PMID- 16327485
OWN - NLM
STAT- MEDLINE
DCOM- 20060117
LR  - 20240314
IS  - 0003-4932 (Print)
IS  - 1528-1140 (Electronic)
IS  - 0003-4932 (Linking)
VI  - 242
IP  - 6
DP  - 2005 Dec
TI  - Outcome of duodenopancreatic resections in patients with multiple endocrine 
      neoplasia type 1.
PG  - 757-64, discussion 764-6
AB  - 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.
FAU - Bartsch, Detlef K
AU  - Bartsch DK
AD  - Department of Surgery, Division of Endocrinology, Philipps-University Marburg, 
      Baldingerstrasse, D-35043 Marburg, Germany. bartsch@mailer.uni-marburg.de
FAU - Fendrich, Volker
AU  - Fendrich V
FAU - Langer, Peter
AU  - Langer P
FAU - Celik, Ilhan
AU  - Celik I
FAU - Kann, Peter H
AU  - Kann PH
FAU - Rothmund, Matthias
AU  - Rothmund M
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Ann Surg
JT  - Annals of surgery
JID - 0372354
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Chi-Square Distribution
MH  - DNA Mutational Analysis
MH  - Female
MH  - Gastrinoma/pathology/surgery
MH  - Humans
MH  - Insulinoma/pathology/surgery
MH  - Lymphatic Metastasis
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/genetics/pathology/*surgery
MH  - Neoplasm Recurrence, Local
MH  - Pancreatic Neoplasms/genetics/pathology/*surgery
MH  - Pancreaticoduodenectomy/*methods
MH  - Postoperative Complications
MH  - Prospective Studies
MH  - Statistics, Nonparametric
MH  - Treatment Outcome
MH  - Vipoma/pathology/surgery
PMC - PMC1409888
EDAT- 2005/12/06 09:00
MHDA- 2006/01/18 09:00
PMCR- 2006/06/01
CRDT- 2005/12/06 09:00
PHST- 2005/12/06 09:00 [pubmed]
PHST- 2006/01/18 09:00 [medline]
PHST- 2005/12/06 09:00 [entrez]
PHST- 2006/06/01 00:00 [pmc-release]
AID - 00000658-200512000-00002 [pii]
AID - 0000658-200512000-00002 [pii]
AID - 10.1097/01.sla.0000189549.51913.d8 [doi]
PST - ppublish
SO  - Ann Surg. 2005 Dec;242(6):757-64, discussion 764-6. doi: 
      10.1097/01.sla.0000189549.51913.d8.