Somatostatinomas

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Somatostatin is a tetradecapeptide gut hormone that inhibits the release or action of almost all other gut hormones, including insulin, glucagon, gastrin, and cholecystokinin. Excessive production of somato-statin by a tumor arising from delta pancreatic islet cells leads to a clinical syndrome featuring steator-rhea, diarrhea, mild diabetes mellitus, cholelithiasis, weight loss, anemia, and hypochlorhydria.34 These are exceedingly rare tumors. In the largest collected series to date describing 48 patients, 27 patients had pancreatic primary tumors and 21 had intestinal primaries.35 The mean age at presentation was 51 years (range 26 to 84 years), and 27 (56%) patients were women. Only 26% of patients were free of metastases at the time of presentation.36

Figure 15-8. This photograph depicts the operative specimen of the en bloc distal pancreatectomy and splenectomy with the cut surface of the glucagonoma tumor.
Figure 15-9. There is rapid resolution of the glucagonoma rash on the patient's trunk after removal of her tumor.

Approximately half of the patients have associated endocrine disorders. Duodenal somatostatinomas have carcinoid-like features and have been associated with von Recklinghausen's disease and pheochromo-cytoma, with or without MEN type IIB.37

The diagnosis is established in a patient with suggestive clinical features by demonstration of increased plasma concentration of somatostatin-like immunoreactivity.38 Tolbutamide or calcium penta-gastrin infusion may be useful as a provocative test in the diagnosis of patients with an unclear clinical picture. Because of the rarity of this entity, few patients have been studied, so the reliability of diagnostic tests is not established.

Similarly, optimum surgical approaches have not been established. For small (< 2 cm) localized intestinal somatostatinomas, local wedge resection seems adequate.39 Pancreatic resection and wedge resection of limited hepatic metastases may be justified for larger tumors as long-term survival has been reported after this operation for metastatic somato-statinomas. No series of metastatic somatostatino-mas treated by chemotherapy have been reported. Most treat these as they would other malignant neuroendocrine tumors, with the preferred regimen usually being streptozocin and doxorubicin.

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