PMID- 12070601
OWN - NLM
STAT- MEDLINE
DCOM- 20020815
LR  - 20161124
IS  - 0945-6317 (Print)
IS  - 0945-6317 (Linking)
VI  - 440
IP  - 6
DP  - 2002 Jun
TI  - Immunohistochemical and molecular analysis of p53, RB, and PTEN in malignant 
      peripheral nerve sheath tumors.
PG  - 610-5
AB  - The molecular basis of both sporadic and neurofibromatosis type 1 
      (NF1)-associated malignant peripheral nerve sheath tumors (MPNSTs) is yet largely 
      undetermined. Therefore, we analyzed a series of 12 MPNSTs - including two cases 
      which arose in the setting of NF1 - for molecular alterations in the p53, 
      retinoblastoma ( Rb), and PTEN tumor suppressor genes. Furthermore, the 
      immunohistochemical expression of p53, RB, and PTEN protein was examined in these 
      tumors. One mutation (8%), an A to T transversion leading to an amino acid 
      exchange, was found in exon 5 of the p53 gene in a sporadic MPNST. In two other 
      sporadic tumors (20%), loss of heterozygosity (LOH) of the p53 gene occurred. 
      Nuclear overexpression of p53 protein was observed in ten tumors (83%). Loss of 
      RB protein expression was seen in two MPNSTs (17%), and LOH of the Rb gene was 
      detected in four tumors (44%), including the two NF1-associated MPNSTs, one of 
      them showing concomitant loss of RB protein expression. No mutation in the PTEN 
      gene was detected, and cytoplasmic immunoreactivity for the PTEN protein was 
      maintained in eight MPNSTs (67%). We suggest that alterations in the p53 and RB 
      pathway, both are essential in controlling the cell-cycle progression, are 
      critical points in the tumorigenesis of sporadic and NF1-associated MPNSTs, 
      whereas the PTEN gene seems to play no significant role in this process.
FAU - Mawrin, Christian
AU  - Mawrin C
AD  - Department of Neuropathology, Otto-von-Guericke-University, Magdeburg, Germany.
FAU - Kirches, Elmar
AU  - Kirches E
FAU - Boltze, Carsten
AU  - Boltze C
FAU - Dietzmann, Knut
AU  - Dietzmann K
FAU - Roessner, Albert
AU  - Roessner A
FAU - Schneider-Stock, Regine
AU  - Schneider-Stock R
LA  - eng
PT  - Journal Article
DEP - 20011116
PL  - Germany
TA  - Virchows Arch
JT  - Virchows Archiv : an international journal of pathology
JID - 9423843
RN  - 0 (Retinoblastoma Protein)
RN  - 0 (Tumor Suppressor Protein p53)
RN  - 0 (Tumor Suppressor Proteins)
RN  - EC 3.1.3.2 (Phosphoric Monoester Hydrolases)
RN  - EC 3.1.3.67 (PTEN Phosphohydrolase)
RN  - EC 3.1.3.67 (PTEN protein, human)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Female
MH  - Gene Expression Regulation, Neoplastic
MH  - Genes, Tumor Suppressor
MH  - Humans
MH  - Immunohistochemistry
MH  - Male
MH  - Middle Aged
MH  - Nerve Sheath Neoplasms/genetics/*metabolism
MH  - PTEN Phosphohydrolase
MH  - *Phosphoric Monoester Hydrolases/biosynthesis/genetics
MH  - *Retinoblastoma Protein/biosynthesis/genetics
MH  - *Tumor Suppressor Protein p53/biosynthesis/genetics
MH  - *Tumor Suppressor Proteins/biosynthesis/genetics
EDAT- 2002/06/19 10:00
MHDA- 2002/08/16 10:01
CRDT- 2002/06/19 10:00
PHST- 2001/05/31 00:00 [received]
PHST- 2001/09/05 00:00 [accepted]
PHST- 2002/06/19 10:00 [pubmed]
PHST- 2002/08/16 10:01 [medline]
PHST- 2002/06/19 10:00 [entrez]
AID - 10.1007/s00428-001-0550-4 [doi]
PST - ppublish
SO  - Virchows Arch. 2002 Jun;440(6):610-5. doi: 10.1007/s00428-001-0550-4. Epub 2001 
      Nov 16.