PMID- 8813467
OWN - NLM
STAT- MEDLINE
DCOM- 19970114
LR  - 20180216
IS  - 0012-2823 (Print)
IS  - 0012-2823 (Linking)
VI  - 57 Suppl 1
DP  - 1996
TI  - Biological aspects of neuroendocrine gastro-enteropancreatic tumours.
PG  - 42-4
AB  - Carcinoid tumours may develop from enterochromaffin cells in the gastrointestinal 
      tract. Benign insulin-producing tumours may develop from islet cells, whereas 
      other islet cell tumours might derive from multipotent stem cells in the 
      pancreatic ducts. The idea that multiple endocrine neoplasia type 1 (MEN-1) 
      tumours in the pancreas originate from multipotent stem cells is supported by our 
      demonstration that CD44 is expressed in exocrine cells, in gastrin-producing 
      endocrine cells only and in some non-functioning islet cell tumours; there are no 
      gastrin-producing cells in the adult pancreas. We have identified phospholipase C 
      beta 3 (PLC beta 3) as the gene implicated in MEN-1. It appears to be a tumour 
      suppressor gene since it is expressed in endocrine pancreatic tumours, some lung 
      carcinoids, and medullary thyroid carcinomas. So neuroendocrine tumours might 
      have a dual growth-regulating system, involving both traditional growth factors 
      through the tyrosine kinase system and also G-protein-mediated growth signals. 
      Deletion of PLC beta 3, which is an important enzyme in the signal transduction 
      pathway of G-protein-mediated signals, might be important in the growth 
      regulation of neuroendocrine tumours. It is proposed that its deletion causes 
      dysregulation of growth control in neuroendocrine cells, with possible distortion 
      of the apoptotic process. In the last stage of the disease, tumour biology is 
      altered and becomes more aggressive. Further, chromogranin A may be both a tumour 
      marker for neuroendocrine tumours and a growth-promoting agent for neuroendocrine 
      tumour cells; it is a very good marker of tumour mass but is also related to poor 
      prognosis of survival. Mutation analyses of PLC beta 3 and studies of the 
      growth-promoting effect of chromogranin are ongoing and should lead to more 
      effective therapies.
FAU - Oberg, K
AU  - Oberg K
AD  - Department of Internal Medicine, Uppsala University, Sweden.
LA  - eng
PT  - Journal Article
PT  - Review
PL  - Switzerland
TA  - Digestion
JT  - Digestion
JID - 0150472
SB  - IM
MH  - Animals
MH  - Humans
MH  - Neuroendocrine Tumors/metabolism/*physiopathology
MH  - Pancreatic Neoplasms/metabolism/*physiopathology
MH  - Stomach Neoplasms/metabolism/*physiopathology
RF  - 5
EDAT- 1996/01/01 00:00
MHDA- 1996/01/01 00:01
CRDT- 1996/01/01 00:00
PHST- 1996/01/01 00:00 [pubmed]
PHST- 1996/01/01 00:01 [medline]
PHST- 1996/01/01 00:00 [entrez]
AID - 10.1159/000201393 [doi]
PST - ppublish
SO  - Digestion. 1996;57 Suppl 1:42-4. doi: 10.1159/000201393.