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

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.

• Authors: Detlef K Bartsch, Volker Fendrich, Peter Langer, Ilhan Celik, Peter H Kann, Matthias Rothmund
• Journal: Annals of surgery (Ann Surg) Vol. 242 Issue 6 Pg. 757-64, discussion 764-6 (Dec 2005) ISSN: 0003-4932 [Print] United States
• PMID: 16327485 (Publication Type: Journal Article)

Topics

• Adolescent
• Adult
• Aged
• Chi-Square Distribution
• DNA Mutational Analysis
• Female
• Gastrinoma (pathology, surgery)
• Humans
• Insulinoma (pathology, surgery)
• Lymphatic Metastasis
• Male
• Middle Aged
• Multiple Endocrine Neoplasia Type 1 (genetics, pathology, surgery)
• Neoplasm Recurrence, Local
• Pancreatic Neoplasms (genetics, pathology, surgery)
• Pancreaticoduodenectomy (methods)
• Postoperative Complications
• Prospective Studies
• Statistics, Nonparametric
• Treatment Outcome
• Vipoma (pathology, surgery)