PMID- 11114630
OWN - NLM
STAT- MEDLINE
DCOM- 20010103
LR  - 20220408
IS  - 0039-6060 (Print)
IS  - 0039-6060 (Linking)
VI  - 128
IP  - 6
DP  - 2000 Dec
TI  - Pancreaticoduodenal endocrine tumors in multiple endocrine neoplasia type 1: 
      surgery or surveillance?
PG  - 958-66
AB  - BACKGROUND: The management of pancreaticoduodenal endocrine tumors (PETs) remains 
      controversial in multiple endocrine neoplasia type 1 (MEN 1). METHODS: Twenty-one 
      patients with MEN 1 and PETs were analyzed for outcome of surgery and 
      surveillance with special regard to the genotype based on MEN1 gene mutation 
      analysis. RESULTS: Nine patients had gastrinomas, 5 had nonfunctioning tumors, 4 
      had insulinomas, 2 had insulinomas and gastrinomas, and 1 had a VIPoma. Seven 
      patients (33%) had malignant tumors. Sixteen patients (76%) were initially 
      treated by pancreatic resections or tumor enucleations or both. Six patients 
      underwent reoperations for recurrences or lymph node metastases or both. Fifteen 
      of the 16 operated patients are alive, and 12 have no evidence of disease after a 
      median follow-up of 78 months (range, 1-198 months). Five patients with 
      gastrinomas or nonfunctioning tumors, but no symptoms, underwent surveillance; 1 
      of them developed lymph node metastases. Patients with truncating mutations in 
      the N- or C-terminal region (exons 2, 9, or 10) of the MEN1 gene had a 
      significantly higher rate of malignant tumors (55% vs 10%; P <.05) than patients 
      with other mutations. CONCLUSIONS: An aggressive surgical approach is justified 
      for PETs in patients with MEN 1. However, MEN1 gene mutations in exons 3 to 8 
      seem to be associated with mild behavior of PETs, possibly allowing surveillance 
      in asymptomatic patients.
FAU - Bartsch, D K
AU  - Bartsch DK
AD  - Department of Surgery, Philipps-University Marburg, Germany.
FAU - Langer, P
AU  - Langer P
FAU - Wild, A
AU  - Wild A
FAU - Schilling, T
AU  - Schilling T
FAU - Celik, I
AU  - Celik I
FAU - Rothmund, M
AU  - Rothmund M
FAU - Nies, C
AU  - Nies C
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Surgery
JT  - Surgery
JID - 0417347
RN  - 0 (MEN1 protein, human)
RN  - 0 (Neoplasm Proteins)
RN  - 0 (Proto-Oncogene Proteins)
SB  - IM
MH  - Adult
MH  - Duodenal Neoplasms/genetics/pathology/*surgery
MH  - Female
MH  - Genotype
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/genetics/pathology/*surgery
MH  - Mutation
MH  - Neoplasm Proteins/genetics
MH  - Pancreatic Neoplasms/genetics/pathology/*surgery
MH  - *Proto-Oncogene Proteins
EDAT- 2000/12/15 11:00
MHDA- 2001/02/28 10:01
CRDT- 2000/12/15 11:00
PHST- 2000/12/15 11:00 [pubmed]
PHST- 2001/02/28 10:01 [medline]
PHST- 2000/12/15 11:00 [entrez]
AID - S0039-6060(00)96337-2 [pii]
AID - 10.1067/msy.2000.109727 [doi]
PST - ppublish
SO  - Surgery. 2000 Dec;128(6):958-66. doi: 10.1067/msy.2000.109727.