By Marilynn Larkin
NEW YORK (Reuters Health) – Although primary gastrinomas of the liver are rare, they do occur and may metastasize to regional lymph nodes, thus warranting aggressive resection, researchers say.
“A gastrinoma is a pancreatic or duodenal neuroendocrine tumor that makes the hormone gastrin, which causes severe peptic ulcer disease and diarrhea,” Dr. Jeffrey Norton of Stanford University, California, told Reuters Health.
“The tumor most commonly occurs in the pancreatic head or duodenum - the gastrinoma triangle,” he explained in an email. “Liver metastases from these tumors portend a poor prognosis and patients have a higher chance of death.”
“In this article, we report on patients who have liver and bile-duct primary gastrinomas, which may be confused with liver metastases,” he noted. “These patients have a similar prognosis to patients with either duodenal or pancreatic primaries and do very well with surgical resection.”
Dr. Norton and colleagues analyzed data from 223 patients at Stanford University Hospital who were enrolled in a prospective trial to treat Zollinger-Ellison syndrome, a life-threatening disease caused by a gastrinoma. The protocol included using proton-pump inhibitors to control acid hypersecretion, plus surgical resection of the tumor.
As reported online January 24 in JAMA Surgery, seven (3.1%) of the patients who underwent surgery to remove the gastrinoma had liver or biliary-tract primary tumors: five men and two women, with a mean age of 43 at diagnosis.
Patients had positive results on a secretin test, and the mean serum gastrin level was 817 pg/mL (reference level, <100 pg/mL). None had evidence of multiple endocrine neoplasia 1 (MEN1). Four had primary tumors in the liver (one in segment II, two in segment IV, and one in segment V); three in the bile duct (one each in the right, left, and common hepatic ducts).
Surgical resections involved one right hepatic lobectomy, one left lateral segmentectomy, two left hepatic lobectomies, one central hepatectomy, and two bile-duct resections.
Whereas four patients had nodal metastases, none had distant metastases.
No deaths occurred during surgery, but three patients had complications, including bile-duct stricture, portal-vein stricture, and biliary fistula.
Patients were disease-free in the immediate postoperative period; however, three had recurrences in the liver and portal lymph nodes at three, 11, and 15 years.
Three patients (43%) remained free of disease at follow-up, which ranged from 24 months to 26 years (median, 13 years).
The authors stress that primary gastrinomas can and do occur outside the gastrinoma triangle “and must be specifically considered.”
“Their discovery changes the operative approach,” the authors state, “because aggressive liver or bile duct resection is indicated, with high rates of long-term cure and survival and acceptable rates of complications.”
“In addition,” they conclude, “their discovery dictates that lymph nodes in the porta hepatis should be routinely excised because nearly 50% of patients will have lymph node metastases.”
Dr. John McAuliffe, a surgical oncologist at Montefiore Einstein Center for Cancer Care in New York City, commented, “Management strategies and conclusions from this small cohort are difficult to draw.”
“However,” he told Reuters Health by email, “resection is a key component for cure or long-term survival or symptom control for all patients with gastrinomas.
“Even for patients with MEN1,” he said, “resection serves an important role for select patients with larger tumors.”
For patients with lymphatic spread (close to half of the study group), “performing a portal lymph node dissection is a reasonable procedure during biliary and/or hepatic resections,” Dr. McAuliffe noted.
“The procedure adds little to the operative time and morbidity,” he said. “Likewise, after biliary and hepatic resection, it is much more challenging to re-explore patients found to have residual portal lymph node disease. Therefore, clearance of the primary and lymphatic disease in one procedure seems most reasonable even if half of the patients don’t have pathologic lymphatic spread.”
Like the authors, Dr. McAuliffe notes that Gallium 68-labeled DOTATOC scans are the most sensitive for gastrinoma detection; however, they were not available for most of the study period.
While these scans now are available at Montefiore, Dr. McAuliffe says, “they are not yet available at all institutions. Therefore, I recommend that patients with neuroendocrine tumors, particularly rare ones such as gastrinomas, should be evaluated at a specialty center for optimal management.”
JAMA Surg 2018.
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