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Risk stratification and outcomes in 210 gynecologic perivascular epithelioid cell tumors (PEComas) cases

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Abstract

Objective

To better understand the risk stratification and outcomes of gynecologic PEComas.

Methods

Clinicopathological features and outcomes of gynecologic PEComas cases reported in both English and Chinese literature before September, 2020 were evaluated. The efficacy of three proposed criteria were compared to verify their practicability in gynecologic PEComas. The Chi-square test and Cox proportional hazard model were performed for statistical analysis.

Results

A total of 210 cases were retrieved: 95 from English literature and 115 from Chinese literature. The Flope criterion achieved an accuracy of 47% for detecting malignancy of gynecologic PEComas, 64.2% for the Schoolmeester criterion, and 63.8% for the WHO criterion. Both Chi-square test and uni-variate analysis showed that tumor size ≥ 5 cm, infiltrative growth pattern, mitotic rate ≥ 1/50 high per filed (HPF), high nuclear grade and cellularity, necrosis, and vascular invasion were significantly related to recurrence and/or metastasis (R/M) of gynecologic PEComas. Still only high mitotic rate (≥ 1/50 HPF), high nuclear grade and cellularity, and necrosis significantly influenced the long-term survival. Multi-variate analysis showed high nuclear grade and cellularity was an independent risk factor for R/M of gynecologic PEComas. No model was fitted for the death rate due to a small number of events. When defined malignant PEComas cases as meeting three or more out of six clinicopathologic features, the accuracy of such attempt was 62%, but the false-negative rate dropped by 37–55%.

Conclusions

Gynecologic PEComas with three or more high-risk factors may be considered as malignant. Further efforts should be invested to look for new potential prognostic factors.

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Data availability

The authors confirm that the data supporting the findings of this study within the article are available within this article and supplementary materials.

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Funding

This work was supported by Hunan Provincial Key Research and Development Project, China (2020SK2073).

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Author notes

  1. Yanping Jiang

    Present address: Department of Physiology, National University of Ireland, Galway, 999015, Ireland

Authors and Affiliations

  1. Department of Gynecology, Xiangya Hospital of Central South University, Hunan Province, Xiangya Road, Kaifu District, Changsha, China

    Yanping Jiang, Xiaoqin Liu, Qian Wang, Qian Xu & Lanqin Cao

  2. Scientific Research Center, Xiangya Hospital of Central South University, Changsha, 410008, China

    Sai Zhang

  3. Department of Physiology, National University of Ireland, Galway, 999015, Ireland

    Kulsoom Ghias

Authors
  1. Yanping Jiang
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  2. Xiaoqin Liu
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Contributions

Great thanks for all authors’ contribution to this paper. YJ: statistical analysis and drafting; XL: data acquisition; QX: data acquisition. SZ: instruction for statistics. QW: critical revision; KG: critical revision; LC: study design and supervision. All authors commented on previous version of the manuscript. All authors read and approved the final manuscript.

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Correspondence to Lanqin Cao.

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Jiang, Y., Liu, X., Zhang, S. et al. Risk stratification and outcomes in 210 gynecologic perivascular epithelioid cell tumors (PEComas) cases. Arch Gynecol Obstet 307, 681–687 (2023). https://doi.org/10.1007/s00404-022-06470-y

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  • DOI: https://doi.org/10.1007/s00404-022-06470-y

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