Neurofibromatosis 1 phenotype associated to malignant peripheral nerve sheath tumours: a case-control study.
Summary of "Neurofibromatosis 1 phenotype associated to malignant peripheral nerve sheath tumours: a case-control study."
Background  Malignant peripheral nerve sheath tumours (MPNSTs) are the main cause of death in neurofibromatosis 1 adult patients. Objectives  To determine the clinical type of neurofibromas associated to MPNSTs. Methods  Case-control study. Cases were neurofibromatosis 1 adults with MPNSTs and controls were patients without MPNSTs individually matched by age and sex (1 : 3). Both were recruited from our database. The following variables were studied: clinical presence of cutaneous, subcutaneous or plexiform neurofibromas and of internal neurofibromas. Internal neurofibromas were confirmed by clinical imaging. Multivariate odds ratios (aORs) were estimated with their 95% confidence interval (CI). Results  From January 1995 to December 2007, 52 patients (cases) were identified with a MPNSTs, 155 controls could be recruited. In the multivariate analysis, MPNSTs were significantly associated with the presence of internal NFs (a
OR:
7.5; 95%
CI:
3.2-17.4), a trend for an association was observed for the presence of subcutaneous neurofibromas (a
OR:
2.11; 95%
CI:
0.89-5). Conclusions  This study confirms the association between the MPNSTs and the internal neurofibromas. The later are indeed associated with a high risk of malignant transformation.
Affiliation
Department of Dermatology, Hôpital Henri-Mondor, APHP, Créteil, and Department of Dermatology, Centre Hospitalier de Colmar, Hôpital Pasteur, Colmar, France Department of Clinical Research and Public Health, Hôpital Henri-Mondor, APHP, Centre de refer
Journal Details
This article was published in the following journal.
Name: Journal of the European Academy of Dermatology and Venereology : JEADV
ISSN: 1468-3083
Pages:
Links
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/22348274
- DOI: http://dx.doi.org/10.1111/j.1468-3083.2012.04485.x
Medical and Biotech [MESH] Definitions
Nerve Sheath Neoplasms
Neoplasms which arise from nerve sheaths formed by SCHWANN CELLS in the PERIPHERAL NERVOUS SYSTEM or by OLIGODENDROCYTES in the CENTRAL NERVOUS SYSTEM. Malignant peripheral nerve sheath tumors, NEUROFIBROMA, and NEURILEMMOMA are relatively common tumors in this category.
Peripheral Nervous System Neoplasms
Neoplasms which arise from peripheral nerve tissue. This includes NEUROFIBROMAS; SCHWANNOMAS; GRANULAR CELL TUMORS; and malignant peripheral NERVE SHEATH NEOPLASMS. (From DeVita Jr et al., Cancer: Principles and Practice of Oncology, 5th ed, pp1750-1)
Myelin Sheath
The lipid-rich sheath surrounding AXONS in both the central and peripheral nervous systems. The myelin sheath is an electrical insulator and allows faster and more energetically efficient conduction of impulses. The sheath is formed by the cell membranes of glial cells (SCHWANN CELLS in the peripheral and OLIGODENDROGLIA in the central nervous system). Deterioration of the sheath in DEMYELINATING DISEASES is a serious clinical problem.
Nerve Fibers, Unmyelinated
A class of nerve fibers as defined by their structure, specifically the nerve sheath arrangement. The AXONS of the unmyelinated nerve fibers are small. The axons to SCHWANN CELLS ratio is greater in the unmyelinated nerve fibers than that in the myelinated fiber (NERVE FIBERS, MYELINATED) which is 1:1. Usually several axons are surrounded by a single Schwann cell in the unmyelinated nerve fibers. Therefore, each unmyelinated fiber is not completely covered by the MYELIN SHEATH formed by the Schwann cell. Unmyelinated nerve fibers conduct impulses at low velocities. They represent the majority of peripheral sensory and autonomic fibers. They are also found in the spinal cord and brain.
Nerve Compression Syndromes
Mechanical compression of nerves or nerve roots from internal or external causes. These may result in a conduction block to nerve impulses (due to MYELIN SHEATH dysfunction) or axonal loss. The nerve and nerve sheath injuries may be caused by ISCHEMIA; INFLAMMATION; or a direct mechanical effect.
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