Summary Malignant peripheral nerve sheath tumor (MPNST) is a rare malignant tumor that develops in soft tissues and MPNST with perineurial cell differentiation (perineurial MPNST) is a subtype of this tumor. This case report describes a 24-year-old Japanese male with perineurial MPNST arising at the root of the tongue. The tumor was diagnosed by immunohistochemical and ultrastructural techniques, and was treated by partial glossectomy. No signs of recurrence or metastasis have been observed for more than 2 years postoperatively. As far as we know, this is the first case report of perineurial MPNST arising in the oral cavity.

Perineurial MPNST arising from the tongue is a rare and potentially treatable malignancy, with no recurrence or metastasis observed after more than 2 years postoperatively.

Malignant peripheral nerve sheath tumor with perineurial cell differentiation arising from the tongue

Key Takeaway: Perineurial MPNST arising from the tongue is a rare and potentially treatable malignancy, with no recurrence or metastasis observed after more than 2 years postoperatively.