Key Concepts

• The presacral (retrorectal) space is the location of a wide range of rare tumors with incidence as low as 1 in 40,000–60,000 hospital admissions. Discovery in asymptomatic patients is increasing due to expanded use of cross-sectional imaging.
• Advances in cross-sectional imaging and understanding of tumor biology have led to better outcomes for these challenging patients.
• MRI is the best imaging study to assist in diagnosis and operative planning.
• Most benign lesions have malignant potential and observation alone in some patients is acceptable when a dedicated surveillance protocol is in place.
• When performed appropriately and selectively, a CT-guided biopsy of the lesion may assist in management of solid and heterogeneous cystic lesions.
• The surgical principles that should guide a surgeon who manages these lesions are a function-sparing approach for benign lesions and an en bloc approach for malignant lesions.