Background: Retrorectal sarcomas are rare, and limited data are available on oncologic outcomes following surgery. Our aim was to evaluate outcomes in this patient population at our institution. Materials and Methods: All patients who underwent surgical resection of a malignant retrorectal/presacral sarcoma between 1985 and 2005 were identified. Data analyzed included demographics, histopathologic diagnosis, surgical morbidity and mortality, use of adjuvant therapy, local and distant recurrence, and survival. Results: A total of 37 patients were identified (20 males) with a median age of 49 years (range, 22-81 years). The most common histopathologic diagnosis was malignant peripheral nerve sheath tumor (n = 8). Also, 22 tumors were high grade and 15 were low grade. Surgical margin status was R0 in 31 patients and R1 in 6. Adjuvant therapy was given to 26 patients. Postoperative morbidity and mortality was 57% and 3%, respectively. Median length of follow-up in 16 patients alive at last contact was 4.7 years. The 5-year survival free of local (LDFS), distant (DDFS), and local or distant recurrence (DFS) was 51, 58, and 39%, respectively. Patient survival at 2, 5, and 10 years was 75, 55, and 47%, respectively. Disease-free survival was not significantly associated with gender (P =.16), primary vs secondary (P =.94), R0 vs R1 resection (P =.26), low vs high tumor grade (P =.17), or the use of surgery with or without adjuvant therapy (P =.33). Conclusions: Retrorectal sarcomas are often high grade and locally advanced. Most tumors are resectable with free margins, and long-term survival may be possible in up to one-half of patients following an aggressive surgical approach.
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