Published online Jan 7, 2022. doi: 10.12998/wjcc.v10.i1.296
Peer-review started: May 18, 2021
First decision: July 5, 2021
Revised: July 15, 2021
Accepted: November 30, 2021
Article in press: November 30, 2021
Published online: January 7, 2022
Processing time: 225 Days and 22.9 Hours
Primary intracranial alveolar soft-part sarcoma (PIASPS) is a rare malignancy. We aimed to investigate the clinical profiles and outcomes for PIASPS.
We firstly reported five consecutive cases from our institute. Then, the cases from previous studies were pooled and analyzed to delineate the characteristics of this disease. Our cohort included two males and three females. The median age was 21-years-old (range: 8-54-years-old). All the patients received surgical treatment. Gross total resection (GTR), radiotherapy, and chemotherapy were administered in 3 patients, 4 patients, and 1 patient, respectively. After a median follow-up of 36 mo, tumor progression was noticed in 4 patients; and 3 patients died of the disease. Pooled data (n = 14) contained 5 males and 9 females with a median age of 19 years. The log-rank tests showed that GTR (P = 0.011) could prolong progression-free survival, and radiotherapy (P < 0.001) resulted in longer overall survival.
Patients with PIASPS suffer from poor outcomes. Surgical treatment is the first choice, and GTR should be achieved when the tumor is feasible. Patients with PIASPS benefit from radiotherapy, which should be considered as a part of treatment therapies.
Core Tip: Alveolar soft part sarcoma, or alveolar soft-part sarcoma (ASPS), is reportedly a rare tumor with a high recurrent rate. Current understanding of primary intracranial ASPS, or primary intracranial ASPS (PIASPS), is limited to case reports or small case series that preclude statistical analysis of outcomes (e.g., progression-free survival or overall survival). In this article, we first reported 5 consecutive cases from our institute. Then, the 9 cases from previous studies were pooled and analyzed to delineate the characteristics of this disease. We found PIASPS was a rare malignancy and predominately affected young females. Patients with PIASPS suffer from poor outcomes, characterized by a high tendency of recurrence. Surgical treatment is the first choice and gross total resection should be achieved when the tumor is feasible. Besides, PIASPS benefit from radiotherapy, which should be considered as a part of treatment therapies.