Recurrent perivascular epithelioid cell tumor of the uterus (PEComa): an immunohistochemical study and review of the literature

Gynecol Oncol. 2003 Sep;90(3):677-81. doi: 10.1016/s0090-8258(03)00325-1.


Background: Neoplasms of the perivascular epithelioid cell, first described in 1992, comprise a family of tumors known as "PEComas." To the best of our knowledge, this is the third case of uterine PEComa reported in the literature to behave in a malignant fashion. The purpose of this report is to provide information that may be used to predict the future behavior of this tumor.

Case: The patient was a 79-year-old woman with a large uterine mass which recurred 2 years following resection. The patient died within months after resection of the recurrent tumor. Retrospective immunohistochemical staining with newly commercially available antibodies including MART-1 was done on both the original and the recurrent tumor, confirming the diagnosis of uterine PEComa.

Conclusion: Uterine PEComas should be regarded as tumors with uncertain malignant potential.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Antigens, Neoplasm
  • Epithelioid Cells / pathology*
  • Female
  • Humans
  • Immunohistochemistry
  • MART-1 Antigen
  • Neoplasm Proteins / analysis
  • Uterine Neoplasms / blood supply
  • Uterine Neoplasms / metabolism
  • Uterine Neoplasms / pathology*


  • Antigens, Neoplasm
  • MART-1 Antigen
  • MLANA protein, human
  • Neoplasm Proteins