Abstract
Primary colorectal sarcomas have been defined as a rare and diverse group of mesenchymal cancers distinct from gastrointestinal stromal tumors (GISTs). Primary colorectal sarcomas have been recognized as a distinct entity from GISTs due to the dramatically worse prognosis these sarcomas carry. Also, primary colorectal sarcomas when compared to the more common colorectal adenocarcinoma, demonstrate more aggressive biology, present at a younger age and carry worse outcomes. At this time, surgery remains the mainstay of treatment and adjuvant chemotherapy has an unclear role in treatment of primary colorectal sarcoma. This paper attempts to review the available data regarding primary colorectal sarcomas.