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New methods of diagnosing and treating Zollinger-Ellison syndrome may improve patient survival by curing the disease before it spreads. Zollinger-Ellison syndrome is an uncommon condition marked by significant ulceration of the duodenum, or small intestine, often accompanied by malignancies either in the duodenum or the pancreas. Symptoms include diarrhea, abdominal pain, heartburn and gastric hypersecretion. New techniques for measuring high levels of the secretion-stimulating hormone called gastrin can be useful in diagnosing Zollinger-Ellison syndrome and appear to be more accurate than traditional computed tomographic scans and magnetic resonance imaging for some types of the disease. Acid secretion can be controlled through the use of the drugs omeprazole, lansoprazole, ranitidine or cimetidine. The tumors can be treated surgically, and cancer that has spread to the liver can also be surgically removed if it is not too extensive. More than 90% of patients without liver tumors will survive for at least five years. The diagnosis and treatment of Zollinger-Ellison syndrome may lead to a better understanding of other endocrine tumors of the pancreas.

Hypothesis: Lymph node metastases decrease survival in patients with pancreatic neuroendocrine tumors (pNETs). Design: Prospective database searches. Setting: National Institutes of Health (NIH) and Stanford University Hospital (SUH). Patients: A total of 326 patients underwent surgical exploration for pNETs at the NIH (n=216) and SUH (n=110). Main Outcome Measures: Overall survival, diseaserelated survival, and time to development of liver metastases. Results: Forty patients (12.3%) underwent enucleation and 305 (93.6%) underwent resection. Of the patients who underwent resection, 117 (35.9%) had partial pancreatectomy and 30 (9.2%) had a Whipple procedure. Forty-one patients also had liver resections, 21 had wedge resections, and 20 had lobectomies. Mean follow-up was 8.1 years (range, 0.3-28.6 years). The 10- year overall survival for patients with no metastases or lymph node metastases only was similar at 80%. As expected, patients with liver metastases had a significantly decreased 10-year survival of 30% (P<.001). The time to development of liver metastases was significantly reduced for patients with lymph node metastases alone compared with those with none (P<.001). For the NIH cohort with longer follow-up, disease-related survival was significantly different for those patients with no metastases, lymph node metastases alone, and liver metastases (P<.001). Extent of lymph node involvement in this subgroup showed that disease-related survival decreased as a function of the number of lymph nodes involved (P=.004). Conclusions: As expected, liver metastases decrease survival of patients with pNETs. Patients with lymph node metastases alone have a shorter time to the development of liver metastases that is dependent on the number of lymph nodes involved. With sufficient long-term follow-up, lymph node metastases decrease diseaserelated survival. Careful evaluation of number and extent of lymph node involvement is warranted in all surgical procedures for pNETs. [ABSTRACT FROM AUTHOR]

Background: Surgery for pancreatic endocrine tumors (PETs) with blood vessel involvement is controversial. Hypothesis: Resection of PETs with major blood vessel involvement can be beneficial. Design: The combined databases of the National Institutes of Health and Stanford University hospitals were queried. Main Outcome Measures: Operation, pathologic condition, complications, and disease-free and overall survival. Results: Of 273 patients with PETs, 46 (17%) had preoperative computed tomography evidence of major vascular involvement. The mean size for the primary PET was 5.0 cm. The involved major vessel was as follows: portal vein (n=20), superior mesenteric vein or superior mesenteric artery (n=16), inferior vena cava (n=4), splenic vein (n=4), and heart (n=2). Forty-two of 46 patients had a PET removed: 12 (27%) primary only, 30 (68%) with lymph nodes, and 18 (41%) with liver metastases. PETs were removed by either enucleation (n=7) or resection (n=35). Resections included distal or subtotal pancreatectomy in 23, Whipple in 10, and total in 2. Eighteen patients had concomitant liver resection: 10 wedge resection and 8 anatomic resections. Nine patients had vascular reconstruction: each had reconstruction of the superior mesenteric vein and portal vein, and 1 had concomitant reconstruction of the superior mesenteric artery. There were no deaths, but 12 patients had complications. Eighteen patients (41%) were immediately disease free, and 5 recurred with follow-up, leaving 13 (30%) disease-free long term. The 10-year overall survival was 60%. Functional tumors were associated with a better overall survival (P<.001), and liver metastases decreased overall survival (P<.001). Conclusion: These findings suggest that surgical resection of PETs with vascular abutment/invasion and nodal or distant metastases is indicated. [ABSTRACT FROM AUTHOR]

