Back to Search
Start Over
Adhesion molecule L1 is down-regulated in malignant peripheral nerve sheath tumors versus benign neurofibromatosis type 1-associated tumors
- Source :
- Oral surgery, oral medicine, oral pathology and oral radiology. 113(2)
- Publication Year :
- 2011
-
Abstract
- Type 1 neurofibromatosis (NF-1), also known as von Recklinghausen disease, is caused by a disorder of a single gene on chromosome 17 that usually restrains cell division. A sequence that is frequently associated with NF-1 is tumor progression from neurofibromas to malignant peripheral nerve sheath tumors (MPNSTs). The aim of this study was to determine the expression of the neural L1 cell adhesion molecule in dermal-diffuse neurofibromas, plexiform neurofibromas, and MPNSTs of NF-1. We retrospectively analyzed surgically resected primary tumors, including 20 dermal neurofibromas, 23 plexiform neurofibromas, and 17 MPNSTs, by immunohistochemistry in paraffin sections of NF-1 tumors with the use of the L1-specific monoclonal antibody UJ127, which does not cross-react with other members of the L1 family. Immunostainings for CD34 and S100 were included to distinguish and allocate L1-expressing Schwann cells and perineural (specialized) fibroblasts. Our data showed that L1 is highly expressed in all benign NF-1 tumors and in some but not all MPNSTs. Furthermore, we demonstrated a correlation between L1 expression and differentiation grade of MPNSTs. There was a significant trend toward lower or nondetectable expression in the poorly differentiated MPNSTs, in contrast to all other tumor entities so far investigated, in which L1 expression correlated positive with malignancy, except for juvenile but not adult-derived neuroblastomas. Future studies are warranted to elucidate the molecular basis of the varying effects of the degree of L1 expression, receptor, and signal transduction mechanisms in different tumors.
- Subjects :
- Adult
Male
Pathology
medicine.medical_specialty
Neurofibromatosis 1
Adolescent
CD34
Neural Cell Adhesion Molecule L1
Malignancy
Nerve Sheath Neoplasms
Pathology and Forensic Medicine
Young Adult
Plexiform neurofibroma
medicine
Humans
Radiology, Nuclear Medicine and imaging
Dentistry (miscellaneous)
Mast Cells
Neurofibromatosis
Child
Aged
Retrospective Studies
Neurofibroma
biology
business.industry
Infant
Middle Aged
medicine.disease
Neurofibromin 1
Immunohistochemistry
Chromosome 17 (human)
Gene Expression Regulation, Neoplastic
Cell Transformation, Neoplastic
Tumor progression
Child, Preschool
biology.protein
Surgery
Female
Oral Surgery
business
Subjects
Details
- ISSN :
- 22124411
- Volume :
- 113
- Issue :
- 2
- Database :
- OpenAIRE
- Journal :
- Oral surgery, oral medicine, oral pathology and oral radiology
- Accession number :
- edsair.doi.dedup.....2af649578271d4fb6da9ac0b35077014