Ectopic ACTH Syndrome Due to MEN-I Associated Metastatic Neuroendocrine Pancreatic Gastrinoma in a 22 Year Old Female without Hyperparathyroidism: Diagnostic and Therapeutic Challenges

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Introduction: Ectopic ACTH production due to malignant tumors is a rare cause of Cushing's syndrome (CS). Diagnosis and treatment of such patients can be challenging. Case: 22 year old female with recent onset fatigue, weight gain, polyuria, polydipsia, presented with melena and hypotension. She had cushingoid facies, acne, central obesity, muscle weakness and purple striae. Labs showed: Cortisol 56 µg/dl (5-15), 24 hour UFC >1000 µg/24hr (5-50), Cortisol after 1mg Dexamethasone suppression 54 µg/dl (Learning points: When evaluating patients with suspected ectopic ACTH production, independent review of radiologic imaging should be done to avoid being misled by prior reports. EUS is a superior method for identifying PNET (1). Biochemical confirmation with IPSS helps exclude Cushing's Disease in the setting of a pituitary adenoma. Medical therapy to control hypercortisolism and anticoagulation are necessary to lower morbidity and mortality associated with severe CS (2). Finally, screening for MEN-1 in these patients is recommended, even in the absence of hyperparathyroidism (3).


Endocrinology, Diabetes, and Metabolism | Medical Sciences | Medicine and Health Sciences


Department of Medicine Faculty

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