Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jan 7, 2022; 10(1): 296-303
Published online Jan 7, 2022. doi: 10.12998/wjcc.v10.i1.296
Clinical characteristics and outcomes of primary intracranial alveolar soft-part sarcoma: A case report
Jun-Yu Chen, Bo Cen, Fei Hu, Yong Qiu, Guo-Min Xiao, Jun-Ge Zhou, Fang-Cheng Zhang
Jun-Yu Chen, Bo Cen, Fei Hu, Yong Qiu, Guo-Min Xiao, Jun-Ge Zhou, Department of Neurosurgery, General Hospital of the Yangtze River Shipping, Wuhan 430010, Hubei Province, China
Fang-Cheng Zhang, Department of Neurosurgery, Wuhan Union Hospital, Wuhan 430010, Hubei Province, China
Author contributions: Chen JY contributed to data curation, formal analysis, and original draft; Chen JY and Zhang FC contributed to conceptualization and methodology; Chen JY, Cen B, Qiu Y, and Zhou JG provided resources; Chen JY, Qiu Y, Xiao GM, and Zhang FC wrote the paper; Cen B and Zhou JG generated the figures/table for data visualization; Cen B and Hu F supervised the study; Fei Hu and Xiao GM validated the findings; Qiu Y, Xiao GM, and Zhang FC reviewed and edited paper.
Informed consent statement: Informed consent was obtained from all individual participants included in the study.
Conflict-of-interest statement: Authors have nothing to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Fang-Cheng Zhang, MD, Professor, Department of Neurosurgery, Wuhan Union Hospital, No. 1277 Jiefang Avenue, Wuhan 430010, Hubei Province, China. zfcwhuh@gmail.com
Received: May 18, 2021
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
Abstract
BACKGROUND

Primary intracranial alveolar soft-part sarcoma (PIASPS) is a rare malignancy. We aimed to investigate the clinical profiles and outcomes for PIASPS.

CASE SUMMARY

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.

CONCLUSION

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.

Keywords: Primary intracranial alveolar soft-part sarcoma; Surgery; Radiotherapy; Progression-free survival; Overall survival; Case report

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.