Endocrine Abstracts

Background: Hormonal disturbances may be encountered in the context of paraneoplastic syndromes. Neuroendocrine tumors (NETs) represent a rare group of cancers, derived from the group of chromaffin cells, thus their paraneoplastic potentials remain relatively unexplored.

Case presentation: Middle-aged female presented to the local A&E with psychosis, hyperglycemia, mild hypokalemia. Background includes resected ovarian cancer and chemotherapy before 30 years, as well as a recent diagnosis of metastatic well-differentiated pancreatic NET, originally treated with distal pancreatectomy and splenectomy, under surveillance monitoring. Blood tests indicated ACTH 445ng/L; cortisol 5,500 nmol/l, suppressed remaining pituitary hormones and slightly elevated amylase. Clinical evaluation identified only mild epigastric tenderness. MRI of the pituitary showed no abnormality. A CT colon showed a 3 cm solid mass in the body of pancreas. The Gallium-68 PET scan showed avid abdominal and left supraclavicular lymph node metastases and small volume liver metastases. The FDG PET scan could not identify avid malignancy. These results were compatible with ectopic ACTH syndrome of rapid onset, hence no cushingoid signs were clinically evident. Treatment involved etomidate infusion to regulate levels of cortisol, bilateral adrenalectomy and replacement with exogenous hydrocortisone and fludrocortisone. Subsequently, 5FCarboStrep chemotherapy was initiated for tumor control. Twenty months later the lady exhibits good partial response of her liver metastases and has no evidence of disease progression in the chest.

Conclusion: Pancreatic NETs represent a source of possible ectopic ACTH production, which may result to the full clinical picture of Cushing’s syndrome in some cases. Treatment consists mainly of bilateral adrenalectomy and hormone replacement.