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The approach to treatment often depends on the results of preoperative localization studies and findings at exploratory laparotomy. At exploration, 85% of these tumors are found in the gastrinoma triangle with 40% on the surface of the pancreas and 40% outside of the pancreas. Only 15% are found within the substance of the pancreas. Percutaneous transhepatic venous sampling may occasionally provide accurate localization of single sporadic gastrinomas. Resection (enucleation of individual tumors, if technically feasible), and even excision of liver metastases, is associated with long-term cure or disease control.[1]

Standard treatment options:

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General Information About Gastrointestinal Stromal Tumors Treatment

Epidemiology Although they comprise fewer than 1% of all gastrointestinal (GI) tumors, gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the GI tract.[1] It has been estimated that there are 3,300 to 6,000 new GIST cases per year in the United States.[2] A study based on Surveillance, Epidemiology and End Results (SEER) registry data found that the age-adjusted yearly incidence of GIST in the United States was 6.8 per million from 1992 to 2000.[3] However, the...

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  1. Single lesion in head of the pancreas:[2,3,4,5]
    • Enucleation.
    • Parietal cell vagotomy and cimetidine.
    • Total gastrectomy (rarely used with the advent of current therapies).
  2. Single or multiple lesions in the duodenum:[2,3,4,5]
    • Pancreatoduodenectomy.
  3. Single lesion in body/tail of the pancreas:[2,3,4,5]
    • Resection of body/tail.
  4. Multiple lesions in pancreas:[2,3,4,5]
    • Resection of body/tail and, if residual disease is present,
    • Parietal cell vagotomy and cimetidine, or
    • Total gastrectomy (rarely used with the advent of current therapies).
  5. No tumor found:
    • Parietal cell vagotomy and cimetidine.
    • Total gastrectomy (rarely used with the advent of current therapies).
  6. Liver metastases:[6,7,8,9,10,11,12,13]
    • Liver resection where possible.
    • Radiofrequency ablation or cryosurgical ablation.
    • Chemoembolization of liver.
  7. Metastatic disease or disease refractory to surgery and cimetidine:[14,15,16,17,18,19,20,21,22,23]

Patients with hepatic-dominant disease and substantial symptoms caused by tumor bulk or hormone-release syndromes may benefit from procedures that reduce hepatic arterial blood flow to metastases (hepatic arterial occlusion with embolization or with chemoembolization). Such treatment may also be combined with systemic chemotherapy in selected patients. Treatment with proton pump inhibitors or H2 blocking agents may aid in control of peptic symptoms.

In the era of proton pump inhibitors and H2 blocking agents, the potentially lethal hyperacidity and hypersecretory states induced by excessive gastrin production can usually be controlled. In a series of 212 patients with Zollinger-Ellison syndrome (ZES), no patients died of causes related to acid hypersecretion. Of those patients, only 2.3% had been subjected to total gastrectomy, and the cohort upon which the report was based had a long median follow-up period of 13.8 years. Although 32% of the patients died during the course of the study, only 50% of the 67 deaths were attributable to ZES-related causes. Those causes were mainly liver metastases with progressive anorexia and cachexia (67%) or secondary endocrine tumors consequent to MEN-1 syndrome. The development of bone or liver metastases (especially diffuse liver disease) or of ectopic Cushing syndrome during the study period predicted for decreased survival times.[24]


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