An unusual endometrial stomal sarcoma is described in a 50-year-old pa
tient. The distinctive feature of this case is the focal occurrence of
sex cord-like pattern and rhabdoid appearance of tumor cells. Rhabdoi
d cells have an eosinophilic cytoplasm and a vesicular nucleus with a
prominent nucleolus. Immunohistochemistry showed positive cytoplasmic
staining for both cytokeratin and vimentin, and ultrastructural examin
ation identified tumor cells with abundant cytoplasmic intermediate fi
laments. To our knowledge, only one case of endometrial stromal sarcom
a with this unusual morphological association has been reported in the
literature.