Scholarlink

<p>Uterine perivascular epithelioid cell tumors (PEComas) are exceptional mesenchymal neoplasms of the gynecologic tract that often mimic common uterine lesions and lack pathognomonic clinical or radiologic features. We report a malignant uterine PEComa in a 44-year-old woman presenting with abnormal uterine bleeding and pelvic pain. Preoperative imaging suggested a fibroid, but definitive diagnosis followed hysterectomy: in fact, histopathology showed epithelioid cells arranged around a rich vasculature with high-grade cytologic atypia, brisk mitotic activity, and necrosis, all features consistent with malignant behavior. Subsequent immunophenotyping demonstrated co-expression of melanocytic and smooth-muscle markers. The disease was organ-confined; no adjuvant therapy was administered, and the patient remains disease-free at 24 months under structured surveillance. To contextualize this case, we conducted a narrative review of the literature through October 2025, synthesizing epidemiology, pathogenesis, diagnosis, histology, treatment, and outcomes of uterine PEComas. Evidence supports complete surgical excision as the cornerstone of management for localized disease. For advanced or recurrent tumors, inhibitors of the mammalian target of rapamycin (mTOR) represent a rational option given frequent dysregulation of this pathway. Overall, malignant uterine PEComa requires multidisciplinary evaluation, pathology-driven diagnosis, and long-term follow-up; accumulating case-based evidence is refining risk stratification and informing the selective use of targeted therapies.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

Malignant uterine PEComa: a narrative literature review and challenging case report

  • 期刊论文

  • 出版模式：

    开放获取

  • 发表日期：

    2026年5月20日

查看全文
  • Mauro Francesco Pio Maiorano,
  • Joana Sorino,
  • Mario Della Mura,
  • Doriana Di Nanni,
  • Gerardo Cazzato,
  • Alessandro Rizzo,
  • Stella D’Oronzo,
  • Marco Cerbone,
  • Ettore Cicinelli,
  • Marco Marinaccio

摘要

Uterine perivascular epithelioid cell tumors (PEComas) are exceptional mesenchymal neoplasms of the gynecologic tract that often mimic common uterine lesions and lack pathognomonic clinical or radiologic features. We report a malignant uterine PEComa in a 44-year-old woman presenting with abnormal uterine bleeding and pelvic pain. Preoperative imaging suggested a fibroid, but definitive diagnosis followed hysterectomy: in fact, histopathology showed epithelioid cells arranged around a rich vasculature with high-grade cytologic atypia, brisk mitotic activity, and necrosis, all features consistent with malignant behavior. Subsequent immunophenotyping demonstrated co-expression of melanocytic and smooth-muscle markers. The disease was organ-confined; no adjuvant therapy was administered, and the patient remains disease-free at 24 months under structured surveillance. To contextualize this case, we conducted a narrative review of the literature through October 2025, synthesizing epidemiology, pathogenesis, diagnosis, histology, treatment, and outcomes of uterine PEComas. Evidence supports complete surgical excision as the cornerstone of management for localized disease. For advanced or recurrent tumors, inhibitors of the mammalian target of rapamycin (mTOR) represent a rational option given frequent dysregulation of this pathway. Overall, malignant uterine PEComa requires multidisciplinary evaluation, pathology-driven diagnosis, and long-term follow-up; accumulating case-based evidence is refining risk stratification and informing the selective use of targeted therapies.