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Peripheral
Nerve Tumors: Management Strategies and Molecular Insights
Asis Kumar Bhattacharyya, Richard Perrin, Abhijit Guha
Division
of Neurosurgery, Toronto Western Hospital, University Health Network,
Canada [A.K.B., R.P.]. Professor, Department of Surgery; Alan and Susan Hudson
Chair in Neurooncology, Toronto Western Hospital, University Health
Network, Canada; Arthur and Sonia Labatts Brain Tumor Centre, Hospital
for Sick Children's Research Institute, University of Toronto, Canada [A.G.].
Because
of its relative rarity and a wide variety of clinical manifestations,
peripheral nerve tumors (PNTs) often present to specialists from
widely different disciplines, thus often resulting in delayed
diagnosis and a non-cohesive pattern of management.
Critical appraisal of the history and physical examination followed by
radiological investigations, by experienced medical personnel,
ultimately suggests that the extremity mass is perhaps a PNT, rather
than the wide variety and more common soft tissue tumors.
Included in this appraisal is a search for a pre-disposition syndrome,
the most common of which are neurofibromatosis-1 and -2 (NF1, NF2) and
schwannomatosis, which may require life-long careful follow up.
Intra-operative and post-operative management decisions in a
multidisciplinary manner with knowledge of the biological,
pathological and clinical behavior of the PNT, is of utmost
importance.
In the context of pre-disposition syndromes, where multiple tumors may
exist along with other nervous system lesions, molecular biological
insights and hopefully the biological therapies that stem from this
knowledge are of interest.
In this article the spectrum of PNTs and their management protocols,
including the algorithm for treatment of malignant PNTs advocated by
our institute, are presented and currently available genetic insights
and probable role of experimental therapies are briefly reviewed.
Keywords: experimental
therapies, malignant peripheral nerve sheath tumor (MPNST),
neurofibromatosis (NF), peripheral nerve tumor (PNT), signaling
Copyright
©
2004 Kluwer Academic Publishers.
All rights reserved
Source: http://ipsapp007.kluweronline.com/IPS/content/ext/x/J/5042/I/124/A/24/abstract.htm
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