Presacral (retrorectal) tumors: a review of the literature for correct diagnosis and treatment, with emphasis on the most common tumors

Abstract

Presacral tumors are rare tumors of the lesser pelvis. They are reported in the literature as case report studies or case series studies with a small number of patients, collected after a long period of time in tertiary hospitals. We carried out an extensive review of the literature and tried to cite the most recent publications on presacral tumors. PubMed and Google Scholar databases were searched systematically for studies related to presacral tumors from 1970 to 2022. Clinical and imaging data, main anatomical data with interest in surgical therapy, and pathological data of presacral tumors were collected. Surgical therapies, the implication of surgical specialties or other therapies as chemoradiation were reviewed. Most common presacral tumors (55–70%) are congenital. The majorities are benign, and the most common benign presacral tumors are developmental cysts, while chordoma is the most common malignant presacral tumor and it is resistant to chemoradiation. After congenital tumors, neurogenic tumors follow in frequency (10% of presacral tumors), and the majority are benign entities (85% of neurogenic tumors). The next in frequency are osseous tumors (6–11% of presacral tumors), with the most common being the giant cell tumors and Ewing’s sarcoma. Surgical treatment with radical excision is the main treatment after meticulous preoperative planning of the surgical approach to the tumors, based on imaging data collected, most surgical specialties may be involved in therapy. Surgery offers excellent results in benign entities. In malignant tumors, chemoradiation has limited indications as in Ewing’s sarcoma. The majority of the remaining sarcomas, after surgery, present high recurrences rates, high morbidity and low survival rates. Surgery in benign presacral tumors (the majority of presacral tumors) offers excellent results. In malignant tumors, radical surgical resection with free surgical limits is the goal of surgery.