PMID- 10803862
OWN - NLM
STAT- MEDLINE
DCOM- 20000703
LR  - 20190905
IS  - 0334-018X (Print)
IS  - 0334-018X (Linking)
VI  - 13
IP  - 5
DP  - 2000 May
TI  - A concise genetic and clinical guide to multiple endocrine neoplasias and related 
      syndromes.
PG  - 457-65
AB  - Several familial neoplastic syndromes are associated with endocrine gland 
      oncogenesis. The main ones are: multiple endocrine neoplasia type 1 (MEN 1), 
      which affects primarily the pituitary, pancreas, and parathyroid glands; MEN 2A 
      and MEN 2B, which involve mainly the thyroid and parathyroid glands and the 
      adrenal medulla; familial medullary thyroid carcinoma (FMTC), which affects only 
      the thyroid gland; and, finally, Carney complex, which affects the adrenal 
      cortex, pituitary, thyroid gland, and the gonads. Carney complex is also 
      associated with pigmentation abnormalities and myxoid and other neoplasms of 
      mesenchymal origin. Thus, this syndrome also belongs to another group of genetic 
      disorders, those associated with pigmentation defects and multiple tumors, 
      including tumors of the endocrine glands. Peutz-Jeghers syndrome and Cowden 
      disease are just two of these disorders that have recently been elucidated at the 
      molecular level. von Hippel-Lindau disease is another condition that affects the 
      pancreas and adrenal medulla and its gene is also known. The inheritance of the 
      MENs, Carney complex, and related syndromes is autosomal dominant. Clinical 
      recognition of these syndromes at a young age improves clinical outcome and 
      prognosis of the various tumors and decreases associated morbidity and mortality. 
      This review considers a wider, more inclusive view of the MEN syndromes, 
      summarizes their clinical features and presents the newest information on their 
      molecular elucidation.
FAU - Stratakis, C A
AU  - Stratakis CA
AD  - Unit of Genetics and Endocrinology, Developmental Endocrinology Branch, National 
      Institute of Child Health and Human Development, National Institutes of Health, 
      Bethesda, MD 20892-1862, USA. stratakc@cc1.nichd.nih.gov
FAU - Ball, D W
AU  - Ball DW
LA  - eng
PT  - Journal Article
PT  - Review
PL  - Germany
TA  - J Pediatr Endocrinol Metab
JT  - Journal of pediatric endocrinology & metabolism : JPEM
JID - 9508900
SB  - IM
MH  - Humans
MH  - Male
MH  - Multiple Endocrine Neoplasia/*genetics/*physiopathology
RF  - 36
EDAT- 2000/05/10 09:00
MHDA- 2000/07/08 11:00
CRDT- 2000/05/10 09:00
PHST- 2000/05/10 09:00 [pubmed]
PHST- 2000/07/08 11:00 [medline]
PHST- 2000/05/10 09:00 [entrez]
AID - 10.1515/jpem.2000.13.5.457 [doi]
PST - ppublish
SO  - J Pediatr Endocrinol Metab. 2000 May;13(5):457-65. doi: 
      10.1515/jpem.2000.13.5.457.