Retrorectal or presacral tumors are rare lesions located in the presacral area and considered as being derived from multiple embryological remnants. These tumors are classified as congenital, neurogenic, osseous, inflammatory, or miscellaneous. The most common among these are congenital benign lesions that present with non-specific symptoms, such as lower back pain and change in bowel habit. Although congenital and developmental tumors occur in younger patients, the median age of presentation is reported to be 45 years. Magnetic resonance imaging plays a crucial role in treatment management through accurate diagnosis of the lesion, the evaluation of invasion to adjacent structures, and the decision of appropriate surgical approach. The usefulness of preoperative biopsy is still debated; currently, it is only indicated for solid or heterogeneous tumors if it will alter the treatment management. Surgical resection with clear margins is considered the optimal treatment; described approaches are transabdominal, perineal, combined abdominoperineal, and minimally invasive. Benign retrorectal tumors have favorable long-term outcomes with a low incidence of recurrence, whereas malignant tumors have a potential for distant organ metastasis in addition to local recurrence.
Keywords: Congenital cystic lesions; Perineal approach; Retrorectal tumors; Teratomas; Transabdominal approach, Combined abdominoperineal approach.
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