The majority of tumors were benign PNSTs from the brachial plexus region. Most of the benign PNSTs in all locations were neurofibromas, with sporadic neurofibromas predominating. Similar numbers of schwannomas were found in the upper and lower extremities, whereas neurofibromas were more prevalent in the upper extremities. Despite aggressive limb-ablation or limb-sparing surgery plus adjunctive therapy, malignant PNSTs continue to be associated with high morbidity and mortality rates.
Over a 22-year period, operations were performed on 263 patients for 288 primary benign tumors of major peripheral nerves. The tumors included 85 schwannomas, 197 neurofibromas, and six plexiform neurofibromas. Total removal was achieved in 83 of the 85 schwannomas, and 76 of these patients were available for follow-up evaluation. Motor function either improved or was unchanged in 87% of these patients and 85% of those with pain in the distribution of the involved nerve had either total or partial resolution of their symptoms. Of the neurofibromas, 123 occurred in 121 patients without von Recklinghausen's disease. All tumors within this group were completely excised using a fascicular approach to the tumor. Of the 99 patients available for follow-up evaluation, 90% had either improved or unchanged motor function and 88% had partial or complete resolution of pain syndromes. Fifty-nine patients with von Recklinghausen's disease had 80 tumors removed: 74 fusiform tumors (58 of which were completely removed) and six plexiform tumors. Forty-eight of the 58 patients with gross total removal of fusiform tumors were available for follow-up evaluation, of whom 83% had improved or unchanged motor function and 74% had partial or complete resolution of pain syndromes. All six patients with plexiform tumors had progression of symptoms postoperatively. One brachial plexus schwannoma recurred and was re-excised without subsequent recurrence at the 5-year follow-up evaluation. Several incompletely excised plexiform neurofibromas have recurred with a symptomatic presentation.
The clinical presentation, electrical studies, imaging characteristics, and operative observations regarding peroneal intraneural ganglia are predictable. Treatment must address the underlying pathoanatomy and should include decompression of the cyst and ligation of the articular branch of the nerve. To avoid extraneural recurrence, resection of the superior tibiofibular joint may also be necessary, but indications for this additional procedure need to be defined. These recommendations are based on the authors' belief that intraneural peroneal ganglia arise from the superior tibiofibular joint and are connected to it by the articular branch.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.