Exocrine pancreatic insufficiency and pancreatic fibrosis due to duodenal somatostatinoma in a patient with neurofibromatosis.
A case of duodenal somatostatinoma is described in a patient with Von Recklinghausen neurofibromatosis. The patient presented with exocrine pancreatic insufficiency, probably due to distal obstruction of the pancreatic duct by the tumor. Preoperative evaluation with calcium-pentagastrin and tolbutamide stimulation tests were nondiagnostic. At laparotomy, local excision of the tumor was performed. Pathological findings were compatible with duodenal somatostatinoma, causing pancreatic fibrosis. Somatostatin extracted from the tumor coeluted with the somatostatin-14 standard on high performance liquid chromatography (HPLC).
Hagen
E C
EC
Department of General Internal Medicine, University of Leiden, The Netherlands.
Houben
G M
GM
Nikkels
R E
RE
Jansen
J B
JB
Lamers
C B
CB
Gooszen
H G
HG
van Duinen
S G
SG
Meinders
A E
AE
D000328:Adult; D001706:Biopsy; D002851:Chromatography, High Pressure Liquid; D004379:Duodenal Neoplasms / Q000737:chemistry / Q000150:complications* / Q000648:ultrastructure; D010188:Exocrine Pancreatic Insufficiency / Q000209:etiology*; D005260:Female; D005355:Fibrosis; D005755:Gastrins / Q000097:blood; D005934:Glucagon / Q000097:blood; D006801:Humans; D008854:Microscopy, Electron; D009456:Neurofibromatosis 1 / Q000737:chemistry / Q000150:complications* / Q000648:ultrastructure; D010179:Pancreas / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D010191:Pancreatic Polypeptide / Q000097:blood; D011863:Radioimmunoassay; D013004:Somatostatin / Q000032:analysis / Q000097:blood; D013005:Somatostatinoma / Q000737:chemistry / Q000150:complications* / Q000648:ultrastructure; D014660:Vasoactive Intestinal Peptide / Q000097:blood
D005755:Gastrins; D014660:Vasoactive Intestinal Peptide; D013004:Somatostatin; D010191:Pancreatic Polypeptide; D005934:Glucagon