PMID- 17961653
OWN - NLM
STAT- MEDLINE
DCOM- 20071030
LR  - 20191110
IS  - 0003-4266 (Print)
IS  - 0003-4266 (Linking)
VI  - 68 Suppl 1
DP  - 2007 Jun
TI  - [Endocrine tumors of the pancreas (EPTs) in multiple endocrine neoplasia (MEN1): 
      up-date on prognostic factors, diagnostic procedures and treatment].
PG  - 1-8
AB  - Endocrine pancreatic tumors (EPTs) are uncommon tumors, representing 1-2% of all 
      pancreatic neoplasms. They are categorized on the basis of their clinical 
      features into functioning and non-functioning tumors. EPTs may be part of the 
      multiple endocrine neoplasia type 1 (MEN 1), an autosomal dominant syndrome due 
      to inactivating germline mutation of the menin gene. Somatic mutations of menin 
      are present in about 20% of sporadic neoplasms, particularly gastrinomas and 
      insulinomas. 30-75% of patients with MEN1 have EPTs. The most prevalent are the 
      gastrinomas (20-60%), then the insulinomas (5-10%), the glucagonamas and VIPomas 
      (6-10%), whereas the nonfunctioning EPTs are present in 20-40% of patients. The 
      most important biochemical screening marker for EPTs is chromogranin A, as it 
      increases in 50-80% of patients. The most important negative prognostic factors 
      are the presence of metastases, the gross invasion of adjacent organs, the 
      angioinvasion, the perineural invasion and an immunopositivity for Ki-67 > 2%. 
      Among the different imaging techniques, echoendoscopy, computed tomography (CT) 
      and magnetic resonance imaging (MRI) are indicated for the detection of the 
      primary tumor, but (III)In-octreotide scintigraphy has the highest sensitivity 
      for detecting metastases. The choice of treatment is still debated and is 
      different when the tumor occurs as a part of the MEN syndrome. The surgical 
      treatment is the first choice for insulinomas and is more controversial for 
      gastrinomas. The medical treatment includes somatostatin analogues (SA), 
      chemotherapy and interferon-alpha (IFN-alpha). SA seem to improve the symptoms 
      and have an antiproliferative effect, the most striking effect being seen in 
      patients with VIPomas. Chemotherapy, which is generally proposed as a combination 
      of streptozotocin (STZ) and 5-fluorouracil (5-FU) or doxorubicin, is indicated 
      when the tumors tend to grow. Interferon-alpha (IFN-alpha) stimulates the immune 
      system, blocks the tumor cells in the G1/S-phase of the cell cycle, inhibits 
      protein and hormone synthesis and inhibits angionenesis. Treatment with IFN has 
      been shown to produce symptomatic response in 40-60% of patients, a biochemical 
      response in 30-60% and tumor shrinkage in 10-15%.
FAU - Libe, R
AU  - Libe R
AD  - INSERM U567, lnstitut Cochin, Endocrinology, Metabolism & Cancer Department, 27 
      rue du Fbg St. Jacques, 75014 Paris, France. rossella.libe@cch.aphp.fr
FAU - Chanson, P
AU  - Chanson P
LA  - fre
PT  - English Abstract
PT  - Journal Article
TT  - Les tumeurs endocrines du pancreas lors de la neoplasie endocrinienne multiple 
      type 1 (NEM1): actualites sur les facteurs pronostiques, l'imagerie et le 
      traitement.
PL  - France
TA  - Ann Endocrinol (Paris)
JT  - Annales d'endocrinologie
JID - 0116744
SB  - IM
EIN - Ann Endocrinol (Paris). 2008 Nov;69(5):459-60
MH  - Antineoplastic Combined Chemotherapy Protocols/therapeutic use
MH  - *Carcinoma, Islet Cell/diagnosis/epidemiology/pathology/therapy
MH  - Humans
MH  - *Multiple Endocrine Neoplasia Type 1/diagnosis/epidemiology/pathology/therapy
MH  - Prognosis
EDAT- 2007/06/01 00:00
MHDA- 2007/06/01 00:01
CRDT- 2007/06/01 00:00
PHST- 2007/06/01 00:00 [pubmed]
PHST- 2007/06/01 00:01 [medline]
PHST- 2007/06/01 00:00 [entrez]
AID - S0003-4266(07)80002-3 [pii]
AID - 10.1016/s0003-4266(07)80002-3 [doi]
PST - ppublish
SO  - Ann Endocrinol (Paris). 2007 Jun;68 Suppl 1:1-8. doi: 
      10.1016/s0003-4266(07)80002-3.