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.

doi:10.1016/j.tripleo.2011.04.019

« Previous Next »Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology
Volume 113, Issue 2 , Pages 239-244, February 2012

Adhesion molecule L1 is down-regulated in malignant peripheral nerve sheath tumors versus benign neurofibromatosis type 1–associated tumors

Marco Blessmann, MD, DMD, PhD
- Department of Oral and Maxillofacial Surgery, University Medical Center Hamburg-Eppendorf, Germany
- Reprint requests: Marco Blessmannn, MD, DMD, Department of Oral and Maxillofacial Surgery, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246 Hamburg, Germany
Alexander Gröbe, MD, DMD
- Department of Oral and Maxillofacial Surgery, University Medical Center Hamburg-Eppendorf, Germany
Alexander Quaas, MD
- Institute for Pathology, University Medical Center Hamburg-Eppendorf, Germany
Jussuf T. Kaifi, MD, PhD
- Department of General, Visceral, and Thoracic Surgery, University Medical Center Hamburg-Eppendorf, Germany
Georgios Mistakidis, MD, DMD
- Department of Oral and Maxillofacial Surgery, University Medical Center Hamburg-Eppendorf, Germany
Christian Bernreuther, MD, PhD
- Department of Neuropathology, University Medical Center Hamburg-Eppendorf, Germany
Guido Sauter, MD, PhD
- Institute for Pathology, University Medical Center Hamburg-Eppendorf, Germany
Stephanie Gros, MD
- Department of General, Visceral, and Thoracic Surgery, University Medical Center Hamburg-Eppendorf, Germany
Tamina Rawnaq, MD
- Department of General, Visceral, and Thoracic Surgery, University Medical Center Hamburg-Eppendorf, Germany
Reinhard Friedrich, MD, DMD, PhD
- Department of Oral and Maxillofacial Surgery, University Medical Center Hamburg-Eppendorf, Germany
Victor F. Mautner, MD, PhD
- Department of Oral and Maxillofacial Surgery, University Medical Center Hamburg-Eppendorf, Germany
Ralf Smeets, MD, DMD, PhD
- Department of Oral and Maxillofacial Surgery, University Medical Center Hamburg-Eppendorf, Germany
Max Heiland, MD, DMD, PhD
- Department of Oral and Maxillofacial Surgery, University Medical Center Hamburg-Eppendorf, Germany
Melitta Schachner, ScD, PhD
- Center for Molecular Neurobiology, University Medical Center Hamburg-Eppendorf, Germany
- Center for Neuroscience, Shantou University Medical College, Shantou, China
- Melitta Schachner, ScD, PhD, Center for Neuroscience, Shantou University Medical College, 22 Xin Ling Road, Shantou 515041, P.R. China
Jakob R. Izbicki, MD, PhD
- Department of General, Visceral, and Thoracic Surgery, University Medical Center Hamburg-Eppendorf, Germany

Received 6 January 2011; received in revised form 14 April 2011; accepted 15 April 2011. published online 12 August 2011.

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.