https://orcid.org/0000-0002-1835-0960
Abstract
Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors originating from perivascular epithelioid cells. In gynecological system, the uterus is one of the most common sites affected by PEComas. Most PEComas are benign, and patients usually have a good prognosis. However, malignant uterus PEComa is rare, and better comprehensive epidemiological investigations are needed. To date, there are a few reported cases of uterus PEComa. We herein report a rare case of malignant PEComa occurred in the uterine corpus and cervix, possibly accompanied by pulmonary lymphangioleiomyomatosis (PLAM). In addition, 55 cases of malignant uterus PEComa were picked out and collected in the data base of PubMed and Medline. On the one hand, the age of onset, population distribution, clinical manifestations, metastatic sites and routes of metastasis were analysed. On the other hand, a summary of the epidemiology, pathogenesis, diagnosis, and treatments of uterus PEComa was given.
Data Sharing Statement
This case report contains clinical data from the medical records in the Affiliated Hospital of Southwest Medical University. Additional information is available from the corresponding author Q. Wang or Y. Yang upon reasonable request.
Ethics Approval
The study was conducted in accordance with the Declaration of Helsinki. The Affiliated Hospital of Southwest Medical University has approved the release of case details.
Consent Statement
The patient gave her consent for publication of the case report.
Disclosure
None of the authors declare any conflict of interest.