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Peripheral primitive neuroectodermal tumor of the cerebellopontine angle
Isam Alobid1, Felix Gastón, Antonio Morello
1Department of Otorhinolaryngology, Hospital Clínic, Barcelona, Spain. 3287iao@comb.es
Acta Oto-Laryngologica
|April 13, 2005
Summary
Peripheral primitive neuroectodermal tumors (pNETs) are aggressive neoplasms. This case highlights successful multimodal treatment for a rare pNET invading the internal auditory canal, achieving long-term remission.
Area of Science:
- Neuro-oncology
- Surgical Pathology
Background:
- Peripheral primitive neuroectodermal tumors (pNETs) are rare, highly malignant small cell neoplasms.
- These tumors can occur in various locations, including the head and neck region.
Observation:
- A 27-year-old female presented with right-sided facial pain, progressive facial muscle weakness, tinnitus, hearing loss, and facial palsy.
- T2-weighted MRI revealed a lesion invading the right internal auditory canal.
Findings:
- Pathological examination confirmed sheets of small cells with irregular nuclei.
- Immunohistochemistry showed positivity for neuron-specific enolase, synaptophysin, chromogranin, vimentin, S-100 protein, and MIC-2 gene product.
- The patient underwent surgical removal, chemotherapy, and radiotherapy, with initial success.
Implications:
- Despite initial treatment, the patient developed cervical metastasis, necessitating further surgery and chemotherapy.
- Long-term follow-up demonstrated no local recurrence or distant metastasis, suggesting the efficacy of multimodal therapy for pNETs in this location.