Benign and Malignant Peripheral Nerve Sheath Tumors of the Oral Cavity: Two-Case Series Emphasizing Diagnostic Challenges

Background: Peripheral nerve sheath tumors of the oral cavity are rare and encompass both benign and malignant entities. Differentiating between these lesions remains challenging due to overlapping clinical and histopathological characteristics. Case presentation: We present two cases illustrating the biological spectrum of peripheral nerve sheath tumors in the oral cavity. The first case involves a 76-year-old male with a recurrent lower lip lesion initially diagnosed as benign, which progressed to a high-grade malignant peripheral nerve sheath tumor (MPNST). The second case describes a 20-year-old male presenting with a nodular lesion of the tongue, initially suspected to be reactive following trauma, but histologically confirmed as a benign schwannoma. Both patients underwent surgical treatment with favorable immediate postoperative outcomes. Conclusions: These cases highlight the diagnostic complexity and heterogeneous behavior of peripheral nerve sheath tumors. Histopathological and immunohistochemical evaluations are essential for definitive diagnosis. Clinicians should maintain a high index of suspicion and consider possible association with NF1 or schwannomatosis, particularly in recurrent or atypical lesions.