Perivascular epithelioid cell tumor of the uterus and pelvic cavity.

Front Oncol

Department of Obstetrics and Gynecology, West China Second University Hospital, Sichuan University, Chengdu, China.

Published: October 2024

AI Article Synopsis

  • Primary perivascular epithelioid cell tumors (PEComas) in the female reproductive tract are rare, with limited data on their risks and invasiveness, prompting a need for better understanding in diagnosis and treatment.
  • A study conducted on eight patients with PEComa saw varied outcomes, with some undergoing successful surgeries while others showed new lesions or nodules post-treatment.
  • Emphasizing complete lesion resection is crucial, as inadequate treatment raises recurrence and metastasis risks, highlighting the need for improved risk assessment strategies in managing PEComas.

Article Abstract

Background: Primary perivascular epithelioid cell tumors (PEComas) of the female reproductive tract have been primarily reported as case reports owing to their clinical rarity. Limited incidence rates and clinical case data hinder a comprehensive understanding of the risks and invasiveness of this disease. We discuss herein the diagnosis, treatment, and prognosis of this disease to enhance comprehension and therapeutic strategies.

Methods: We conducted a clinical analysis of patients with PEComa treated at the Gynecology Department of The West China Second University Hospital of Sichuan University between May 2018 and January 2024. Diagnosis and treatment were evaluated based on pertinent literature.

Results: Overall, eight patients (seven patients with tumors in the uterus and one patient with tumors in the pelvic cavity) were evaluated. One patient with PEComa of unknown malignant potential and two patients with malignant PEComa underwent hysterectomy and bilateral adnexectomy with or without adjuvant therapy and did not develop recurrence. Meanwhile, three patients who underwent lesion resection only exhibited radiological evidence of new lesions. Furthermore, postoperative imaging identified new pulmonary nodules in three patients.

Conclusion: Although the current criteria are generally effective in assessing the tumor invasiveness of PEComa, emphasizing the significance of complete lesion resection remains crucial. Inadequate treatment significantly increases the risks of recurrence and metastasis. Additionally, the prevalence of pulmonary metastases may have been underestimated. Refining risk stratification to prevent overtreatment of low-grade malignancies or overlooking highly aggressive tumors is an important area for further study.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11557460PMC
http://dx.doi.org/10.3389/fonc.2024.1449936DOI Listing

Publication Analysis

Top Keywords

perivascular epithelioid
8
epithelioid cell
8
pelvic cavity
8
diagnosis treatment
8
lesion resection
8
patients
5
cell tumor
4
tumor uterus
4
uterus pelvic
4
cavity background
4

Similar Publications

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!