Alveolar soft tissue sarcoma: a report of 50 cases at a single institution

Abstract

Background

Alveolar soft-part sarcoma (ASPS) is a rare soft tissue sarcoma subtype, occurring mainly in young people, with poor prognosis.

Materials and methods

We conducted a retrospective analysis of localized or metastatic ASPS patients admitted to the First Affiliated Hospital of Zhengzhou University (China) from 2012 to 2020, focusing on treatment and prognosis.

Results

The median age at diagnosis was 24 years (range: 1.4–78 years). Women (n = 29, 58%), especially those aged <30 years, dominated this series. The most common metastasis site was lung. Thirty-one (62%) patients developed lung metastasis (localized: n = 9 [18%]; metastatic: n = 22 [44%]). Only a tumor maximum diameter ≥ 5 cm was associated with a high lung metastasis rate (p = 0.039). The mean follow-up time was 37.5 months (1–108 months), and the 5-year overall survival (OS) rate was 84.7%. Univariate analysis indicated that distant metastasis observed at the initial visit and incomplete resection of the primary tumor were associated with poor OS. For localized cases, neither surgery plus radiotherapy (p = 0.486) nor surgery plus chemotherapy (p = 0.536) improved progression-free survival compared to surgery alone. Among the metastatic cases, the disease control rate (PR + SD) was higher for targeted therapy (60%) and combined immunosuppressive therapy (100%) than for conventional cytotoxic chemotherapy (26%).

Conclusions

Postoperative adjuvant radiotherapy and chemotherapy do not provide good local control for patients with localized disease. Although there is no standard treatment strategy for patients with advanced or metastatic disease, they can benefit from targeted therapy and/or immunosuppressive therapy.

Keywords:

• Alveolar soft tissue sarcoma
• clinical characteristics
• treatment methods
• prognostic factors

Acknowledgments

We thank Dr. Shenglei Li and Dr. Daoming Li (Zhengzhou University) for kindly providing help on review of the pathology slides.

Author contributions

All authors meet the authorship requirements. Pengyuan Zhao participated in the design of the study, drafted the manuscript and performed the experiments. Huixiang Li, Xueyan Zhao, Huifen Huang, Zhongqin Meng, Junna Kou, Na Wei, Mingfang Sun, Yuqiong Liu, Wencai Li analyzed the data and performed the statistical analysis. Hongyan Zhang and Yue Xu prepared the figures. Huayan Ren conceived and designed the study. All authors read and approved the final manuscript.

Disclosure statement

No potential conflict of interest was reported by the author(s).

Additional information

Funding

This study was funded by National Natural Science Foundation of China, grant [number 81803011]. It was also funded by The First Affiliated Hospital of Zhengzhou University.