Abstract
BACKGROUND AND OBJECTIVE: Alveolar soft part sarcoma is a rare malignancy of the lower extremities considered to be of unknown cellular origin. Common in young female patients, the ASPS usually has a strong metastatic potential, mostly involving the lungs at the time of presentation. Cases involving the CNS primarily, without any evidence of systemic disease have emerged i.e. the primary intracranial alveolar soft part sarcoma (PIASPS). We aim to determine the clinical manifestations, natural progression and management of primary intracranial alveolar soft part sarcoma. MATERIALS AND METHODS: After an extensive literature search using PubMed Central and Google Scholar, seven case reports and two case series of 14 patients with PIASPS were retrieved and included using the Preferred Reporting Items for Systemic review and Meta-analyses guidelines, published between 2000 and 2023. RESULTS: Female preponderance of the neoplasm (57.14%) with an average age of 24.9 years was noted. Headache (36.36%) and papilledema (25%) were the most common presenting complaint and sign, respectively. Brain parenchymal involvement was noted in 71.42% cases, mostly the frontal lobe and left hemisphere. The lesion appeared isointense to hypointense on T1-weighted images and hyperintense on T2-weighted images (66.67%) on MRI. Gross Total Resection was possible in 57.14% lesions. On immunohistochemistry, the tumor cells manifested TFE3 and PAS positivity in 25% and 20% of the cases respectively. An average survival after follow up duration was found to be 31.79 months. CONCLUSION: Surgery and radiotherapy are the mainstay of treatment of PIASPS, while meningioma an important differential diagnosis. TFE3 fusion product is readily been helpful in diagnosis. Evidence of a primary lesion in the CNS cannot rule out the presence of an occult primary elsewhere in the body. This, along with the slow growing nature of the neoplasm and delayed localized recurrences, require a long-term follow-up.