Abstract
We present a 36-year-old woman diagnosed with von Hippel-Lindau disease. A haemangioblastoma of the central nervous system was successfully excised ten years ago and a left nephrectomy had been performed because of clear cell carcinoma. For the last six months, the patient has suffered from amenorrhoea, asthenia, muscular debility and capillary fragility. Physical examination showed hirsutism and bruising. Laboratory findings supported ectopic Cushing's syndrome. A solid pancreatic mass was identified on computed tomography. 111In-DPTA-pentetreotide scintigraphy demonstrated marked uptake at this location, suggesting a neuroendocrine tumour. Duodenopancreatectomy was performed. After surgery, the cortisol levels returned to normal with complete resolution of the syndrome. This is the first case of Cushing's syndrome caused by an ectopic ACTH-secreting neuroendocrine tumour of the pancreas associated with von Hippel-Lindau disease.
Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
