PMID- 1357827
OWN - NLM
STAT- MEDLINE
DCOM- 19921104
LR  - 20190824
IS  - 0364-2313 (Print)
IS  - 0364-2313 (Linking)
VI  - 16
IP  - 4
DP  - 1992 Jul-Aug
TI  - Pancreatic tumors in multiple endocrine neoplasia type 1: clinical presentation 
      and surgical treatment.
PG  - 611-8; discussion 618-9
AB  - Among 33 patients with endocrine pancreatic tumors due to multiple endocrine 
      neoplasia type 1 (MEN-1), 19 (58%) patients had hypergastrinemia, 7 (21%) 
      patients had hyperinsulinism, and 7 (21%) patients had clinically non-functioning 
      lesions. At least one gross tumor was found in all patients undergoing pancreatic 
      surgery, including those with negative localization studies prior to operation. 
      The patients also had additional macroscopic tumors as well as numerous 
      microadenomas, and the lesions frequently were positive for immunostaining with 
      multiple hormones, mainly pancreatic polypeptide, insulin, glucagon, and 
      somatostatin. Duodenal endocrine lesions were found in 4 of 5 investigated 
      patients and stained with gastrin and somatostatin antibodies. Distal, mainly 
      subtotal pancreatic resection, was performed in 18 patients, eventually combined 
      with caput tumor enucleation or duodenotomy, while a few patients underwent only 
      tumor enucleation or a Whipple procedure. The long-term outcome of operation was 
      most favorable in patients with hyperinsulinism; only 1 patient had clinical 
      recurrence. Patients with hypergastrinemia experienced only transitory lowering 
      of serum gastrin values after pancreatic surgery and 47% of them had or developed 
      metastases. Such tumor spread was seen in 57% of the patients with 
      non-functioning lesions. Nine patients died from progressive tumor disease during 
      follow-up. Consistent with previous studies, we found that surgery is indicated 
      in MEN-1 patients with hyperinsulinism even if a lesion is not visualized by 
      radiology. In addition, these indications should be extended to also include 
      patients with only biochemical markers of disease, including elevations of 
      gastrin, as these indicate the presence of gross tumors.(ABSTRACT TRUNCATED AT 
      250 WORDS)
FAU - Grama, D
AU  - Grama D
AD  - Department of Surgery, University Hospital, Uppsala, Sweden.
FAU - Skogseid, B
AU  - Skogseid B
FAU - Wilander, E
AU  - Wilander E
FAU - Eriksson, B
AU  - Eriksson B
FAU - Martensson, H
AU  - Martensson H
FAU - Cedermark, B
AU  - Cedermark B
FAU - Ahren, B
AU  - Ahren B
FAU - Kristofferson, A
AU  - Kristofferson A
FAU - Oberg, K
AU  - Oberg K
FAU - Rastad, J
AU  - Rastad J
AU  - et al.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - World J Surg
JT  - World journal of surgery
JID - 7704052
RN  - 0 (Gastrins)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Female
MH  - Follow-Up Studies
MH  - *Gastrinoma/diagnosis/mortality/surgery
MH  - Gastrins/blood
MH  - Humans
MH  - Hyperinsulinism/etiology
MH  - *Insulinoma/diagnosis/mortality/surgery
MH  - Life Tables
MH  - Male
MH  - Middle Aged
MH  - *Multiple Endocrine Neoplasia/diagnosis/mortality/surgery
MH  - *Pancreatic Neoplasms/diagnosis/mortality/surgery
MH  - Retrospective Studies
EDAT- 1992/07/01 00:00
MHDA- 1992/07/01 00:01
CRDT- 1992/07/01 00:00
PHST- 1992/07/01 00:00 [pubmed]
PHST- 1992/07/01 00:01 [medline]
PHST- 1992/07/01 00:00 [entrez]
AID - 10.1007/BF02067335 [doi]
PST - ppublish
SO  - World J Surg. 1992 Jul-Aug;16(4):611-8; discussion 618-9. doi: 
      10.1007/BF02067335.