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Peripheral primitive neuroectodermal tumor of the cerebellopontine angle.
Acta Otolaryngol. 2005 Apr; 125(4):426-9.AO

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+Show Affiliations

Department of Otorhinolaryngology, Hospital Clínic, Barcelona, Spain. 3287iao@comb.esNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

15823816

Citation

Alobid, Isam, et al. "Peripheral Primitive Neuroectodermal Tumor of the Cerebellopontine Angle." Acta Oto-laryngologica, vol. 125, no. 4, 2005, pp. 426-9.
Alobid I, Gastón F, Morello A, et al. Peripheral primitive neuroectodermal tumor of the cerebellopontine angle. Acta Otolaryngol. 2005;125(4):426-9.
Alobid, I., Gastón, F., Morello, A., Guilemany, J. M., & Mullol, J. (2005). Peripheral primitive neuroectodermal tumor of the cerebellopontine angle. Acta Oto-laryngologica, 125(4), 426-9.
Alobid I, et al. Peripheral Primitive Neuroectodermal Tumor of the Cerebellopontine Angle. Acta Otolaryngol. 2005;125(4):426-9. PubMed PMID: 15823816.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Peripheral primitive neuroectodermal tumor of the cerebellopontine angle. AU - Alobid,Isam, AU - Gastón,Felix, AU - Morello,Antonio, AU - Guilemany,Jose María, AU - Mullol,Joaquim, PY - 2005/4/13/pubmed PY - 2005/9/30/medline PY - 2005/4/13/entrez SP - 426 EP - 9 JF - Acta oto-laryngologica JO - Acta Otolaryngol. VL - 125 IS - 4 N2 - 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. SN - 0001-6489 UR - https://www.unboundmedicine.com/medline/citation/15823816/Peripheral_primitive_neuroectodermal_tumor_of_the_cerebellopontine_angle_ L2 - http://www.tandfonline.com/doi/full/10.1080/00016480410025234 DB - PRIME DP - Unbound Medicine ER -