An unusual case of uterine clear cell tumor is reported in a 56-year-o
ld woman. The neoplasm was identified as a peculiar low-grade leiomyos
arcoma, composed mainly of watery, clear large cells with round, fairl
y regular nuclei. No appreciable pleomorphism or high mitotic activity
was noted. There was no necrosis, The presence of occasional areas of
transition between these cells and typical spindle leiomyosarcoma cel
ls, together with the immunohistochemical results, allowed the recogni
tion of a smooth muscle origin of this clear cell tumor. The patient w
as treated by hysterectomy. Six years later she developed an inguinal
metastasis resembling an hibernoma, and then later developed a large r
etroperitoneal metastasis, which was composed entirely of clear cells
and was distinguished from a liposarcoma by immunohistochemistry.