PMID- 14871962
OWN - NLM
STAT- MEDLINE
DCOM- 20040928
LR  - 20201113
IS  - 1078-0432 (Print)
IS  - 1078-0432 (Linking)
VI  - 10
IP  - 3
DP  - 2004 Feb 1
TI  - Meningiomas may be a component tumor of multiple endocrine neoplasia type 1.
PG  - 869-80
AB  - PURPOSE: Recently, an increased incidence of some nonendocrine tumors are 
      reported in patients with multiple endocrine neoplasia type 1 (MEN 1). There are 
      rare reports of meningiomas and other central nervous system tumors in these 
      patients, but it is unknown if they are more frequent or if allelic loss of the 
      MEN1 gene is important in their pathogenesis. The aim of this study was to 
      address these two latter questions. EXPERIMENTAL DESIGN: Results from a 
      prospective study of 74 MEN 1 patients with suspected/proven pancreatic endocrine 
      tumors (PETs) were analyzed, as well as molecular studies performed on a resected 
      meningioma. All patients had serial brain imaging studies (computed tomography, 
      magnetic resonance imaging, and octreoscanning since 1994) and yearly studies 
      evaluating MEN 1 involvement with a mean follow-up of 7.2 years. Results were 
      compared with 185 patients with sporadic Zollinger-Ellison syndrome. RESULTS: Six 
      patients (8%) had meningiomas. Meningiomas were single and found late in the MEN 
      1 course (mean age = 51 years). Magnetic resonance imaging/computed tomography 
      were more sensitive than octreoscanning. Their diagnosis averaged 18 years after 
      the onset of hyperparathyroidism, 10-15 years after pituitary disease or PETs. 
      Meningiomas were 11 times more frequent in patients with PETs with MEN 1 than 
      without MEN 1 (P = 0.017). No clinical, laboratory, or MEN 1 feature 
      distinguished patients with meningiomas. Meningiomas were asymptomatic and 60% 
      showed no growth. A resected meningioma showed loss of heterozygosity at 11q13 
      and 1p, including at p73 and ARHI/NOEY2 locus, but not at the neurofibromatosis 2 
      gene locus. CONCLUSIONS: These results show meningiomas are not an infrequent 
      occurrence in MEN 1, and loss of the function of the MEN1 gene product plays a 
      role in their pathogenesis in these patients.
FAU - Asgharian, Behnam
AU  - Asgharian B
AD  - Digestive Diseases Branch, National Institute of Diabetes and Digestive and 
      Kidney Diseases/NIH, Building 10, 10 Center Drive MSC 1804, Bethesda, MD 
      20892-1804, USA.
FAU - Chen, Yuan-Jia
AU  - Chen YJ
FAU - Patronas, Nicholas J
AU  - Patronas NJ
FAU - Peghini, Paolo L
AU  - Peghini PL
FAU - Reynolds, James C
AU  - Reynolds JC
FAU - Vortmeyer, Alexander
AU  - Vortmeyer A
FAU - Zhuang, Zhengping
AU  - Zhuang Z
FAU - Venzon, David J
AU  - Venzon DJ
FAU - Gibril, Fathia
AU  - Gibril F
FAU - Jensen, Robert T
AU  - Jensen RT
LA  - eng
PT  - Journal Article
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - Clin Cancer Res
JT  - Clinical cancer research : an official journal of the American Association for 
      Cancer Research
JID - 9502500
RN  - 0 (DNA-Binding Proteins)
RN  - 0 (Neurofibromin 2)
RN  - 0 (Nuclear Proteins)
RN  - 0 (TP73 protein, human)
RN  - 0 (Tumor Protein p73)
RN  - 0 (Tumor Suppressor Proteins)
SB  - IM
MH  - Adult
MH  - Alleles
MH  - Brain/pathology
MH  - Chromosomes/ultrastructure
MH  - Chromosomes, Human, Pair 11
MH  - Chromosomes, Human, Pair 22
MH  - DNA-Binding Proteins/genetics
MH  - Fathers
MH  - Female
MH  - Genes, Tumor Suppressor
MH  - Humans
MH  - Loss of Heterozygosity
MH  - Magnetic Resonance Imaging
MH  - Male
MH  - Meningioma/metabolism/*pathology
MH  - Microsatellite Repeats
MH  - Middle Aged
MH  - Mothers
MH  - Multiple Endocrine Neoplasia Type 1/*pathology
MH  - Neurofibromin 2/genetics
MH  - Nuclear Proteins/genetics
MH  - Pancreatic Neoplasms/*pathology
MH  - Polymerase Chain Reaction
MH  - Prospective Studies
MH  - Tomography, X-Ray Computed
MH  - Tumor Protein p73
MH  - Tumor Suppressor Proteins
MH  - Zollinger-Ellison Syndrome/genetics
EDAT- 2004/02/12 05:00
MHDA- 2004/09/29 05:00
CRDT- 2004/02/12 05:00
PHST- 2004/02/12 05:00 [pubmed]
PHST- 2004/09/29 05:00 [medline]
PHST- 2004/02/12 05:00 [entrez]
AID - 10.1158/1078-0432.ccr-0938-3 [doi]
PST - ppublish
SO  - Clin Cancer Res. 2004 Feb 1;10(3):869-80. doi: 10.1158/1078-0432.ccr-0938-3.