A huge malignant perivascular epithelioid cell tumor (PEComa) of the uterine cervix and vagina

Pathol Res Pract. 2014 Mar;210(3):186-8. doi: 10.1016/j.prp.2013.10.003. Epub 2013 Nov 20.


Perivascular epithelioid cell tumors (PEComas) are a family of rare mesenchymal neoplasms, including angiomyolipoma, clear-cell "sugar" tumor of the lung and extrapulmonary sites, lymphangioleiomyomatosis, clear-cell myomelanocytic tumor of the falciform ligament/ligamentum teres, and clear-cell tumors at various other anatomic sites. These tumors are characterized by a proliferation of epithelioid cells with clear to eosinophilic cytoplasm, perivascular distribution, and coexpression of myogenic and melanocytic markers. PEComas show a female predominance, occur with some frequency in the gynecological tract, and have an unpredictable biological behavior. We report a case of a huge malignant PEComa arising from the uterine cervix and vagina. To the best of our knowledge, only 6 cases of PEComa in the cervix and 2 cases in the vagina have been reported in the literature.

Keywords: Malignant behavior; Myomelanocytic tumor; PEComa; Uterine tumor.

Publication types

  • Case Reports

MeSH terms

  • Biomarkers, Tumor / analysis
  • Female
  • Humans
  • Immunohistochemistry
  • Middle Aged
  • Multimodal Imaging / methods
  • Perivascular Epithelioid Cell Neoplasms / chemistry
  • Perivascular Epithelioid Cell Neoplasms / pathology*
  • Perivascular Epithelioid Cell Neoplasms / surgery
  • Positron-Emission Tomography
  • Tomography, X-Ray Computed
  • Tumor Burden
  • Uterine Cervical Neoplasms / chemistry
  • Uterine Cervical Neoplasms / pathology*
  • Uterine Cervical Neoplasms / surgery
  • Vaginal Neoplasms / chemistry
  • Vaginal Neoplasms / pathology*
  • Vaginal Neoplasms / surgery


  • Biomarkers, Tumor