Differences between neurofibromatosis-1-associated malignant peripheral nerve sheath tumors (MPNST) and sporadic MPNST: The Mayo Clinic experience.

Abstract

10066 Background: Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disorder associated with several clinical manifestations, including peripheral neurofibromas. Malignant transformation of these neurofibromas into malignant peripheral nerve sheath tumors (MPNST) is a potential life-threatening sequelae of this syndrome. Methods: A retrospective review of 57 patients with NF1-associated MPNST from 1985-2010 was performed. These patients were compared to 118 patients from the same time period diagnosed with MPNST not associated with NF1 to identify differences in presentations and outcomes. Results: Patients with NF1-associated MPNST were significantly younger at diagnosis than those that were not (39.9 years ±13.7 vs 47.8 years ±17.7, p=<0.01). NF1-associated tumors were also significantly larger (9.89 cm ±6.47 vs. 6.57 cm ±5.55, p=<0.01). No significant difference in tumor grade, depth, location, or recurrence (either local or distant) was found between the two gropus. Furthermore, there we...