Nonfunctional Tumors

Most patients with functional tumors have other nonfunctional tumors or those secreting hormones that produce no identifiable syndrome, most commonly arising in the distal pancreas. Most of these are found before their malignant potential is evident and before a functional syndrome has developed. Their presence is one of the primary reasons for recommending a distal pancreatectomy in all MEN 1-ZES and insulinoma patients.30'44

Approximately 5% to 10% of MEN 1 patients acquire neuroendocrine tumors, producing symptoms related entirely to their size, local invasion, or hepatic metastases. These patients may present with weight loss, abdominal pain, jaundice, an abdominal mass, or gastrointestinal hemorrhage. In those with large tumors of the head but without hepatic metastases, Whipple's procedure may be curative. Even when the superior mesenteric vein is involved by a neuroendocrine tumor, resection and vein replacement are indicated whenever such involvement is the only factor preventing total excision of the tumor. Tumors localized to the neck or uncinate involving the superior mesenteric vein or artery may cause portal hypertension with bleeding varices or visceral ischemia, respectively. Surgical resection and revascularization may offer significant palliation, if not cure, and should be considered. Finally, size alone does not always imply local invasion or metastatic disease, and we have resected three neuroendocrine tumors in MEN 1 patients that were 10 cm or larger that showed no features of malignancy or subsequent recurrence.


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