[Zollinger-Ellison syndrome]

Chirurg. 2005 Mar;76(3):217-26. doi: 10.1007/s00104-004-0995-5.
[Article in German]

Abstract

The preoperative localization of gastrinomas often fails despite all modern imaging methods. Therefore, after biochemical confirmation of the diagnosis and exclusion of diffuse metastases, a meticulous surgical exploration including intraoperative ultrasound (IOUS) and duodenal exploration after duodenotomy should be performed. The experienced surgeon will be able to identify more than 90% of the primary tumors. Depending on the localization, excision of the tumor in the duodenal wall or enucleation from the pancreatic head should be performed. If the tumor is localized in the tail of the pancreas, distal pancreatectomy is the treatment of choice. Complete resection of the tumor is the only curative approach for the patients. For MEN-1 gastrinomas a spleen-preserving distal pancreatectomy with enucleation of tumors of the pancreatic head and duodenotomy with excision of duodenal gastrinomas should be performed. If the source of gastrin secretion can be regionalized to the pancreatic head by a preoperative SASI angiography, a pylorus-preserving partial pancreaticoduodenectomy might be the treatment of choice.

Publication types

  • English Abstract

MeSH terms

  • Adult
  • Aged
  • Chromosomes, Human, Pair 11
  • Duodenal Neoplasms / diagnosis
  • Duodenal Neoplasms / genetics
  • Duodenal Neoplasms / mortality
  • Duodenal Neoplasms / surgery*
  • Endosonography
  • Female
  • Follow-Up Studies
  • Gastrinoma / diagnosis
  • Gastrinoma / genetics
  • Gastrinoma / pathology
  • Gastrinoma / surgery*
  • Gastrins / blood
  • Genes, Dominant
  • Germ-Line Mutation
  • Humans
  • Male
  • Middle Aged
  • Multiple Endocrine Neoplasia Type 1 / diagnosis
  • Multiple Endocrine Neoplasia Type 1 / genetics
  • Multiple Endocrine Neoplasia Type 1 / mortality
  • Multiple Endocrine Neoplasia Type 1 / surgery*
  • Pancreatic Neoplasms / diagnosis
  • Pancreatic Neoplasms / genetics
  • Pancreatic Neoplasms / mortality
  • Pancreatic Neoplasms / surgery*
  • Pancreaticoduodenectomy / methods
  • Reoperation / methods
  • Secretin
  • Stomach Neoplasms / diagnosis
  • Stomach Neoplasms / genetics
  • Stomach Neoplasms / mortality
  • Stomach Neoplasms / surgery*
  • Survival Rate
  • Zollinger-Ellison Syndrome / diagnosis
  • Zollinger-Ellison Syndrome / genetics
  • Zollinger-Ellison Syndrome / mortality
  • Zollinger-Ellison Syndrome / surgery*

Substances

  • Gastrins
  • Secretin