Neuroendocrine tumor of the pancreas and bilateral adrenal pheochromocytomas. A rare manifestation of von Hippel-Lindau disease in childhood.
BACKGROUND
A 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.


METHODS
von 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.


CONCLUSIONS
Open 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.
Langrehr
Jan M
JM
Department for General, Visceral and Transplantation Surgery, Charité, Campus Virchow Clinic, 13353 Berlin, Germany. jan.langrehr@charite.de
Bahra
Marcus
M
Kristiansen
Glen
G
Neumann
Hartmut P H
HP
Neumann
Luitgard M
LM
Plöckinger
Ursula
U
Lopez-Hänninen
Enrique
E
D000310:Adrenal Gland Neoplasms / Q000175:diagnosis / Q000601:surgery; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D018358:Neuroendocrine Tumors / Q000175:diagnosis / Q000601:surgery; D010190:Pancreatic Neoplasms / Q000175:diagnosis / Q000601:surgery; D010673:Pheochromocytoma / Q000175:diagnosis / Q000601:surgery; D006623:von Hippel-Lindau Disease / Q000175:diagnosis* / Q000601:surgery*