Malignant peripheral nerve sheath tumor of the oral mucosa: Case report and literature review

Background

Malignant peripheral nerve sheath tumor (MPNST) is an uncommon tumor in the head and neck region that develops either from a preexisting neurofibroma or de novo.

Observation

We report here a case of MPNST of the buccal mucosa in a 40 years old female whose chief complaint was a recurrent painful nodule. The diagnosis is considered as the most elusive and difficult among soft tissue sarcomas because of its non specific presentation, both clinically and pathologically.

Conclusion

This clinical case illustrates the challenge of delivering a pertinent diagnosis of MPNST due to its non-specific presentation both clinically and histopathologically. It also emphasizes the importance of a high level of vigilance when assessing painful mucosal lesions of normal aspect.