PMID- 11914931
OWN - NLM
STAT- MEDLINE
DCOM- 20020521
LR  - 20220316
IS  - 1435-2443 (Print)
IS  - 1435-2443 (Linking)
VI  - 386
IP  - 8
DP  - 2002 Mar
TI  - Timing and extent of surgery in symptomatic and asymptomatic neuroendocrine 
      tumors of the pancreas in MEN 1.
PG  - 558-69
AB  - Pancreaticoduodenal tumors develop in a majority of patients with multiple 
      endocrine neoplasia type 1 (MEN 1) and have a pronounced effect on life 
      expectancy as the principal cause of disease related death. Previous discussion 
      of therapy has focused mainly on syndromes of hormone excess and especially the 
      management of MEN 1 associated Zollinger-Ellison syndrome (ZES). The syndromes of 
      hormone excess, however, may be late features of the endocrinopathy and, when 
      developed, indicate presence of metastases in more than one-third of patients. 
      Recent possibilities for genetic diagnosis have emphasized requirements of 
      prophylactic operation for prevention of malignant development. We recommend 
      screening with biochemical markers and endoscopic ultrasound for early detection, 
      and strong efforts of operative tumor removal before metastases have occurred. 
      Surgery is generally recommended in patients with or without hormonal syndromes 
      in the absence of spread hepatic metastases. Operative procedures include 
      enucleation of tumors in the head of the pancreas, excision of duodenal 
      gastrinomas together with clearance of lymph gland metastases, and as prophylaxis 
      against tumor recurrence combination with distal 80% subtotal pancreatic 
      resection. More extensive surgical tumor reduction is believed to reduce the 
      risks for malignant progression of the pancreaticoduodenal tumors, but this 
      requires further evaluation in MEN 1.
FAU - Akerstrom, Goran
AU  - Akerstrom G
AD  - Department of Surgical Sciences, University Hospital, 75185 Uppsala, Sweden. 
      goran.akerstrom@kirurgi.uu.se
FAU - Hessman, Ola
AU  - Hessman O
FAU - Skogseid, Britt
AU  - Skogseid B
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20020124
PL  - Germany
TA  - Langenbecks Arch Surg
JT  - Langenbeck's archives of surgery
JID - 9808285
RN  - 0 (Biomarkers, Tumor)
SB  - IM
MH  - Adenoma/etiology/pathology/surgery
MH  - Biomarkers, Tumor/metabolism
MH  - Duodenal Neoplasms/etiology/pathology/*surgery
MH  - Glucagonoma/etiology/pathology/surgery
MH  - Humans
MH  - Hyperparathyroidism/*diagnosis/etiology
MH  - Insulinoma/etiology/pathology/surgery
MH  - Multiple Endocrine Neoplasia Type 1/complications/diagnosis/genetics/*surgery
MH  - Pancreatic Neoplasms/etiology/pathology/*surgery
MH  - Prognosis
MH  - Survival Analysis
MH  - Tomography, X-Ray Computed
RF  - 69
EDAT- 2002/03/27 10:00
MHDA- 2002/05/22 10:01
CRDT- 2002/03/27 10:00
PHST- 2001/10/12 00:00 [received]
PHST- 2001/12/02 00:00 [accepted]
PHST- 2002/03/27 10:00 [pubmed]
PHST- 2002/05/22 10:01 [medline]
PHST- 2002/03/27 10:00 [entrez]
AID - 10.1007/s00423-001-0274-6 [doi]
PST - ppublish
SO  - Langenbecks Arch Surg. 2002 Mar;386(8):558-69. doi: 10.1007/s00423-001-0274-6. 
      Epub 2002 Jan 24.