Endocrine Abstracts

The ectopic secretion of corticotropin from no pituitary tumours causes ~10–20% of cases of Cushing’s syndrome. Enteropancreatic neuroendocrine carcinoma represents a rare cause of ectopic ACTH syndrome.

Case: A 51 years-old woman without past medical history was admitted by gain weight, facial oedema 4 months previous. Physical examination: BP 167/124, cardiac rate 49 bpm, facial oedema, troncular obesity. No others abnormalities. Laboratory test. Glucose 148 mg/dl. Kaliemia 2.8 mEq/l. Cortisol 102 μg/dl, FCU 4000 μg/24 h, chromogranin A 526 ng/ml, indolacétic acid 6.8 mg/24 h. Nocturnal cortisol 132 μg/dl, ACTH: 427 pg/ml. Nuggent test. Cortisol 129 μg/dl. Suppression with 8 mg of dexamethasone: cortisol basal 141 μg/dl, post 146 μg/dl, ACTH basal 577 pg/ml post 586 pg/ml. CT scan revealed a pancreatic mass of 30×20 mm and enlargement of adrenal glands. PET scan displayed abnormal accumulations of fluorodeoxyglucose in pancreatic area, distribution at multiple sites in the liver and enlargement of both adrenal glands. Surgical procedure was carry out, and large pancreatic tumour with multiples hepatic metastasis were observed. Pancreatectomy and splenectomy was carry out. Hystopatologycal study showed pancreatic neuroendocrine tumour, positive to cromogranin, sinaptofisine and CKAE1-AE3, Ki 67 up 50%, with lymph nodes and liver metastasis of neuroendocrine tumour. Surgical treatment of adrenal gland was discarded. Treatment with ketoconazole and metyrapone was initiated without improvement and mifepristone was added, lowering cortisol levels, however, outcome was unfavourable and patient died 3 months after diagnosis by respiratory distress and complications of Cushing’s syndrome.

Discussion: Patients with pancreatic neuroendocrine carcinoma represent a rare cause of ectopic ACTH syndrome. A typical Cushingoid appearance is less frequent in ectopic ACTH syndrome. Treatment of ectopic ACTH syndrome is excision of the primary tumour. However, curative surgery is successful in only 30–47%. If the tumour cannot be resected, bilateral adrenalectomy offers effective permanent treatment. Medical cortisol inhibitors include ketoconazole, metyrapone.