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Peripheral primitive neuroectodermal tumor of the cerebellopontine angle

jys527683426 添加于 2017/5/21 9:34:22  178次阅读 | 0次推荐 | 0个评论

Peripheral primitive neuroectodermal tumors are highly malignant small cell neoplasms. A 27-year-old female presented with a 6-month history of right-sided facial pain and progressive weakness of the facial muscles. She had non-pulsative tinnitus, progressive right hearing loss and facial palsy. T2-weighted MRI showed a heterogeneous hyperintense lesion invading the right internal auditory canal. Surgical removal was performed. Pathological examination showed sheets of small cells with irregular nuclei. Immunohistochemical studies demonstrated positive immunoreactivity for neuron-specific enolase, synaptophysin, chromogranin, vimentin, S-100 protein and p30-32 MIC-2 gene product. The patient was treated with chemotherapy (etoposide, vincristine, adriamycin, ifosfamide and actinomycin D) and radiotherapy. After 65 months of follow-up, the patient presented with cervical metastasis. Radical cervical dissection was performed and the patient was treated with a second course of chemotherapy. At control MRI after 29 months of follow-up the patient showed no signs of local recurrence or distant metastasis.

作 者:Isam Alobid, Felix Gastón, Antonio Morello, Jose María Guilemany & Joaquim Mullol
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学科领域:医学科学 » 神经系统和精神疾病 » 神经症和应激相关障碍
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原文链接:http://www.tandfonline.com/doi/abs/10.1080/00016480410025234
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