Dr Detlef Bartsch and colleagues evaluated the outcome of an aggressive surgical approach for duodenopancreatic neuroendocrine tumors.
The investigators assessed the association of duodenopancreatic neuroendocrine tumors associated with multiple endocrine neoplasia type 1 (MEN1).
The management of duodenopancreatic neuroendocrine tumors is still controversial in the setting of the autosomal dominant inherited MEN1 syndrome.
MEN1 patients that had either biochemical evidence of functioning duodenopancreatic neuroendocrine tumors were operated.
The investigators also operated patients with visualized nonfunctioning duodenopancreatic neuroendocrine tumors larger than 1 cm in size on imaging.
Since 1997, patients were followed annually by biochemical testing and imaging studies.
|7 of 11 patients with Zollinger-Ellison syndrome were biochemically cured|
|Annals of Surgery|
The team reported that 26 genetically confirmed MEN1 patients underwent duodenopancreatic resection for functioning or nonfunctioning duodenopancreatic neuroendocrine tumors.
The investigators found that 38% patients had malignant duodenopancreatic neuroendocrine tumors.
The malignant duodenopancreatic neuroendocrine tumors were characterized by the presence of lymph node and/or distant metastases.
The surgical approach was selected based on the type, location, and size of duodenopancreatic neuroendocrine tumors.
The team noted that 4 Zollinger-Ellison syndrome patients required pylorus preserving pancreaticoduodenectomy as initial or redo procedure.
A further 20 patients underwent other duodenopancreatic resections, and 2 patients had simple enucleations of duodenopancreatic neuroendocrine tumors.
After median 83 months, the investigators observed that 24 patients were alive and 2 patients died of an unrelated cause.
All patients with insulinoma or vipoma and 7 of 11 patients with Zollinger-Ellison syndrome were biochemically cured.
The investigators noted that the patients who underwent pylorus preserving pancreaticoduodenectomy were also biochemically cured.
However, 19 of 26 patients developed new small duodenopancreatic neuroendocrine tumors less than 1 cm in the pancreatic remnant.
The investigators noted that no patient had yet detectable metastases on imaging.
Dr Bartsch's team commented, “Early and aggressive surgery of duodenopancreatic neuroendocrine tumors in MEN1 patients prevents the development of liver metastases, which are the most life-threatening determinant.”
“Pylorus preserving pancreaticoduodenectomy, might be the procedure of choice for MEN1-Zollinger-Ellison syndrome.”
“This has to be proven in large scale studies.”