Short-term and long-term outcomes after robotic radical surgery for rectal gastrointestinal stromal tumor.

BACKGROUND: The optimal approach for ensuring both complete resection and preservation of anal function in rectal gastrointestinal stromal tumor (GIST) remains unknown. The aim of this study was to clarify short-term and long-term outcomes after robotic radical surgery for rectal GIST. METHODS: A total of 13 patients who underwent robotic radical surgery for rectal GIST between December 2011 and April 2022 were included. All robotic procedures were performed using a systematic approach. A supplemental video of robotic radical surgery for rectal GIST is attached. The short-term outcome was the incidence of postoperative complications during the first 30 days after surgery. Surgical outcomes were retrieved from a prospective database. Long-term outcomes, including overall survival and recurrence-free survival, were determined in all patients. RESULTS: Median distance from the tumor to the anal verge was 4.0 cm. Surgical margins were negative in all patients. Two patients underwent neoadjuvant imatinib therapy. All patients underwent sphincter-preserving surgery. None underwent conversion to open or laparoscopic surgery. The incidence of postoperative Clavien-Dindo grade II and grade ≥ III complications was 7.7% and 0%, respectively. The median postoperative hospital stay was 7 days. Twelve patients (92.3%) underwent stoma closure within 5 months of the initial surgery. Median follow-up time was 76 months. The 5-year overall survival and recurrence-free survival rates were both 100%. None of the patients had recurrence. CONCLUSION: Short-term and long-term outcomes after radical robotic surgery for rectal GIST were favorable. Robotic surgery might be a useful surgical approach for rectal GIST.

Comparison of outcomes between neoadjuvant imatinib and upfront surgery in patients with localized rectal GIST: An inverse probability of treatment weighting analysis.

BACKGROUND AND OBJECTIVES: This study aimed to compare outcomes between neoadjuvant imatinib and upfront surgery in patients with localized rectal gastrointestinal stromal tumors (GIST) patients. METHODS: Eighty-five patients with localized rectal GIST were divided into two groups: upfront surgery ± adjuvant imatinib (Group A, n = 33) and the neoadjuvant imatinib + surgery + adjuvant imatinib (Group B, n = 52). Baseline characteristics between groups were controlled for with inverse probability of treatment weighting (IPTW) adjusted analysis. RESULTS: The response rate to neoadjuvant imatinib was 65.9%. After the IPTW-adjusted analysis, patients who underwent neoadjuvant therapy had better distant recurrence-free survival (DRFS) and disease-specific survival (DSS) compared with those who underwent upfront surgery (5-year DRFS 97.8 vs. 71.9%, hazard ratio [HR], 0.15; 95% CI, 0.03-0.87; p = 0.03; 5-year DSS 100 vs. 77.1%; HR, 0.11; 95% CI, 0.01-0.92; p = 0.04). While no significant association was found between overall survival (OS) and treatment groups (p = 0.07), 5-year OS was higher for the neoadjuvant group than upfront surgery group (97.8% vs. 71.9%; HR, 0.2; 95% CI, 0.03-1.15). CONCLUSIONS: In patients with localized rectal GIST, neoadjuvant imatinib not only shrunk the tumor size but also decreased the risk of metastasis and tumor-related deaths when compared to upfront surgery and adjuvant imatinib alone.

Transvaginal excision of rectal stromal tumors: case reports and a literature review.

BACKGROUND: Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. Rectal locations are very rare, and minimally invasive surgery is a good choice for the treatment of rectal GISTs. CASE PRESENTATION: Two women each had a mass located on the lower vaginal-rectal space as determined by transvaginal ultrasound (TV-US), pelvis MR imaging, and colonoscopy. The patients successfully underwent transvaginal excision. The spindle-shaped cells were found in pathological test. The immunohistochemical analysis showed that CD117 and Dog-1 were stained positively. These results confirmed the masses as GISTs. The postoperative period was uneventful without anal dysfunction. Two patients were received adjuvant treatment with imatinib after surgery. CONCLUSION: Transvaginal excision could be a minimally invasive and safe alternative treatment in the management of rectal GISTs in lower locations.

Management of a case of high-risk gastrointestinal stromal tumor in rectum by transanal minimal invasive surgery.

BACKGROUND: Rectal gastrointestinal stromal tumor (GIST) is a very rare tumor of gastrointestinal tract. Surgical management of rectal GIST requires special attention for preserving of anal and urinary functions. Transanal minimal invasive surgery (TAMIS) is a well-developed minimally invasive technique for local excision of benign and early malignant rectal tumors; however, the application of TAMIS for rectal GIST is rarely and inadequately reported. We report the novel application of TAMIS for rectal GIST with considerations for anal and urinary functions. CASE PRESENTATION: A 67 years old female, who presented with history of per rectal bleeding, was diagnosed with submucosal GIST of 4.5 cm in diameter at right posterior wall of 7 cm from anal verge. Histology of biopsy showed abundant spindle-shaped cells arranged in bundles that were positive for CD34 and negative for C-Kit, desmin, smooth muscle actin (SMA), and S-100. The tumor was excised by TAMIS successfully. Final histopathology showed pT2 tumor with C-Kit positive and mitosis count 10 per 50 HPF. Postoperative period was uneventful, and she was discharged on adjuvant imatinib mesylate for 3 years. CONCLUSION: TAMIS can be used safely in the management of rectal GIST after appropriate evaluation of tumor size, extent, location, and experience of operating surgeon.

