title: Peripheral primitive neuroectodermal tumor of the cerebellopontine angle.
abstract: 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.
authors.affiliation: Department of Otorhinolaryngology, Hospital Clínic, Barcelona, Spain. 3287iao@comb.es
authors.forename: Isam
authors.initials: I
authors.lastname: Alobid
authors.forename: Felix
authors.initials: F
authors.lastname: Gastón
authors.forename: Antonio
authors.initials: A
authors.lastname: Morello
authors.forename: Jose María
authors.initials: JM
authors.lastname: Guilemany
authors.forename: Joaquim
authors.initials: J
authors.lastname: Mullol
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis; D002528:Cerebellar Neoplasms / Q000473:pathology* / Q000628:therapy; D002530:Cerebellopontine Angle / Q000473:pathology* / Q000601:surgery; D007758:Ear, Inner / Q000473:pathology / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006258:Head and Neck Neoplasms / Q000473:pathology / Q000628:therapy; D006801:Humans; D008279:Magnetic Resonance Imaging; D009361:Neoplasm Invasiveness; D016609:Neoplasms, Second Primary / Q000473:pathology / Q000628:therapy; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000473:pathology* / Q000601:surgery / Q000628:therapy; D019233:Retreatment
chemical_list: D014408:Biomarkers, Tumor