Malignant Peripheral Nerve Sheath Tumor of the Oral Cavity

Professor and Consultant, Department of Otolaryngology and Head and Neck Surgery, Army Hospital Research and Referral, Delhi Cantt, Delhi, India.
Journal of oral and maxillofacial surgery: official journal of the American Association of Oral and Maxillofacial Surgeons (Impact Factor: 1.43). 08/2012; 70(10):e581-5. DOI: 10.1016/j.joms.2012.05.027
Source: PubMed
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    ABSTRACT: We report the CT and MR findings in a patient with malignant peripheral nerve sheath tumor in the parapharyngeal space who had neither a family history nor stigmata of neurofibromatosis. A high-resolution CT scan of the temporal bone revealed bony erosion and widening of both the foramen ovale and the bony portion of the eustachian tube. A temporal bone MR image showed an intensely enhancing solid mass in the parapharyngeal space, which extended into the middle ear cavity via the eustachian tube.
    American Journal of Neuroradiology 05/2001; 22(4):748-50. · 3.59 Impact Factor
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    ABSTRACT: Magnetic resonance (MR) images of 16 peripheral nerve tumors (14 patients) were correlated with histopathologic appearance. Thirteen patients had surgically proved neuro-fibromatosis. There were 10 neurofibromas, four schwannomas, and two neurofibrosarcomas. Seven of the 10 neurofibromas showed a target pattern of increased peripheral signal intensity and decreased central signal intensity on T2-weighted images. This pattern appeared to correspond to a distinctive zonal histologic appearance that was found only in the neurofibromas. This pattern was not seen on MR images of the other peripheral tumors.
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    ABSTRACT: The incidence of malignant peripheral nerve sheath tumor (MPNST) is approximately 0.001%. Those involving intracranial nerves are even more exceptional. Little information is available concerning work up and management. Our objective is: (1) to review all cases of intracranial MPNST described in the literature, (2) to highlight the suspicion of intracranial MPNST, (3) to identify the gross pathology, the histopathology, the immunohistochemistry, (4) to discuss the differential diagnosis, the treatment, the recurrence rate, the follow-up, the incidence of metastasis and the prognosis. We reviewed English, Spanish and French literature published from 1950 to date. We used the following Keywords: "malignant peripheral nerve sheath tumor", "cranial nerve", "neurosarcoma", "malignant schwannoma", "neurofibroma", "malignant neurofibroma" and "nerve tumor". We considered cases where MPNST involved an intracranial cranial nerve. The results yielded 20 relevant studies, in which 31 patient's records were transcribed. We also added our case to this series. We identified 32 cases of cranial MPNST including our case. The age ranged from 5 to 75 years old with most patients being in the 5th and 6th decade. Male to female ratio is 2.5:1. Most cases are developed sporadically (50%), 31% arise from a malignant transformation of schwannoma and 19% from a neurofibroma. Imaging findings were not specific. The cranial nerve VIII is the most involved (15/32), followed by the Vth (10/32) and the VIIth (5/32). 4 cases had neurofibromatosis type 1 and 2 had neurofibromatosis type 2. MPNST will strongly express protein S-100 and collagen IV-laminin. 13 cases were treated with radiotherapy for tumor recurrence and metastasis. In these cases the survival rate was better than the cases without radiotherapy. Fatal outcome occurred in 66% of patients whereas 19% were reported alive with or without complications. The seven cases reported to have metastasis were entirely to the spine. The mean time of recurrence or metastasis is 12.2 months. MPNST of cranial nerves are very rare. In neurofibroma, even though MPNST is mainly associated to type 1, we should keep in mind its association to NF2. Mainstay of treatment is radical resection with adjuvant radiotherapy. Inaccessibility of cranial MPNST may explain the subtotal resection and thus the poor prognosis. Metastasis to the spinal cord is the most frequent one. A close postoperative follow-up is mandatory to eliminate recurrence.
    Auris, nasus, larynx 10/2010; 37(5):539-45. DOI:10.1016/j.anl.2010.02.009 · 1.14 Impact Factor