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Five cases of uterine perivascular epithelioid cell tumors (PEComas) and review of literature

  • Weiwei Shan
  • Yue Shi
  • Qin Zhu
  • Bingyi Yang
  • Liying Xie
  • Bing Li
  • Chengcheng Ning
  • Qiaoying Lv
  • Yali Cheng
  • Bingying Xie
  • Mingzhu Bai
  • Yuhui Xu
  • Xiaojun Chen
  • Xuezhen Luo
Gynecologic Oncology
  • 12 Downloads

Abstract

Objectives

Perivascular epithelioid cell tumor (PEComa) is a rare condition and the recognition of this condition is limited. Here we report five cases of uterine PEComa to add to the limited understanding of this rare condition.

Methods

Five cases from Obstetrics and Gynecology Hospital of Fudan University were diagnosed as uterine PEComas. We collected the patients’ clinical and pathological data as well as their outcomes.

Results

All the five cases were diagnosed post-operationally. Fertility-sparing surgery was done for the first case and had a mass resection only. She delivered a healthy boy through the cesarean section in November 2016 and neither recurrence nor metastasis was found for 71 months. Hysterectomy was done for the other four cases. Adjuvant chemotherapy was also given for case 2 and case 4. Case 2 had combined endometrial cancer, which could be associated with tuberous sclerosis complex (TSC). She was followed up for 22 months and neither recurrence nor metastasis was detected. Neither recurrence nor metastasis was found in case 3 for 33 months. However, the patient in case 4 died of multiple dissemination and multiple organs failures, 10 months after the second surgery. The patient in case 5 had the hysterectomy and left adnexal resection and in this case we had no data about her long-term outcomes.

Conclusion

It is still challenging to detect and diagnose uterine PEComa clinically and no consensus or guidelines have been established regarding the treatment of this condition. More case studies are needed to enlighten the underlying mechanism and help optimize the therapies for this condition.

Keywords

PEComa Uterus Case reports 

Notes

Author contributions

WWS: manuscript writing/editing. XJC: supportive supervision. XZL: provided the source information and idea, manuscript editing, supervision. YS: language editing. QZ: pathology review. BYY, LYX, BL, CCN, QYLV, YLC, BYX, MZB, YHX: follow-up and searching medical records.

Compliance with ethical standards

Conflict of interest

All authors declare no conflict of interest.

Ethical approval

This report complies with the tenets of the Declaration of Helsinki, and the medical ethics committee of the Obstetrics and Gynecology Hospital of Fudan University approved this study. Informed consent was obtained from all the five patients in this report.

References

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of GynecologyObstetrics and Gynecology Hospital of Fudan UniversityShanghaiPeople’s Republic of China
  2. 2.Shanghai Key Laboratory of Female Reproductive Endocrine Related DiseasesShanghaiChina
  3. 3.Department of PathologyObstetrics and Gynecology Hospital of Fudan UniversityShanghaiPeople’s Republic of China

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