PMID- 15249710
OWN - NLM
STAT- MEDLINE
DCOM- 20050202
LR  - 20071115
IS  - 1424-3903 (Print)
IS  - 1424-3903 (Linking)
VI  - 4
IP  - 5
DP  - 2004
TI  - Hereditary pancreatic endocrine tumours.
PG  - 417-33; discussion 434-5
AB  - The two main types of hereditary pancreatic neuroendocrine tumours are found in 
      multiple endocrine neoplasia type 1 (MEN-1) and von Hippel-Lindau disease (VHL), 
      but also in the rarer disorders of neurofibromatosis type 1 and tuberous 
      sclerosis. This review considers the major advances that have been made in 
      genetic diagnosis, tumour localization, medical and surgical treatment and 
      palliation with systemic chemotherapy and radionuclides. With the exception of 
      the insulinoma syndrome, all of the various hormone excess syndromes of MEN-1 can 
      be treated medically. The role of surgery however remains controversial ranging 
      from no intervention (except enucleation for insulinoma), intervening for tumours 
      diagnosed only by biochemical criteria, intervening in those tumours only 
      detected radiologically (1-2 cm in diameter) or intervening only if the tumour 
      diameter is > 3 cm in diameter. The extent of surgery is also controversial, 
      although radical lymphadenectomy is generally recommended. Pancreatic tumours 
      associated with VHL are usually non-functioning and tumours of at least 2 cm in 
      diameter should be resected. Practice guidelines recommend that screening in 
      patients with MEN-1 should commence at the age of 5 years for insulinoma and at 
      the age of 20 years for other pancreatic neuroendocrine tumours and variously at 
      10-20 years of age for pancreatic tumours in patients with VHL. The evidence is 
      increasing that the life span of patients may be significantly improved with 
      surgical intervention, mandating the widespread use of tumour surveillance and 
      multidisciplinary team management.
CI  - Copyright 2004 S. Karger AG, Basel and IAP
FAU - Alexakis, N
AU  - Alexakis N
AD  - Department of Surgery, Royal Liverpool University Hospital, Liverpool, UK.
FAU - Connor, S
AU  - Connor S
FAU - Ghaneh, P
AU  - Ghaneh P
FAU - Lombard, M
AU  - Lombard M
FAU - Smart, H L
AU  - Smart HL
FAU - Evans, J
AU  - Evans J
FAU - Hughes, M
AU  - Hughes M
FAU - Garvey, C J
AU  - Garvey CJ
FAU - Vora, J
AU  - Vora J
FAU - Vinjamuri, S
AU  - Vinjamuri S
FAU - Sutton, R
AU  - Sutton R
FAU - Neoptolemos, J P
AU  - Neoptolemos JP
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20040706
PL  - Switzerland
TA  - Pancreatology
JT  - Pancreatology : official journal of the International Association of 
      Pancreatology (IAP) ... [et al.]
JID - 100966936
SB  - IM
MH  - Endocrine Gland Neoplasms/diagnosis/*genetics/therapy
MH  - Humans
MH  - Multiple Endocrine Neoplasia Type 1/diagnosis/therapy
MH  - Neurofibromatosis 1/diagnosis/therapy
MH  - Pancreatic Neoplasms/diagnosis/*genetics/therapy
MH  - Tuberous Sclerosis/diagnosis/therapy
MH  - von Hippel-Lindau Disease/diagnosis/therapy
RF  - 138
EDAT- 2004/07/14 05:00
MHDA- 2005/02/03 09:00
CRDT- 2004/07/14 05:00
PHST- 2004/07/14 05:00 [pubmed]
PHST- 2005/02/03 09:00 [medline]
PHST- 2004/07/14 05:00 [entrez]
AID - S1424-3903(04)80054-1 [pii]
AID - 10.1159/000079616 [doi]
PST - ppublish
SO  - Pancreatology. 2004;4(5):417-33; discussion 434-5. doi: 10.1159/000079616. Epub 
      2004 Jul 6.