2019
DOI: 10.1210/js.2019-mon-318
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MON-318 Selective ACTH Sampling in Localizing Source of ACTH in Von Hippel Lindau Disease with Pancreatic Neuroendocrine Tumour and Renal Cell Carcinoma

Abstract: Background: Cushing’s syndrome (CS) in a patient with VHL has been attributed to a number of possible causes; pancreatic NET and renal cell carcinoma. The precise location of ectopic ACTH aid enormously in the management of VHL. Clinical case: A 31-year-old woman with Type 2 diabetes and family history of VHL presented with florid features of CS in her second trimester of pregnancy. A diagnosis of ACTH dependent CS was made based on elevated 24h urinary cortisol (1122 and 2448… Show more

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