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Outcome of duodenopancreatic resections in patients with multiple endocrine neoplasia type 1.
Ann Surg. 2005 Dec; 242(6):757-64, discussion 764-6.AnnS

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+Show Affiliations

Department of Surgery, Division of Endocrinology, Philipps-University Marburg, Baldingerstrasse, D-35043 Marburg, Germany. bartsch@mailer.uni-marburg.deNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

16327485

Citation

Bartsch, Detlef K., et al. "Outcome of Duodenopancreatic Resections in Patients With Multiple Endocrine Neoplasia Type 1." Annals of Surgery, vol. 242, no. 6, 2005, pp. 757-64, discussion 764-6.
Bartsch DK, Fendrich V, Langer P, et al. Outcome of duodenopancreatic resections in patients with multiple endocrine neoplasia type 1. Ann Surg. 2005;242(6):757-64, discussion 764-6.
Bartsch, D. K., Fendrich, V., Langer, P., Celik, I., Kann, P. H., & Rothmund, M. (2005). Outcome of duodenopancreatic resections in patients with multiple endocrine neoplasia type 1. Annals of Surgery, 242(6), 757-64, discussion 764-6.
Bartsch DK, et al. Outcome of Duodenopancreatic Resections in Patients With Multiple Endocrine Neoplasia Type 1. Ann Surg. 2005;242(6):757-64, discussion 764-6. PubMed PMID: 16327485.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Outcome of duodenopancreatic resections in patients with multiple endocrine neoplasia type 1. AU - Bartsch,Detlef K, AU - Fendrich,Volker, AU - Langer,Peter, AU - Celik,Ilhan, AU - Kann,Peter H, AU - Rothmund,Matthias, PY - 2005/12/6/pubmed PY - 2006/1/18/medline PY - 2005/12/6/entrez SP - 757-64, discussion 764-6 JF - Annals of surgery JO - Ann Surg VL - 242 IS - 6 N2 - 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. SN - 0003-4932 UR - https://www.unboundmedicine.com/medline/citation/16327485/Outcome_of_duodenopancreatic_resections_in_patients_with_multiple_endocrine_neoplasia_type_1_ L2 - https://Insights.ovid.com/pubmed?pmid=16327485 DB - PRIME DP - Unbound Medicine ER -