Surgical management of rectal gastrointestinal stromal tumors

Department of Surgical, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands
Journal of Surgical Oncology (Impact Factor: 3.24). 03/2013; 107(4). DOI: 10.1002/jso.23223
Source: PubMed


Five percent of gastrointestinal stromal tumors (GISTs) are primarily localized in the rectum. We analyzed the outcome of multimodality treatment for rectal GIST in a multicenter retrospective series.

All surgically treated patients with a rectal GIST were identified from four specialized centers in the Netherlands. Primary endpoints were disease-free survival (DFS) and overall survival (OS).

Thirty-two patients (22 men and 10 women) with rectal GISTs were identified. Twenty-two patients received imatinib before surgery for a median of 9 (range 2-53) months (Group 1). Ten patients received no imatinib because of small tumor size or lack of availability (Group 2). Median tumor size before treatment was 9.3 (range 6-17) cm in Group 1 and median 6 (range 4-14) cm in Group 2. A complete resection was possible in 17/22 (77%) patients in Group 1 versus 7/10 (70%) in Group 2. Median DFS was not reached in Group 1, while it was 36 months in Group 2. Median OS was not reached in both groups.

Preoperative imatinib leads to downsizing of the tumors in Group 1. However, it has not led to less extensive surgery. The DFS is longer in patients treated with pre- and post-operative imatinib, without an effect on OS.

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    • "There are a few studies that have shown that the use of IM adjuvant therapy and subsequent local resection is better than, or at least not inferior to, LAR or APR for anorectal GISTs (9,10,11). The present study reports the cases of two rectal GISTs that were treated by IM adjuvant therapy and subsequent transsacral local resections. "
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    ABSTRACT: Gastrointestinal stromal tumors (GISTs) are rare in the rectum. Radical surgery, such as an abdominoperineal resection, is necessary for large rectal GISTs, which can result in the loss of function of involved organs. Imatinib mesylate can be used as perioperative therapy and may reduce tumor size, and it is now approved for use in the adjuvant therapy of locally resected anorectal GISTs. The present study describes two cases of large rectal GISTs, for which abdominoperineal resections were initially planned. The two patients received pre-operative imatinib mesylate treatment, and the therapeutic response was assessed by magnetic resonance imaging. Finally, transsacral local resection was successfully performed for these two GISTs. A macroscopically complete resection was achieved, and microscopically, the resection margin was negative. One patient experienced the complication of rectal leakage, which was successfully managed by drainage. No recurrence occurred in the two patients after more than two years. Pre-operative imatinib mesylate therapy with subsequent transsacral local resection for selected rectal GISTs is a feasible treatment modality and can prevent extended surgery.
    Full-text · Article · Oct 2014 · Oncology letters
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    • "Negative surgical margins and preventing intraoperative rupture of the tumor are of utmost importance, while lymph node dissection is not necessary, except in patients where suspicious nodes are encountered such as in our case. With regard to anorectal GISTs, local excision may be sufficient with a minimum risk of morbidity and preservation of the anal sphincter, while in some cases more extensive surgery is unavoidable [1, 6]. "
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    ABSTRACT: Gastrointestinal stromal tumors (GISTs) are the most frequent mesenchymal tumors of the gastrointestinal tract with surgical resection remaining the cornerstone of therapy. Pararectal lesions are considered to be technically difficult and pose in some cases a challenge. We report, to the best of our knowledge, the first robotic-assisted pararectal GIST excision. A 43-year-old man was referred to our center with pararectal GIST recurrence, despite treatment with targeted therapy. Eleven years ago, he underwent extensive abdominal surgery including cystoprostatectomy with ileal neobladder diversion due to GIST resection in the rectoprostatic space. Robot-assisted surgical resection was successfully performed without the need for temporary colostomy. The postoperative course of the patient was uneventful, and the pathology report confirmed a GIST recurrence with negative surgical margins and pelvic lymph nodes free of any tumor. Robotic-assisted pelvic surgery can be extended to incorporate excision of pararectal GISTs, as a safe, less invasive surgical alternative with promising oncological results and minimal injury to adjacent structures.
    Full-text · Article · Sep 2014
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    • "However, our team believes that the information provided in this series is useful because tumor mutational testing is routinely performed in only a minority (approximately 5%) of GISTs worldwide. Nevertheless, the mutation frequency in rectal GISTs has not been specifically investigated [15,16] except in Miettinen’s study [8], which found mutations in 18 of 29 (62%) of the cases evaluated, with a predominance of KIT exon 11 mutations, a lack of KIT exon 13 mutations, and only one KIT exon 9 mutation. "
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    ABSTRACT: Rectal and pararectal gastrointestinal stromal tumors (GISTs) are rare. The optimal management strategy for primary localized GISTs remains poorly defined. We conducted a retrospective analysis of 41 patients with localized rectal or pararectal GISTs treated between 1991 and 2011 in 13 French Sarcoma Group centers. Of 12 patients who received preoperative imatinib therapy for a median duration of 7 (2-12) months, 8 experienced a partial response, 3 had stable disease, and 1 had a complete response. Thirty and 11 patients underwent function-sparing conservative surgery and abdominoperineal resection, respectively. Tumor resections were mostly R0 and R1 in 35 patients. Tumor rupture occurred in 12 patients. Eleven patients received postoperative imatinib with a median follow-up of 59 (2.4-186) months. The median time to disease relapse was 36 (9.8-62) months. The 5-year overall survival rate was 86.5%. Twenty patients developed local recurrence after surgery alone, two developed recurrence after resection combined with preoperative and/or postoperative imatinib, and eight developed metastases. In univariate analysis, the mitotic index (<=5) and tumor size (<=5 cm) were associated with a significantly decreased risk of local relapse. Perioperative imatinib was associated with a significantly reduced risk of overall relapse and local relapse. Perioperative imatinib therapy was associated with improved disease-free survival. Preoperative imatinib was effective. Tumor shrinkage has a clear benefit for local excision in terms of feasibility and function preservation. Given the complexity of rectal GISTs, referral of patients with this rare disease to expert centers to undergo a multidisciplinary approach is recommended.
    Full-text · Article · Mar 2014 · BMC Cancer
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