Background: Ectopic ACTH syndrome (EAS) is exceedingly rare in children and scarcely reported. Pancreatic Neuroendocrine tumours (NET) can rarely lead to secretion of ectopic adrenocorticotropic hormone (ACTH).
Case Characteristics: A14-year-old adolescent boy presented with isolated hyperpigmentation and intermittent abdominal pain and underwent evaluation for primary adrenal insufficiency, but turned out to have subclinical Cushings instead. An incidental pancreatic mass discovered on routine ultrasonogram revealed the source of ACTH. He underwent successful excision with resolution of hypercortisolism. The histopathology revealed a Pancreatic Neuroendocrine Tumor (NET)and immunohistochemistry (IHC) was positive for ACTH stain.
Message: The extra-ordinary features of this case were the absence of clinical Cushing's in the presence of severe biochemical hypercortisolism, the equivocal ACTH levels not correlating with the degree of hypercortisolism or hyperpigmentation, the incidental discovery of pancreatic mass laying rest to the dilemma over source of ACTH- Pituitary versus Ectopic, and the rarity of Pancreatic NET causing EAS in children.
19 - 21 Sep 2019
European Society for Paediatric Endocrinology