title: Von Hippel Lindau disease with metastatic pancreatic neuroendocrine tumor causing ectopic Cushing's syndrome.
abstract: We present a 39-year-old woman who was previously diagnosed with Von Hippel Lindau Disease (VHLD). She had surgery and radiotherapy for cranial hemangioblastoma (HA) 11 years ago and had unilateral adrenalectomy for pheochromocytoma in another hospital 6 month prior to her admission to our center. Moon face, buffalo hump, central obesity, progressive weight gain and menstrual irregularities persisted after adrenalectomy. Her laboratory results were consistent with ectopic Cushing's syndrome (ECS). A pancreatic solid mass with a nodule on the left lung were revealed upon computed tomography. In addition, Gallium-68 Somatostatin Receptor PET confirmed the pancreatic involvement and demonstrated additional lesions on the left lung and in the aortocaval lymphatic system on the right side, suggesting metastatic pancreatic neuroendocrine tumor (PNET). Peptide receptor radionuclide therapy (PRRT) with [177Lutetium-DOTA0,Tyr3] octreotate was performed on the patient, with no side effects observed. She was discharged from the hospital 10 days after the first cycle.
authors.affiliation: Department of Internal Medicine, Istanbul University, Istanbul, Turkey.
authors.forename: Esra
authors.initials: E
authors.lastname: Hatipoglu
authors.forename: Hasan
authors.initials: H
authors.lastname: Kepicoglu
authors.forename: Elif
authors.initials: E
authors.lastname: Rusen
authors.forename: Levent
authors.initials: L
authors.lastname: Kabasakal
authors.forename: Sadi
authors.initials: S
authors.lastname: Gundogdu
authors.forename: Pinar
authors.initials: P
authors.lastname: Kadioglu
mesh_terms: D000328:Adult; D018278:Carcinoma, Neuroendocrine / Q000150:complications* / Q000532:radiotherapy / Q000556:secondary; D003480:Cushing Syndrome / Q000209:etiology* / Q000532:radiotherapy; D005260:Female; D006801:Humans; D008187:Lutetium / Q000627:therapeutic use; D010190:Pancreatic Neoplasms / Q000150:complications* / Q000473:pathology / Q000532:radiotherapy; D010456:Peptides, Cyclic / Q000627:therapeutic use; D011868:Radioisotopes / Q000627:therapeutic use; D006623:von Hippel-Lindau Disease / Q000150:complications*
chemical_list: D010456:Peptides, Cyclic; D011868:Radioisotopes; C470036:octreotate, Tyr(3)-; D008187:Lutetium