Peripheral nerve tumors: management strategies and molecular insights

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.