Primary alveolar soft part sarcoma arising from the cerebellopontine angle

Area of Science:

• Neuro-oncology
• Pathology

Background:

• Alveolar soft part sarcoma (ASPS) is a rare soft tissue malignancy.
• ASPS commonly metastasizes to the brain, but primary intracranial origin is exceptionally rare.

Observation:

• A case of primary intracranial ASPS in the cerebellopontine angle (CPA) in an 11-year-old girl is presented.
• The patient underwent multiple treatments including surgery, radiation, chemotherapy, and gamma knife surgery for recurrent tumors.
• Despite aggressive treatment, multifocal intracranial lesions recurred, with no evidence of extracranial metastasis.

Findings:

• Pathological diagnosis confirmed ASPS arising primarily within the cranial cavity.
• The intracranial ASPS demonstrated aggressive behavior with rapid recurrence and dissemination within the central nervous system.
• Extracranial metastasis was notably absent throughout the clinical course.

Implications:

• Primary intracranial ASPS, though rare, presents a significant challenge due to its aggressive nature.
• The findings suggest a poor prognosis for primary intracranial ASPS, mirroring outcomes seen in extracranial ASPS.
• This case underscores the need for further research into effective therapeutic strategies for this rare and aggressive tumor.