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Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models

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Facial malignant peripheral nerve sheath tumors.

N Martin-Duverneuil¹, M Auriol, A Behin

¹Service de Neuroradiologie, GH Pitié-Salpêtrière, 47 Bd de l'Hôpital, 75013 Paris. nadine.martin-duverneuil@psl.aphp.fr

Journal of Neuroradiology = Journal De Neuroradiologie

|October 17, 2006

Summary

Malignant peripheral nerve sheath tumors (MPNSTs) in the jaw are rare and difficult to diagnose. Early diagnosis requires correlating clinical, imaging, and pathological findings for timely treatment.

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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.