MPNST are aggressive sarcomas that present challenges in diagnosis and treatment. In our series, NF1-related MPNST patients had the highest OS, likely associated with close monitoring for MPNST among the high-risk NF1-population. Nonextremity tumor locations, recurrent tumors, and postoperative complications were associated with inferior OS and PFS. Multi-institutional studies are warranted to investigate the impact of these prognostic factors in a larger, more heterogeneous MPNST patient cohort and examine the utility of surveillance in the neurofibromatosis patient population under a multidisciplinary team.
Horowitz et al. (2026) studied this question.