PMID- 26660147
OWN - NLM
STAT- MEDLINE
DCOM- 20161019
LR  - 20230722
IS  - 1868-8500 (Electronic)
IS  - 1868-8497 (Print)
IS  - 1868-8497 (Linking)
VI  - 7
IP  - 1
DP  - 2016 Feb
TI  - 5th International ACC Symposium: Hereditary Predisposition to Childhood ACC and 
      the Associated Molecular Phenotype: 5th International ACC Symposium Session: Not 
      Just for Kids!
PG  - 36-9
LID - 10.1007/s12672-015-0244-z [doi]
AB  - Adrenocortical carcinoma (ACC) affects children and adults. Roughly 50% of very 
      early onset ACCs occur in children with germline TP53 mutations. Several recent 
      studies have extended our understanding in basic, clinical, and translational 
      genetics with regard to TP53 germline predisposition in ACC patients. The recent 
      description of the molecular landscape of pediatric ACCs provided insight into 
      differences of tumors arising in patients with and without TP53 germline 
      mutation. Another recent important finding is that not all TP53 mutations are 
      equal in their tumor suppressing potential. It has now been shown that family 
      histories as well as molecular characteristics of preserved TP53 functions vary 
      greatly between mutations. It also has become clear that adult patients with ACC 
      often harbor germline mutations causing hereditary syndromes, including 
      Li-Fraumeni syndrome (LFS), Lynch syndrome, and multiple endocrine neoplasia type 
      1 (MEN1).
FAU - Else, Tobias
AU  - Else T
AD  - Division of Metabolism, Endocrinology and Diabetes (MEND), University of Michigan 
      Health System, 2560E, MSRB2, 1500 East Medical Center Dr, Ann Arbor, MI, 
      48109-5674, USA. telse@umich.edu.
FAU - Rodriguez-Galindo, Carlos
AU  - Rodriguez-Galindo C
AD  - Department of Global Pediatric Medicine, St Jude Children's Research Hospital, 
      Memphis, TN, USA.
AD  - Department of Oncology, St Jude Children's Research Hospital, Memphis, TN, USA.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20151210
PL  - United States
TA  - Horm Cancer
JT  - Hormones & cancer
JID - 101518427
RN  - 0 (TP53 protein, human)
RN  - 0 (Tumor Suppressor Protein p53)
SB  - IM
MH  - Adolescent
MH  - Adrenal Cortex Neoplasms/diagnosis/*genetics
MH  - Adrenocortical Carcinoma/diagnosis/*genetics
MH  - Age of Onset
MH  - Child
MH  - Child, Preschool
MH  - Colorectal Neoplasms, Hereditary Nonpolyposis/genetics
MH  - Congresses as Topic
MH  - Genetic Predisposition to Disease
MH  - *Germ-Line Mutation
MH  - Humans
MH  - Infant
MH  - Li-Fraumeni Syndrome/genetics
MH  - Multiple Endocrine Neoplasia Type 1/genetics
MH  - Tumor Suppressor Protein p53/*genetics
PMC - PMC10355924
COIS- The authors declare that they have no competing interests.
EDAT- 2015/12/15 06:00
MHDA- 2016/12/30 06:00
PMCR- 2015/12/10
CRDT- 2015/12/15 06:00
PHST- 2015/11/27 00:00 [received]
PHST- 2015/12/01 00:00 [accepted]
PHST- 2015/12/15 06:00 [entrez]
PHST- 2015/12/15 06:00 [pubmed]
PHST- 2016/12/30 06:00 [medline]
PHST- 2015/12/10 00:00 [pmc-release]
AID - 10.1007/s12672-015-0244-z [pii]
AID - 244 [pii]
AID - 10.1007/s12672-015-0244-z [doi]
PST - ppublish
SO  - Horm Cancer. 2016 Feb;7(1):36-9. doi: 10.1007/s12672-015-0244-z. Epub 2015 Dec 
      10.