PMID- 27207564
OWN - NLM
STAT- MEDLINE
DCOM- 20180117
LR  - 20191008
IS  - 1479-6821 (Electronic)
IS  - 1351-0088 (Print)
IS  - 1351-0088 (Linking)
VI  - 23
IP  - 6
DP  - 2016 Jun
TI  - Endocrine neoplasms in familial syndromes of hyperparathyroidism.
PG  - R229-47
LID - 10.1530/ERC-16-0059 [doi]
AB  - Familial syndromes of hyperparathyroidism, including multiple endocrine neoplasia 
      type 1 (MEN1), multiple endocrine neoplasia type 2A (MEN2A), and the 
      hyperparathyroidism-jaw tumor (HPT-JT), comprise 2-5% of primary 
      hyperparathyroidism cases. Familial syndromes of hyperparathyroidism are also 
      associated with a range of endocrine and nonendocrine tumors, including potential 
      malignancies. Complications of the associated neoplasms are the major causes of 
      morbidities and mortalities in these familial syndromes, e.g., parathyroid 
      carcinoma in HPT-JT syndrome; thymic, bronchial, and enteropancreatic 
      neuroendocrine tumors in MEN1; and medullary thyroid cancer and pheochromocytoma 
      in MEN2A. Because of the different underlying mechanisms of neoplasia, these 
      familial tumors may have different characteristics compared with their sporadic 
      counterparts. Large-scale clinical trials are frequently lacking due to the 
      rarity of these diseases. With technological advances and the development of new 
      medications, the natural history, diagnosis, and management of these syndromes 
      are also evolving. In this article, we summarize the recent knowledge on 
      endocrine neoplasms in three familial hyperparathyroidism syndromes, with an 
      emphasis on disease characteristics, molecular pathogenesis, recent developments 
      in biochemical and radiological evaluation, and expert opinions on surgical and 
      medical therapies. Because these familial hyperparathyroidism syndromes are 
      associated with a wide variety of tumors in different organs, this review is 
      focused on those endocrine neoplasms with malignant potential.
CI  - (c) 2016 Society for Endocrinology.
FAU - Li, Yulong
AU  - Li Y
AD  - Metabolic Diseases BranchNational Institute of Diabetes and Digestive and Kidney 
      Diseases.
FAU - Simonds, William F
AU  - Simonds WF
AD  - National Institutes of HealthBethesda, Maryland, USA wfs@helix.nih.gov.
LA  - eng
GR  - Z01 DK043012-06/Intramural NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Intramural
PT  - Review
DEP - 20160520
PL  - England
TA  - Endocr Relat Cancer
JT  - Endocrine-related cancer
JID - 9436481
SB  - IM
MH  - *Endocrine Gland Neoplasms/diagnosis/epidemiology/genetics/therapy
MH  - Humans
MH  - *Hyperparathyroidism/diagnosis/epidemiology/genetics/therapy
MH  - Syndrome
PMC - PMC4917437
MID - NIHMS790172
OTO - NOTNLM
OT  - hyperparathyroidism-jaw tumor (HPT-JT)
OT  - malignant tumor
OT  - multiple endocrine neoplasia type 1 (MEN1)
OT  - multiple endocrine neoplasia type 2A (MEN2A)
OT  - neuroendocrine tumor
COIS- Declaration of interest: there is no conflict of interest that could be perceived 
      as prejudicing the impartiality of the research reported.
EDAT- 2016/05/22 06:00
MHDA- 2016/05/22 06:01
PMCR- 2017/05/20
CRDT- 2016/05/22 06:00
PHST- 2016/05/18 00:00 [received]
PHST- 2016/05/20 00:00 [accepted]
PHST- 2016/05/22 06:00 [entrez]
PHST- 2016/05/22 06:00 [pubmed]
PHST- 2016/05/22 06:01 [medline]
PHST- 2017/05/20 00:00 [pmc-release]
AID - ERC-16-0059 [pii]
AID - 10.1530/ERC-16-0059 [doi]
PST - ppublish
SO  - Endocr Relat Cancer. 2016 Jun;23(6):R229-47. doi: 10.1530/ERC-16-0059. Epub 2016 
      May 20.