Background: There is considerable controversy about the treatment of patients with malignant advanced neuroendocrine tumors of the pancreas and duodenum. Aggressive surgery remains a potentially efficacious antitumor therapy but is rarely performed because of its possible morbidity and mortality. Hypothesis: Aggressive resection of advanced neuroendocrine tumors can be performed with acceptable morbidity and mortality rates and may lead to extended survival. Design: The medical records of patients with advanced neuroendocrine tumors who underwent surgery between 1997 and 2002 by a single surgeon at the University of California, San Francisco, were reviewed in an institutional review board–approved protocol. Main Outcome Measures: Surgical procedure, pathologic characteristics, complications, mortality rates, and disease-free and overall survival rates were recorded. Disease-free survival was defined as no tumor identified on radiological imaging studies and no detectable abnormal hormone levels. Proportions were compared statistically using the Fisher exact test. Kaplan-Meier curves were used to estimate survival rates. Results: Twenty patients were identified (11 men and 9 women). Of these, 10 (50%) had gastrinoma, 1 had insulinoma, and the remainder had nonfunctional tumors; 2 had multiple endocrine neoplasia type 1, and 1 had von Hippel-Lindau disease. The mean age was 55 years (range, 34-72 years). In 10 patients (50%), tumors were thought to be unresectable according to radiological imaging studies because of multiple bilobar liver metastases (n = 6), superior mesenteric vein invasion (n = 3), and extensive nodal metastases (n = 1). Tumors were completely removed in 15 patients (75%). Surgical procedures included 8 proximal pancreatectomies (pancreatoduodenectomy or whipple procedure), 3 total pancreatectomies, 9 distal pancreatectomies, and 3 tumor enucleations from the pancreatic head. Superior mesenteric vein reconstruction was done in 3 patients. Liver resections were done in 6 patients, and an extended periaortic node dissection was performed in 1. The spleen was removed in 11 patients, and the left kidney was removed as a result of tumor metastases in 2. Eighteen patients had primary pancreatic tumors, and 2 had duodenal tumors; 2 patients with multiple endocrine neoplasia type 1 had both pancreatic and duodenal tumors. The mean tumor size was 8 cm (range, 0.5-23 cm). Of the patients, 14 (70%) had lymph node metastases and 8 (40%) had liver metastases. The mean postoperative hospital stay was 11.5 days (range, 6-26 days). Six patients (30%) had postoperative complications. There was a significantly greater incidence of pancreatic fistulas with enucleations compared with resections (P = .04). There were no operative deaths. The mean follow-up period was 19 months (range, 1-96 months); 18 patients (90%) are alive, 2 died of progressive tumor, and 12 (60%) are disease-free. The actuarial overall survival rate is 80% at 5 years, and disease-free survival rates indicate that all tumors will recur by the 7-year follow-up visit. Conclusions: Aggressive surgery including pancreatectomy, splenectomy, superior mesenteric vein reconstruction, and liver resection can be done with acceptable morbidity and low mortality rates for patients with advanced neuroendocrine tumors. Although survival rates following surgery are excellent, most patients will develop a recurrent tumor. These findings suggest that conventional contraindications to surgical resection, such as superior mesenteric vein invasion and nodal or distant metastases, should be reconsidered in patients with advanced neuroendocrine tumors. [ABSTRACT FROM AUTHOR]

Presents advances in the treatment of gastric hypersecretion and gastrinoma in the Zollinger-Ellison syndrome (ZES). ZES case studies; Clinical manifestations of the syndrome; Nature of gastrinomas associated with ZES; Relationship between ZES and multiple endocrine neoplasia type 1; Treatment of gastric acid hypersecretion with hydrogen/potassium adenosine triphosphatase inhibitors.

Presents a letter to the editor about HIV testing of pregnant women.