A retrospective case study of 13 uterine perivascular epithelioid cell neoplasm (Pecoma) patients

Abstract

Introduction and Hypothesis: Perivascular epithelioid cell neoplasms (PEComas) are rare mesenchymal tumors that originate from perivascular epithelioid cells. The uterus is the second most common organ to be affected by PEComa. Most PEComas are benign and the prognosis is usually good. Surgery is the main treatment at present, and adjuvant therapy is mainly used for malignant cases. However, because of the lack of described cases, the best diagnosis and treatment of these tumors cannot be determined. Methods: From 2009 to 2020, 13 patients from Shengjing Hospital (China Medical University), with uterine PEComa, who met the inclusion criteria and appropriate pathological diagnosis were enrolled in this study. Clinical, pathological, and therapeutic features were retrospectively analyzed to determine the best approach towards diagnosis and treatment. Results: All the enrolled patients underwent surgical treatment; four of them had a malignant PEComa. Three of the malignant patients received chemotherapy after surgery; among them, one died, another showed no obvious recurrence after regular re-examination, and the third did not undergo any further treatment despite short-term recurrence. However, upon regular re-examination, no progress was observed. The fourth malignant patient did not receive chemotherapy after surgery and showed no obvious recurrence during regular reviews. Conclusion: The preoperative diagnosis of uterine PEComa lacks specificity and therefore is often confused with uterine leiomyoma or leiomyosarcoma. We conclude that uterine PEComa can be diagnosed by combined analysis of immunohistochemistry and postoperative pathology. Though surgical resection is still the main treatment, high-risk patients can be given adjuvant treatment to strengthen disease control.