Endocrine Abstracts

Introduction: Paraneoplastic Cushing syndrome is a rare form of endogenous ACTH (adrenocorticotropic hormone) dependent hypercortisolism. In some cases no overt ACTH secretion tumor can be found, which is why steroidogenesis inhibitors, and bilateral adrenalectomy remain the main therapeutic options.

Material and method: This is a case report investigated in several centers by performing biochemical, hormonal and imagery tests.

Case report: A 52-year female, without medical personal history was admitted for marked asthenia, muscle weakness, polyuria and polydipsia. Clinical examination revealed central obesity (BMI of 30 kg/m²), enlarged supra-clavicle fat pads, blood pressure of 150/80 mmHg, hyperpigmented skin, legs edema. Hormonal profile showed high levels of morning plasma cortisol (634 mg/dl, normal 5–25 mg/dL), with loss of circadian rhythm, very high CLU (free urinary cortisol), of 4728 mg/24 h (normal 50–190 mg/24 h), high ACTH level (619.9 ng/l, normal 7.2–63.3 ng/l), non-suppression at low dose DXM (dexamatasone) and 2 day of 8 mg DXM, high testosterone level (2.16 ng/ml, normal 0.2–0.75 ng/ml). Biochemical parameters indicated hyperglycemia and high HgA1c (glycated hemoglobin of 10.2%, normal 4.8–5.6%), hypercholesterolemia, low potassium (1.84 mmol/l, normal 3.5–5.1 mmol/l), and hypocalcemia. No morphological changes were found on pituitary MRI (magnetic resonance imaging) and pulmonary CT (computed tomography) scan. Abdominal CT scan showed bilateral hyperplasia. PET-CT scan (positron emission tomography) revealed an uncertainty mesenteric mass measuring less than 1 cm. Diagnosis of paraneoplastic Cushing syndrome was established. All neuroendocrine tumor markers were in the normal reference range. Potassium supplements, insulin and methyrapone therapy were started, followed by bilateral adrenalectomy. A month later obvious clinical and biochemical improvement was achieved under therapy with glucocorticoid and mineralocorticoid therapy. Because ACTH level is still high (of 1329 pg/ml) a careful follow-up is required. The primary site of neoplasia is still under investigation.

Conclusion: Diagnostic and treatment of paraneoplastic Cushing syndrome are essential for limiting the metabolic consequences and improvement quality of life. Long life follow-up is needed especially for variants without obvious tumor.