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Neuroendocrine tumor of the pancreas and bilateral adrenal pheochromocytomas. A rare manifestation of von Hippel-Lindau disease in childhood |
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| Authors: | Langrehr Jan M Bahra Marcus Kristiansen Glen Neumann Hartmut P H Neumann Luitgard M Plöckinger Ursula Lopez-Hänninen Enrique |
| Affiliation: | a Department for General, Visceral and Transplantation Surgery, Charité, Campus Virchow Clinic, 13353 Berlin, Germany b Institute of Pathology, Charité, 10117 Berlin, Germany c Institute of Human Genetics, Charité, 13353 Berlin, Germany d Department for Gastroenterology and Hepatology, Charité, 13353 Berlin, Germany e Institute for Radiology, Charité, 13353 Berlin, Germany f Department of Nephrology and General Medicine, Albert-Ludwigs University, 79095 Freiburg, Germany |
| Abstract: | BackgroundA 12-year-old girl presenting with intermittent epigastric pains and diarrhea was referred to our clinic. Diagnostic workup revealed nonfunctional bilateral adrenal pheochromocytomas as well as a neuroendocrine tumor of the pancreatic head. This is the first report on the combination of a neuroendocrine pancreatic tumor with adrenal pheochromocytoma in a pediatric patient with von Hippel-Lindau (VHL) disease.Methodsvon Hippel-Lindau disease was confirmed by molecular genetic analysis of peripheral blood lymphocytes, which revealed the mutation VHL c. 695 G > A. The family history showed also VHL disease in the mother who carried the same mutation.Results and ConclusionOpen laparotomy, organ-sparing enucleation of pheochromocytoma, and pylorus-preserving resection of the pancreatic head tumor were successfully performed. After an uneventful postoperative course, the child fully recovered. She was free of further manifestations of VHL disease 30 months after surgery. |
| Keywords: | von Hippel-Lindau disease Pancreatic head resection Adrenal surgery |
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