Prognosis And Metastatic Disease

Currently, approximately 95% of sporadic patients will have gastrinoma found at surgery, and 60 to 68% of patients will be cured.5,26 The 5-year survival for patients with distant metastatic disease is, on average, no better than 40% (Figure 14-19).23 27 In addition, a minority of these patients will experience accelerated tumor growth, which ultimately results in a more rapid demise. Although the exact mechanism underlying this more malignant tumor behavior has yet to be elucidated, it correlates with the serum level of gastrin and the presence of bilobar liver or bony metastasis.20 It appears that surgery is a potentially effective treatment for metastatic gastrinoma. Patients with localized lymph node metastases seem to benefit most from surgery, and up to 30% may be biochemically cured,4 whereas patients with resected localized metastatic liver disease have an 85% 5-year survival (Figure 14-20).27 Further resection is not

Figure 14-18. Illustration of closure techniques after duodenotomy. Under usual circumstances, the incision is closed transversely (A and B) to minimize the risk of leakage or duodenal stenosis. However, in cases for which a long duodenotomy is necessary to examine the duodenum for the presence of a small gastrinoma, the incision may be closed longitudinally (C).

Figure 14-18. Illustration of closure techniques after duodenotomy. Under usual circumstances, the incision is closed transversely (A and B) to minimize the risk of leakage or duodenal stenosis. However, in cases for which a long duodenotomy is necessary to examine the duodenum for the presence of a small gastrinoma, the incision may be closed longitudinally (C).

Figure 14-19. Kaplan-Meier survival curves for a group of patients with gastrinomas who were evaluated and treated surgically at the National Institutes of Health (NIH). Again, the impact of metastatic disease is illustrated by the decreased survival of those individuals with metastatic liver disease.

Figure 14-19. Kaplan-Meier survival curves for a group of patients with gastrinomas who were evaluated and treated surgically at the National Institutes of Health (NIH). Again, the impact of metastatic disease is illustrated by the decreased survival of those individuals with metastatic liver disease.

always necessary because patients have had similar benefits from surgical ablative therapy of liver metastases.28 Currently, aggressive surgery in appropriate patients with hepatic metastases seems to demonstrate a survival advantage.27

In patients with MEN type I and gastrinoma, the identification of all tumor foci is problematic, and surgery results in a significantly lower cure rate.4'22,28-31 With successful control of gastric acid hypersecretion and the indolent growth pattern of the gastrinoma, distant metastatic disease is the most important cause of morbidity and mortality. Although it has been suggested that gastrinomas in patients with MEN type I appear to behave less aggressively than those found in patients with sporadic disease, they do seem to have an equal rate of metastasis to lymph nodes. In fact, in one report, it was found that 86% of tumors had metastasized to lymph nodes at the time of exploration.29 Duodenal primaries do, however, seem to have a lower rate of metastases to liver. Even in those patients with unresectable disease, hepatic cryosurgery or radiofrequency ablation may reduce symptoms, and the long-term effect of these modalities on survival is similar to resection.

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