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Ann Surg. 2005 Dec;242(6):757-64, discussion 764-6.

Outcome of duodenopancreatic resections in patients with multiple endocrine neoplasia type 1.

Author information

  • 1Department of Surgery, Division of Endocrinology, Philipps-University Marburg, Baldingerstrasse, D-35043 Marburg, Germany. bartsch@mailer.uni-marburg.de

Abstract

OBJECTIVE:

To evaluate the outcome of an aggressive surgical approach for duodenopancreatic neuroendocrine tumors (PETs) associated with multiple endocrine neoplasia type 1 (MEN1).

SUMMARY BACKGROUND DATA:

The management of PETs is still controversial in the setting of the autosomal dominant inherited MEN1 syndrome.

METHODS:

MEN1 patients that had either biochemical evidence of functioning PETs or visualized nonfunctioning PETs larger than 1 cm in size on imaging were operated. Since 1997, patients were followed annually by biochemical testing and imaging studies.

RESULTS:

Twenty-six genetically confirmed MEN1 patients underwent duodenopancreatic resection for functioning (n = 17) or nonfunctioning (n = 9) PETs. Ten (38%) patients had malignant PETs as characterized by the presence of lymph node (10 patients) and/or distant metastases (2 patients). The surgical approach was selected based on the type, location, and size of PETs. Four Zollinger-Ellison syndrome (ZES) patients required pylorus preserving pancreaticoduodenectomy (PPPD) as initial or redo procedure, 20 patients underwent other duodenopancreatic resections, and 2 patients had simple enucleations of PETs. After median 83 months (range, 5-241 months), 24 patients were alive and 2 patients died of an unrelated cause. All patients with insulinoma or vipoma and 7 of 11 patients with ZES were biochemically cured, including the ZES patients who underwent PPPD. However, 19 of 26 (73%) patients developed new small PETs (<1 cm) in the pancreatic remnant, but no patient had yet detectable metastases on imaging.

CONCLUSIONS:

Early and aggressive surgery of PETs in MEN1 patients prevents the development of liver metastases, which are the most life-threatening determinant. PPPD might be the procedure of choice for MEN1-ZES, which has to be proven in large scale studies.

PMID:
16327485
PMCID:
PMC1409888
[PubMed - indexed for MEDLINE]
Free PMC Article
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