Perivascular epithelioid cell tumor of the uterus: immunohistochemical, ultrastructural and molecular study

Pathol Int. 2003 Nov;53(11):800-5. doi: 10.1046/j.1440-1827.2003.01557.x.


A case of perivascular epithelioid cell tumor of the uterus is reported, occurring in a 32-year-old woman. The tumor (8.0 cm in dimension) showed exophytic growth from the outer half of the myometrium. Histopathologically, the tumor was composed of thick blood vessels and perivascular epithelioid cells. The neoplastic cells were strongly immunoreactive for HMB45 antigen, CD117 (c-kit), vimentin and the progesterone receptor, but completely negative for S-100 protein, smooth muscle actin, desmin, CD34, the estrogen receptor and p16. The Ki-67 labeling index was low (1.25%). Ultrastructurally, the neoplastic cells had numerous premelanosomes with some glycogen deposits. Single-stranded DNA conformational polymorphism of p53 and methylation-specific polymerase chain reaction of p16 revealed negative results. Definite melanosomes on electron microscopic analysis and coexpression of HMB45 antigen and stem cell factor receptor (CD117) may provide the clue to understanding perivascular epithelioid cell tumor because angiomyolipoma also coexpresses HMB45 antigen and CD117.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adult
  • Antigens, Neoplasm
  • Blood Vessels / pathology
  • Cyclin-Dependent Kinase Inhibitor p16 / genetics
  • Epithelioid Cells / chemistry
  • Epithelioid Cells / pathology*
  • Epithelioid Cells / ultrastructure
  • Female
  • Humans
  • Immunohistochemistry
  • Melanoma-Specific Antigens
  • Microscopy, Electron
  • Neoplasm Proteins / analysis
  • Polymerase Chain Reaction / methods
  • Polymorphism, Single-Stranded Conformational
  • Proto-Oncogene Proteins c-kit / analysis
  • Receptors, Progesterone / analysis
  • Tumor Suppressor Protein p53 / genetics
  • Uterine Neoplasms / genetics
  • Uterine Neoplasms / metabolism
  • Uterine Neoplasms / pathology*
  • Vimentin / analysis


  • Antigens, Neoplasm
  • Cyclin-Dependent Kinase Inhibitor p16
  • Melanoma-Specific Antigens
  • Neoplasm Proteins
  • Receptors, Progesterone
  • Tumor Suppressor Protein p53
  • Vimentin
  • Proto-Oncogene Proteins c-kit