PMID- 15640547
OWN - NLM
STAT- MEDLINE
DCOM- 20050602
LR  - 20191109
IS  - 1046-3976 (Print)
IS  - 1046-3976 (Linking)
VI  - 15
IP  - 3
DP  - 2004 Fall
TI  - Endocrine tumor syndromes in infancy and childhood.
PG  - 223-6
AB  - Of endocrine disorders that present in the pediatric age range, three heritable 
      tumor syndromes have received a large amount of attention over the past decade, 
      because the relevant genes have been discovered and we have begun to understand 
      the role of some of the proteins involved. In this short review article, the 
      dominantly inherited tumor syndromes multiple endocrine neoplasia type 1 (MEN1), 
      caused by mutations in the menin gene, type 2 (MEN2), caused by mutations in the 
      RET protooncogene, and von Hippel-Lindau disease (VHL), caused by VHL gene 
      abnormalities including mutations, are discussed with an emphasis on the age and 
      mode of presentation of the constituent tumors. Also, current screening and/or 
      treatment guidelines are included.
FAU - de Krijger, Ronald R
AU  - de Krijger RR
AD  - Department of Pathology, Josephine Nefkens Institute, Erasmus MC-University 
      Medical Center, Rotterdam, The Netherlands. r.dekrijger@erasmusmc.nl
LA  - eng
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Endocr Pathol
JT  - Endocrine pathology
JID - 9009288
RN  - 0 (MEN1 protein, human)
RN  - 0 (Proto-Oncogene Proteins)
RN  - 0 (Tumor Suppressor Proteins)
RN  - EC 2.3.2.27 (Ubiquitin-Protein Ligases)
RN  - EC 2.3.2.27 (Von Hippel-Lindau Tumor Suppressor Protein)
RN  - EC 2.7.10.1 (Proto-Oncogene Proteins c-ret)
RN  - EC 2.7.10.1 (Receptor Protein-Tyrosine Kinases)
RN  - EC 6.3.2.- (VHL protein, human)
SB  - IM
MH  - Child
MH  - Child, Preschool
MH  - Humans
MH  - Infant
MH  - Infant, Newborn
MH  - Multiple Endocrine Neoplasia Type 1/genetics/*pathology
MH  - Multiple Endocrine Neoplasia Type 2a/genetics/*pathology
MH  - Mutation
MH  - Proto-Oncogene Proteins/genetics
MH  - Proto-Oncogene Proteins c-ret
MH  - Receptor Protein-Tyrosine Kinases/genetics
MH  - Tumor Suppressor Proteins/genetics
MH  - Ubiquitin-Protein Ligases/genetics
MH  - Von Hippel-Lindau Tumor Suppressor Protein
MH  - von Hippel-Lindau Disease/genetics/*pathology
RF  - 17
EDAT- 2005/01/11 09:00
MHDA- 2005/06/03 09:00
CRDT- 2005/01/11 09:00
PHST- 2005/01/11 09:00 [pubmed]
PHST- 2005/06/03 09:00 [medline]
PHST- 2005/01/11 09:00 [entrez]
AID - EP:15:3:223 [pii]
AID - 10.1385/ep:15:3:223 [doi]
PST - ppublish
SO  - Endocr Pathol. 2004 Fall;15(3):223-6. doi: 10.1385/ep:15:3:223.