Adhesion molecule L1 is down-regulated in malignant peripheral nerve sheath tumors versus benign neurofibromatosis type 1-associated tumors
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Adhesion molecule L1 is down-regulated in malignant peripheral nerve sheath tumors versus benign neurofibromatosis type 1-associated tumors. / Blessmann, Marco; Gröbe, Alexander; Quaas, Alexander; Kaifi, Jussuf T; Mistakidis, Georgios; Bernreuther, Christian; Sauter, Guido; Gros, Stephanie; Rawnaq, Tamina; Friedrich, Reinhard; Mautner, Victor F; Smeets, Ralf; Heiland, Max; Schachner, Melitta; Izbicki, Jakob R.
In: OR SURG OR MED OR PA, Vol. 113, No. 2, 01.02.2012, p. 239-44.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Adhesion molecule L1 is down-regulated in malignant peripheral nerve sheath tumors versus benign neurofibromatosis type 1-associated tumors
AU - Blessmann, Marco
AU - Gröbe, Alexander
AU - Quaas, Alexander
AU - Kaifi, Jussuf T
AU - Mistakidis, Georgios
AU - Bernreuther, Christian
AU - Sauter, Guido
AU - Gros, Stephanie
AU - Rawnaq, Tamina
AU - Friedrich, Reinhard
AU - Mautner, Victor F
AU - Smeets, Ralf
AU - Heiland, Max
AU - Schachner, Melitta
AU - Izbicki, Jakob R
N1 - Copyright © 2012 Elsevier Inc. All rights reserved.
PY - 2012/2/1
Y1 - 2012/2/1
N2 - 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.
AB - 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.
KW - Adolescent
KW - Adult
KW - Aged
KW - Cell Transformation, Neoplastic
KW - Child
KW - Child, Preschool
KW - Female
KW - Gene Expression Regulation, Neoplastic
KW - Humans
KW - Immunohistochemistry
KW - Infant
KW - Male
KW - Mast Cells
KW - Middle Aged
KW - Nerve Sheath Neoplasms
KW - Neural Cell Adhesion Molecule L1
KW - Neurofibroma
KW - Neurofibromatosis 1
KW - Retrospective Studies
KW - Young Adult
U2 - 10.1016/j.tripleo.2011.04.019
DO - 10.1016/j.tripleo.2011.04.019
M3 - SCORING: Journal article
C2 - 22677742
VL - 113
SP - 239
EP - 244
JO - OR SURG OR MED OR PA
JF - OR SURG OR MED OR PA
SN - 2212-4403
IS - 2
ER -