Transzygomatic, transcondylar excision of the sarcoma of infratemporal fossa

Malignant peripheral nerve sheath tumor (MPNST) is a rare pathologic lesion in a patient with solitary neurofibroma. A 32-year-old man presented with a firm and slightly tender mass in the right infratemporal region involving the right preauricular and temporomandibular joint area. The patient has a history of removal of a solitary neurofibroma 22 years back in the same region. The lesion had enlarged rapidly over the past 3 months, and a spindle cell lesion was diagnosed through a superficial incisional biopsy. Surgical removal of the lesion using modified preauricular transzygomatic approach was done. Histopathologically, it was diagnosed as an MPNST.