Enrico Martin

36 Chapter 2 Figure 1 Kaplan-Meier curves per tumor site (A) overall survival (B) disease-specific survival Overall and disease-specific survival Patients with intracranial tumors showed superior overall survival followed by limbs, head and neck, and spine. Tumors arising from the core had the worse overall survival (overall difference between curves p<0.001, Figure 1A ) . Differences across sites in disease-specific survival seem to be similar to overall survival, but disparities between limbs, head and neck, and spine were less obvious (overall difference between curves p<0.001, Figure 1B ). Discussion Using the SEER database we identified that the site of origin is an independent prognostic factor for survival in MPNSTs. Intracranial tumors tend to have a better survival than those arising in extremities. Tumors arising from core sites are associated with the poorest survival; head and neck tumors were also associated with worse survival compared to limb sites. Pediatric cases were significantly associated with better survival compared to adult cases independent from tumor site, size, and treatment modality. Other factors associated with worse survival were older age, male gender, black race, higher tumor grade and large tumors. Treatment modalities appear to vary slightly across site of origin. Intracranial MPNST Literature on intracranial MPNSTs is scarce, consisting only of multiple case reports, small case series, and some systematic reviews. This analysis presents the largest group of intracranial MPNSTs reported in literature to date. Patients with intracranial MPNSTs are believed to have a short survival. 27–30 1-year survival has been reported to be as little as 33%, while others found a 3-year overall survival of 64.0%. 27,28,31 The