PMID- 36925188
OWN - NLM
STAT- MEDLINE
DCOM- 20230322
LR  - 20240402
IS  - 1558-5042 (Electronic)
IS  - 1055-3207 (Print)
IS  - 1055-3207 (Linking)
VI  - 32
IP  - 2
DP  - 2023 Apr
TI  - Multiple Endocrine Neoplasia Type 1 Syndrome Pancreatic Neuroendocrine Tumor 
      Genotype/Phenotype: Is There Any Advance on Predicting or Preventing?
PG  - 315-325
LID - S1055-3207(22)00088-6 [pii]
LID - 10.1016/j.soc.2022.10.008 [doi]
AB  - Multiple endocrine neoplasia type 1 syndrome (MEN1) is a disease caused by 
      mutations in the MEN1 tumor suppressor gene leading to hyperparathyroidism, 
      pituitary adenomas, and entero-pancreatic neuroendocrine tumors. Pancreatic 
      neuroendocrine tumors (PNETs) are a major cause of mortality in patients with 
      MEN1. Identification of consistent genotype-phenotype correlations has remained 
      elusive, but MEN1 mutations in exons 2, 9, and 10 may be associated with 
      metastatic PNETs; patients with these mutations may benefit from more intensive 
      surveillance and aggressive treatment. In addition, epigenetic differences 
      between MEN1-associated PNETs and sporadic PNETs are beginning to emerge, but 
      further investigation is required to establish clear phenotypic associations.
CI  - Published by Elsevier Inc.
FAU - Ramamoorthy, Bhavishya
AU  - Ramamoorthy B
AD  - Surgical Oncology Program, Endocrine Surgery Section, National Cancer Institute, 
      NIH, 10 Center Drive, Building 10 - Room 45952, Bethesda, MD 20892, USA.
FAU - Nilubol, Naris
AU  - Nilubol N
AD  - Surgical Oncology Program, Endocrine Surgery Section, National Cancer Institute, 
      NIH, 10 Center Drive, Building 10 - Room 45952, Bethesda, MD 20892, USA. 
      Electronic address: naris.nilubol@nih.gov.
LA  - eng
GR  - Z99 CA999999/ImNIH/Intramural NIH HHS/United States
GR  - ZIA BC011286/ImNIH/Intramural NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Intramural
PT  - Review
PL  - United States
TA  - Surg Oncol Clin N Am
JT  - Surgical oncology clinics of North America
JID - 9211789
RN  - 0 (MEN1 protein, human)
SB  - IM
MH  - Humans
MH  - Genotype
MH  - *Multiple Endocrine Neoplasia Type 1/genetics/complications/pathology
MH  - *Neuroectodermal Tumors, Primitive/complications
MH  - *Neuroendocrine Tumors/genetics
MH  - *Pancreatic Neoplasms/genetics/pathology
MH  - Phenotype
PMC - PMC10348402
MID - NIHMS1871724
OTO - NOTNLM
OT  - Epigenetic
OT  - Genotype
OT  - Menin
OT  - Multiple endocrine neoplasia type 1 syndrome (MEN1)
OT  - Pancreatic neuroendocrine tumor (PNET)
OT  - Phenotype
COIS- Conflict of interests The authors declare no conflict of interest.
EDAT- 2023/03/17 06:00
MHDA- 2023/03/21 06:00
PMCR- 2024/04/01
CRDT- 2023/03/16 21:57
PHST- 2023/03/16 21:57 [entrez]
PHST- 2023/03/17 06:00 [pubmed]
PHST- 2023/03/21 06:00 [medline]
PHST- 2024/04/01 00:00 [pmc-release]
AID - S1055-3207(22)00088-6 [pii]
AID - 10.1016/j.soc.2022.10.008 [doi]
PST - ppublish
SO  - Surg Oncol Clin N Am. 2023 Apr;32(2):315-325. doi: 10.1016/j.soc.2022.10.008.