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ea0022p404 | Endocrine tumours & neoplasia (<emphasis role="italic">Generously supported by Novartis</emphasis>) | ECE2010

Ectopic insulin secretion from distant metastasis or ectopic metaplasia after total pancreatectomy for ACTH-secreting endocrine pancreatic tumor revealing a Von Hippel Lindau disease

Vantyghem Marie-Christine , Wild Damian , Neraud Barbara , Nghi-Beron Amandine , Raverdy Violeta , Steinling Marc , Wemeau Jean-Louis , Pattou Francois

Von Hippel Lindau disease (VHL) induces tumors of kidneys, central nervous system, pancreas and paragangliomas We report one case where ectopic Cushing syndrome revealed VHL. A 19-year old was referred for hirsutism and spaniomenorrhea revealing an ACTH-dependent Cushing syndrome. Mineralocorticoids, calcium metabolism, chromogranine A, calcitonine, pancreatic hormones and urinary 5-HIA were normal. Blood nor- and metanephrine were 5.12 μg/l (n<5) and 0.92 &#95...

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Endocrine Abstracts

Ectopic insulin secretion from distant metastasis or ectopic metaplasia after total pancreatectomy for ACTH-secreting endocrine pancreatic tumor revealing a Von Hippel Lindau disease

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