Perivascular epithelioid cell tumor of the sigmoid colon with transcription factor E3 expression

We describe here a 62-year-old woman who presented with a perivascular epithelioid cell tumor arising in the sigmoid colon. Computed tomography revealed a 5-cm-sized intraluminal fungating mass. Histologically, the tumor consisted of plump, epithelioid cells with abundant clear-to-lightly eosinophilic cytoplasm and round nuclei, arranged in an alveolar or trabecular pattern. The tumor cells were strongly positive for HMB-45 and TFE3, but negative for vimentin, cytokeratin, smooth muscle actin, S100 protein, CD117, CD34, synaptophysin, chromogranin, CD10, hepatocyte antigen, CD1a, and desmin. The tumor cells had a high Ki-67 labeling index (up to 20%). Fluorescent in situ hybridization showed no evidence of the EWS rearrangement. Based on these histologic and immunohistochemical features, our patient was diagnosed with a perivascular epithelioid cell tumor of the sigmoid colon.