PEComa, otherwise known as perivascular epithelioid cell tumor, is a group of rare soft tissue sarcomas or cancers. This type of tumor is predominantly found in females versus males in a ratio estimated to be upwards of seven to one, respectively. The average age of tumor presentation is around 45 years; however, they have been seen in people ages 3 to 97. These tumors can occur in various locations in the body, mainly the abdominal cavity, pelvic region, uterus in women, and the abdominal tract. Most PEComas are benign or non-cancerous. In rare cases, some PEComas may be malignant, or cancerous, with no approved therapies until recently.