotasemia, muscle weakness, refractory hypertension, peripheral edema. He had a history of second operation 3 months ago due to tumoral enlargement at the base of the urinary bladder and it was evaluated as infiltration of the prostate carcinoma. 24-h urinary free cortisol, ACTH measurement, DST demonstrated an ACTH dependent hypercortisolism. CT-scans of the thorax and abdomen, FDG-PET/CT scan and Ga68 DOTATATE PET/CT scans, were negative for malignancy. Medical therapy was started. But refractory hypertension, parenteral potasium infusion need were not suspended. Bilateral adrenalectomy was perfomed. He died due to pulmonary embolism 2 months after adrenalectomy.

Conclusion: Common treatmant options of Cushing syndrome consist of tumor management, somatostatin analogs, steroidogenesis inhibitors. Bilateral adrenalectomy is a highly effective treatment for patients with severe hypercortisolism if rapid control of hypercortisolism is desired. Mortality is high especially in patients with severe co-morbidities and mostly it depends on the prognosis of the underlying malignant tumor.