Facial malignant peripheral nerve sheath tumors

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) are rare neoplasms, only sporadically reported involving the maxillo-mandibular region (ten cases with CT or MR data). We present here five additional cases with CT and MR findings along with an extensive review of the literature. Accurate diagnosis of MPNSTs is difficult because pathological like radiological criteria are often non specific. Radiological features display a large spectrum of abnormalities from a well-delineated heterogeneous appearance simulating benign schwannoma to extensive erosive patterns. Their development along the mandibular nerve, the absence of any target or central dot sign, their strong predominant peripheral enhancement must suggest the diagnosis of MPNSTs while irregular bone destruction or the detection of poorly defined margins with muscular infiltration are the most reliable criteria of malignancy. Unfortunately, MPNSTs also display a considerably varied histology. Careful clinical and radiological correlation should bring pathologists to examining large samples of the lesion to better evaluate the overall organisation of the lesion and detect some evocative criteria often only present in some areas of the sample as the peculiar curlicue or whorled arrangement of the spindle cells or the alternation of densely cellular fascicles with hypocellular, myxoid zones. Focal, limited immunostaining for S-100 protein is one of the most important additional criterion. If accurate early diagnosis often remains difficult, careful correlation of clinical, pathological and radiological data should in most cases suggest a diagnosis of MPNSTs which display a poor prognosis and requires early and adapted treatment.