期刊
REVISTA ESPANOLA DE MEDICINA NUCLEAR
卷 27, 期 1, 页码 29-33出版社
ELSEVIER SCIENCE INC
DOI: 10.1157/13114367
关键词
von Hippel-Lindau disease; ectopic ACTH syndrome; neuroendocrine tumours; In-111-DTPA-pentetreotide scintigraphy
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. (111)-in-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.
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