[Ectopic Cushing's syndrome caused by a functioning pancreatic neuroendocrine tumour in a patient with von Hippel-Lindau disease].
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.
Benítez Velazco
A
A
Unidad de Medicina Nuclear, Hospital Universitario Reina Sofía, Córdoba, Spain. abvelazco@yahoo.es
Pacheco Capote
C
C
Latre Romero
J M
JM
D000182:ACTH Syndrome, Ectopic / Q000097:blood / Q000188:drug therapy / Q000209:etiology* / Q000601:surgery; D000328:Adult; D003131:Combined Modality Therapy; D003480:Cushing Syndrome / Q000188:drug therapy / Q000209:etiology* / Q000601:surgery; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D007205:Indium Radioisotopes; D007654:Ketoconazole / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D009378:Neoplasms, Multiple Primary / Q000235:genetics; D018358:Neuroendocrine Tumors / Q000150:complications* / Q000000981:diagnostic imaging / Q000378:metabolism / Q000601:surgery; D010190:Pancreatic Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging / Q000378:metabolism / Q000601:surgery; D016577:Pancreaticoduodenectomy; D004369:Pentetic Acid / Q000031:analogs & derivatives; D011877:Radionuclide Imaging; D019275:Radiopharmaceuticals; D014057:Tomography, X-Ray Computed; D051598:Whole Body Imaging; D006623:von Hippel-Lindau Disease / Q000150:complications* / Q000175:diagnosis
C452379:DTPA pentetreotide; D007205:Indium Radioisotopes; D019275:Radiopharmaceuticals; D004369:Pentetic Acid; D007654:Ketoconazole; D006854:Hydrocortisone