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Endocrine Abstracts (2023) 90 EP1081 | DOI: 10.1530/endoabs.90.EP1081

Araba University Hospital, Gasteiz, Spain


Introduction: The most frequent causes of ACTH-dependent Cushing’s syndrome are of pituitary origin and, less frequently, due to ectopic secretion of bronchial carcinoid, small cell lung cancer, and neuroendocrine tumors; it is rarely caused by an ACTH-producing pheochromocytoma.

Methodology: We present a case of a 44-year-old female with a history of poorly controlled type 2 diabetes, hypertension, and long-standing obesity with no genetic predisposition or typical Cushingoid features who was initially admitted due to edema in both legs and hyperglycemic decompensation. On arrival, analysis showed she presented hypokalemia and hyperglycemia, in addition to clinical hypertension that was difficult to control and altered behavior without history of previous psychiatric pathology. After a full-body CT scan incidentally revealed suprarenal damage, we began a radiological study. Adrenal CT scans showed a 4 × 3.5 cm right adrenal nodule and MR imaging of the pituitary did not reveal an adenoma.

Results: After starting pharmacological treatment with ketoconazole, the subject continued to have severe symptoms of hypercortisolism. Therefore, etomidate was administered to inhibit corticosteroid synthesis, alpha blockade was used in the preoperative preparation, and right adrenalectomy surgery was performed. The anatomic pathology results were intermediate risk, moderately differentiated pheochromocytoma (according to GAPP risk stratification). The patient subsequently evolved favorably, with the high levels of cortisol, ACTH, and metanephrine initially found in her urine decreasing to normal levels.

Biochemical tests
Plasma levels24-h urinary measurement
Basal cortisol 114.6 μg/dl (4.0 - 20.0)Cortisol excretion 25199 μg/dl (0 – 134)
ACTH 215 μg/dl (7-62)Normetanephrine 1145 μg/24h (0 – 444)
Metanephrine 756 μg/24h (0 – 341)
Epinephrine 32 µg/24h (0-18)
Norepinephrine 262 µg/24h (0-7)

Conclusions: Although this is an extraordinary case, a full examination must be completed anytime a suprarenal nodule is found, as it may be the result of a ACTH-secreting pheochromocytoma and quick diagnosis and treatment are crucial.

Volume 90

25th European Congress of Endocrinology

Istanbul, Turkey
13 May 2023 - 16 May 2023

European Society of Endocrinology 

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