A Rare Case of Cushing Syndrome Caused by an Ectopic ACTH-Secreting Pancreatic Neuroendocrine Tumor in a Patient with Von Hipple-Lindau Disease

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Introduction: Von Hipple-Lindau (VHL) disease is a rare autosomal dominant disorder, that causes the susceptibility to various tumors, both benign and malignant. The most frequent tumors are central nervous system haemangioblastomas, especially cerebellum and retinal haemangioblastomas, renal cell carcinoma /renal cysts and pheocromocytoma.

Aim(s): We report a case of a 36-year-old woman diagnosed with VHL disease characterized by the uncommon association with ACTH-dpendent Cushing's syndrome.

Materials and methods: Phisycal examination, laboratory findings and traditional and radioreceptorial imaging were performed.

Conference: 9th Annual ENETSConcerence (2012)

Presenting Author:

Authors: Sburlati L, Ridolfi C, Gavazzi F, Colombo P, Brunetta E,

Keywords: von Hipple-Lindau disease, Cushing syndrome ,