Surgical Approach and Oncologic Outcomes Following Multidisciplinary Management of Retrorectal Sarcomas

Abstract

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).

The Value of Preoperative Biopsy in the Management of Solid Presacral Tumors

“…Because most nonchordoma malignant presacral tumors are sarcoma variants, it is our practice to use preoperative radiotherapy and, in selected cases, intraoperative radiation therapy for large, locally advanced high-grade tumors. 5 Because we would not give neoadjuvant therapy without a tissue diagnosis, a biopsy is mandatory. moreover, because all malignant presacral tumors get a wide-margin resection that often includes sacrectomy, we would not subject patients to urinary and sexual dysfunction, or other potentially morbid outcomes, without certainty that the tumor is malignant before the operation.…”

“…in addition, because many benign, solid lesions are neurogenic in origin, we use a nerve-sparing approach that includes leaving the tumor pseudocapsule intact. 5,23 for benign tumors, preservation of function is our primary goal over complete tumor resection. We would not want to mistakenly use this approach in a malignant lesion, compromising the oncologic outcome.…”

“…4 in contrast, although solid tumors can be both benign and malignant, many are malignant with a wide range of pathologic subtypes, which complicates their management. [3][4][5][6][7][8] an accurate preoperative diagnosis of solid or heterogeneous tumors in the presacral space is crucial, because the clinical management of benign and malignant lesions differs considerably. surgically, a wide-margin, en-bloc oncologic resection is indicated for most malignant lesions, but a close-margin approach should be attempted for benign lesions to spare function and avoid morbidity.…”

Surgical resection of retrorectal tumours in adults: long‐term results in 47 patients

“…Four (4/5, 80%) en bloc rectal excisions with primary colorectal anastomosis were performed owing to adherence to the retrorectal lesion. The median hospital stay was 8 (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20) days. There was no postoperative mortality.…”

“…During intrauterine life, the various tissues of the retrorectal space (neurectodermal and endodermal) undergo remodelling, and can therefore be the site of heterogeneous lesions originating from these vestigial tissues. RT may be classified as congenital, neurogenic, osseous or miscellaneous [2,3]. Two-thirds are congenital [4], caused by embryological sequestration during midline fusion or incomplete embryological regression.…”