Primary Intracranial Alveolar Soft-Part Sarcoma: Report of Two Cases and a Review of the Literature.

Background: Alveolar soft part sarcoma (ASPS), a rare malignant soft-tissue sarcoma affecting mainly adolescents and young children, frequently metastasizes to the brain. Primary intracranial ASPS, however, is extremely rare. We present 2 cases of primary intracranial ASPS without demonstrable systemic lesions.

Case Presentation: We report 2 cases of primary intracranial lesions that were surgically treated, and a postoperative diagnosis of ASPS was determined in both of the cases. The tumor in the 28-year-old female patient completely resolved after a treatment course consisting of surgical intervention and radiotherapy. After a follow-up period of 27 months, the patient was tumor-free. The other patient was a 13-year-old boy with a right middle cranial fossa tumor who experienced subtotal surgery, experienced a tumor relapse, and died 2 years after surgery.

Conclusions: This is the fifth report about primary intracranial ASPS. We herein present the clinical pathologic characteristics, imaging features, and differential diagnosis of primary ASPS of the brain. Gross total resection is the most effective therapeutic option for primary intracranial ASPS.