Facial malignant peripheral nerve sheath tumors

Area of Science:

• Oncology
• Radiology
• Pathology

Background:

• Malignant peripheral nerve sheath tumors (MPNSTs) are rare and infrequently involve the maxillo-mandibular region.
• This study presents five new cases with CT and MR findings and reviews existing literature.

Observation:

• Radiological criteria for MPNSTs are often nonspecific, ranging from well-delineated masses to erosive patterns.
• Key indicators for MPNSTs include mandibular nerve involvement, peripheral enhancement, and absence of a central dot sign.
• Malignancy is suggested by irregular bone destruction and poorly defined margins with muscular infiltration.

Findings:

• Histological diagnosis of MPNSTs is challenging due to varied morphology.
• Suggestive histological features include spindle cell arrangement (curlicue/whorled) and alternating cellular zones.
• Focal, limited S-100 protein immunostaining is a crucial diagnostic criterion.

Implications:

• Accurate early diagnosis of MPNSTs remains difficult, necessitating a multidisciplinary approach.
• Correlation of clinical, radiological, and pathological data is vital for suspecting MPNSTs.
• MPNSTs have a poor prognosis, underscoring the need for prompt and appropriate treatment.