Retrorectal Tumors

Retrorectal tumors are a group of rare (incidence <1%), indolent lesions that arise in the presacral space. Herein, we describe the presentation, clinical features, management, and outcomes of these tumors. Retrorectal tumors are heterogeneous and can arise from any of the germ cell layers. Most are congenital and are developmental cysts. The most common malignant tumor is a sacrococcygeal chordoma. Employment of a multidisciplinary team optimizes outcomes given the complexity of the pelvic anatomy. Complete cross-sectional preoperative imaging and selective preoperative biopsy of these tumors helps to guide surgical treatment.