Primary gastrinomas of the hepatobiliary tract may occur in patients with Zollinger-Ellison syndrome

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Lymph nodes proximal to hepatic or biliary gastrinomas will likely require extraction as many in the surround region will have metastasized.
Lymph nodes proximal to hepatic or biliary gastrinomas will likely require extraction as many in the surround region will have metastasized.

Hepatobiliary tract primary gastrinomas, though rare, have altered operative methodologies due to associated resections and are the most common extraduodenopancreatic primary position (second to lymph nodes) for gastrinomas, according to a study published in JAMA Surgery.

Jeffrey A Norton, MD, of the Department of Surgery at Stanford University in California, and colleagues conducted a study to report the occurrence and projection of rare gastrinomas initiated in hepatobiliary tract.

The investigation included 223 patients with Zollinger-Ellison syndrome (ZES) treated with proton pump inhibitors to regulate acid secretion levels. Patients also underwent resection to alleviate tumor development.

Main outcomes included occurrence, site, results of surgery, cure, and overall survival rates among patients with gastrinomas of hepatic or biliary origin, which were present in 3% (n=7) of the total cohort (5 men).

Peptic ulcer disease and diarrhea caused by high acid secretion were common ZES symptoms observed in each of the patients, as well as high levels of fasting serum gastrin (mean, 817pg/mL).

Of the 7 patients with primary hepatobiliary gastrinomas, 4 patients had primary gastrinoma of the liver and 3 patients in the bile duct. Six of the 7 patients underwent one surgical resection, while the 7th patient underwent 3 resections. Average tumor size was 2.6 cm (ranging from 1.4 cm-5.5 cm). Metastasized lymph nodes were reported in 4 patients.

The patients (n=7) were postoperatively disease-free for 1 year, with the majority (86%) reporting no signs of ZES at the second year evaluation. At 3, 11, and 15 years, recurrence was reported in 3 patients (one of these patients was disease-free after surgical intervention at year 11 and 15). Long-term follow-up resulted in 3 diseased patients, 3 disease-free, and 1 deceased at year 7 (median follow-up, 13 years; range, 24 months-26 years).

“Primary gastrinomas of the hepatobiliary tract are controversial because duodenopancreatic gastrinomas characteristically metastasize to the liver; therefore, distinguishing whether a neuroendocrine tumor containing gastrin in the liver is a primary of secondary tumor can be difficult,” reported the authors.

“Our results support the conclusion that primary gastrinomas of the hepatobiliary tract in patients with sporadic ZES do occur,” the authors commented. “In addition, our data suggest that discovery of liver or biliary gastrinomas dictates removal of regional lymph nodes (portal and celiac) because nearly half of these patients will have lymph node metastases.”

Reference

  1. Norton JA, Foster DS, Blumgart LH, et al. Incidence and prognosis of primary gastrinomas in the hepatobiliary tract. JAMA Surg. 2018 Jan 24. doi:10.1001/jamasurg.2017.5083  [Epub ahead of print]
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