Enrico Martin

260 Chapter 11 Results Patient population A total of 756 patients were treated at the participating centers. The mean age was 44.3 years including 72 children ( Table 1 ). Fifty-three percent of the patients were male. NF1 patients comprised 33.4% of all patients. Most tumors were large (73.0% ≥5cm) and deep-seated (81.8%). MPNSTs most commonly occurred at extremity sites (37.8%) and 34.4% were known to originate from a major nerve. Patients presented in 13.4% of cases with synchronous metastases. A mass or spontaneous pain were the most common presenting symptoms, but 15.8% of patients presented with motor deficits and 13.4% with sensory deficits ( Table 1 ). Of patients with a neurologic deficit, 48.3% presented with both motor and sensory deficits. Function loss Postoperative motor deficits were present in 199 patients (34.7%), of which 157 patients (27.2%) were known to have a deficit with less than M3 muscle power of the nerve’s target muscles or an adjacently resected structure ( Table 2 ). Most patients that presented with motor deficits (58%) had persisting motor deficits with less than M3 muscle power after tumor resection ( Table 3 ). NF1 patients, larger, and deep-seated tumors were also associated with an increased risk of developing postoperative motor deficits (all p<0.001). Extremity tumors will develop postoperative motor deficits in 37.1% of cases, but brachial plexus tumors (57%) and pelvic tumors (55%) have the highest risk for persistent motor deficits (p<0.001). MPNSTs originating from major nerves more commonly had postoperative motor deficits (<0.001); incidence of motor deficits were 64.1% for those originating frommotor or mixed nerves. Surgical resection margins were associated with motor grade (p = 0.04). Sensory deficits were postoperatively present in 171 patients (54.6%). Almost half (44.4%) of these cases had at least partial sensory loss of the hand or feet. Patients presenting with sensory loss had persistent loss of critical sensation in 55%. NF1 patients, larger, and deep-seated tumors were associated with loss of critical sensation (all p<0.05). Extremity tumors will develop critical sensory loss in 32.0% of cases, but brachial plexus tumors (63%) and pelvic tumors (44%) were at highest risk for developing critical sensory loss (p<0.001). Peripheral nerve surgeons were more commonly involved in cases with motor and sensory deficits (p<0.001), but were not involved in 64.2% and 60.5% of cases respectively. Amputations were carried out in 61 patients at any point in time. Most amputations (56%) were carried out during initial surgery. For the upper extremity, forequarter amputations were most commonly performed; for the lower extremity, hemipelvectomies were most common. The use of prosthetics, orthoses, and walking aids were commonly unknown.

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