NF1 and malignant peripheral nerve sheath tumor: These instances include a patient with NF-1 mutated MPNST achieving complete remission with pembrolizumab,[12] another showing significant improvement post-radiotherapy with the same agent,[10] a third case where pembrolizumab combined with procarbazine provided therapeutic benefits,[11] and a fourth illustrating a sustained response with nivolumab alongside radiotherapy.[13] These examples underscore the evolving understanding and potential therapeutic implications of immunotherapy in the management of MPNSTs despite the current absence of FDA approval for this specific indication.