J Korean Orthop Assoc.  2024 Feb;59(1):15-21. 10.4055/jkoa.2024.59.1.15 .

Alveolar Soft Part Sarcoma of the Extremities or Trunk: Clinical Manifestation and Treatment Results

• Cho SH
• Kong CB
• Jeon DG
• Kim HW
• Song WS

• KMID: 2553344
• DOI: http://doi.org/10.4055/jkoa.2024.59.1.15

Abstract

Purpose
The purpose of this study was to evaluate the clinical characteristics and treatment outcomes of alveolar soft part sarcoma (ASPS) of the extremities and trunk and investigate the prognostic factors.
Materials and Methods
Forty patients presenting with ASPS in the extremities or trunk between 2001 and 2021 were reviewed retrospectively. We evaluated the clinical manifestations at presentation, local recurrence, and metastasis after treatment. The survival rates and prognostic factors affecting survival were analyzed. Only patients who were followed up for more than one year were included in the study. The mean follow-up time was 58 months (range, 12–120).
Results
The average age of the patients was 26 years (range, 12–54) and the primary tumor developed mainly in the thigh (23 patients, 57.5%). The average size of the tumor was 8.7 cm and metastasis at presentation was seen in 28 (70%) patients. The overall survival rates at five-years and 10-years were 53.7% and 34.5%, respectively. Metastasis at presentation was related to poor survival (p=0.001).
Conclusion
ASPS in the extremities or trunk present with a high rate of metastasis and patients with metastasis showed poorer survival. In the future, further efforts to develop novel therapies like target therapy or immunotherapy for the treatment of metastatic patients are warranted.

Keyword

Sarcoma; alveolar soft part; metastasis; prognosis