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

Abstract:

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. RESULTS AND CONCLUSION: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.

journal_name

J Pediatr Surg

authors

Langrehr JM,Bahra M,Kristiansen G,Neumann HP,Neumann LM,Plöckinger U,Lopez-Hänninen E

doi

10.1016/j.jpedsurg.2007.02.029

subject

Has Abstract

pub_date

2007-07-01 00:00:00

pages

1291-4

issue

7

eissn

0022-3468

issn

1531-5037

pii

S0022-3468(07)00139-X

journal_volume

42

pub_type

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