A Rare Case of a Rectal Gastrointestinal Stromal Tumor (GIST) Discovered During a Routine Colonoscopy.

A gastrointestinal stromal tumor (GIST) is a rare malignancy, accounting for only 0.1% to 3% of all gastrointestinal (GI) malignancies. Although GISTs are the most common mesenchymal tumor of the GI tract, they are primarily found within the stomach, with rectal GISTs rarely reported. They may present with rectal bleeding, constipation, pain, or a palpable mass while some are found incidentally. The incidence of GISTs has been on the rise, possibly due to advancements in diagnostic technology. In this case report, we present a 50-year-old female who presented with intermittent constipation and rectal pain and was found to have a submucosal rectal mass during a routine diagnostic colonoscopy. Further evaluation confirmed the presence of a spindle-cell neoplasm, which was mildly cellular and showed positive expression of CD34 and CD117 on immunohistochemistry, consistent with the diagnosis of GIST of the rectum. This case report emphasizes the importance of routine colonoscopies in the early detection of neoplastic lesions of the colon and highlights the rare incidence of GISTs, their risk factors, pathogenesis, and common sites of occurrence.

Gastrointestinal Stromal Tumours (GIST) of the Rectum: A Systematic Review and Meta-Analysis.

Background: Rectal gastrointestinal stromal tumours (GISTs) have many treatment options, but uncertainty remains regarding the best treatment regimen for this rare pathology. The aim of this review is to assess the optimal management approach including timing of chemotherapy. Methods: PubMed, EMBASE, and Cochrane databases were searched for relevant articles comparing the impact of radical vs. local excision, and neoadjuvant vs. adjuvant therapy had on outcomes in the management of rectal GISTs. We specifically evaluated the influence that the aforementioned factors had on margins, recurrence, overall survival, 5-year disease-free survival, and hospital length of stay. Results: Twenty-eight studies met our predefined criteria and were included in our study, twelve of which were included in the quantitative synthesis. When comparing neoadjuvant versus adjuvant chemotherapy, our meta-analysis noted no significance in terms of margin negativity (R0) (odds ratio [OR] 2.01, 95% confidence interval [CI], 0.7−5.79, p = 0.20) or recurrence rates (OR 0.22, 95% CI, 0.02−1.91, p = 0.17). However, there was a difference in overall 5-year survival in favour of neoadjuvant therapy (OR 3.19, 95% CI, 1.37−7.40, * p = 0.007). Comparing local excision versus radical excision, our meta-analysis observed no significance in terms of overall 5-year survival (OR1.31, 95% CI, 0.81−2.12, p = 0.26), recurrence (OR 0.67, 95% CI, 0.40−1.13, p = 0.12), or 5-year disease-free survival (OR 1.10, 95% CI, 0.55−2.19, p = 0.80). There was a difference in length of hospital stay with a reduced mean length of stay in local excision group (mean difference [MD] 6.74 days less in the LE group; 95% CI, −6.92−−6.56, * p =< 0.00001) as well as a difference in R0 rates in favour of radical resection (OR 0.68, 95% CI, 0.47−0.99, * p = 0.05). Conclusion: Neoadjuvant chemotherapy is associated with improved overall 5-year survival, while local excision is associated with reduced mean length of hospital stay. Further large-volume, prospective studies are required to further define the optimal treatment regimen in this complex pathology.

Rectal gastrointestinal stromal tumor: an unusual presentation of an uncommon pathology (a case report).

Gastrointestinal stromal tumours (GIST) are a rare form of neoplasm. The stomach is the commonest location while gastrointestinal bleeding and pain are the usual presentations. Rectal GIST has been reported in literature as a rare occurrence. We report the rare case of a 37-year-old man who presented with large bowel obstruction and acute urinary retention arising from a rectal GIST. Radiological investigations showed features in keeping with intestinal obstruction. He had a divided colostomy and tumour debulking. Histology of tumour revealed a rectal GIST and immunohistochemical staining was positive for CD34 and CD117. Postoperatively Imatinib was commenced and patient did well. We report this case to highlight the unusual symptoms that may arise from a rare pathology like rectal GIST and the need to consider an alternative diagnosis-such as GIST, in a young adult presenting with large bowel obstruction in the absence of risk factors for bowel adenocarcinoma.

Transsacrococcygeal approach in rectal gastrointestinal stromal tumour resection: 10-year experience at a single centre.

BACKGROUND: The transsacrococcygeal (TSC) approach in rectal gastrointestinal stromal tumour (GIST) resection is clinically challenging and controversial, and we evaluated its value in the present study. METHODS: We enrolled patients who underwent rectal GIST resection by the TSC approach during 2008-2018. The clinicopathological index, surgical outcome, and prognosis were analysed. Prognostic information was obtained from medical records and follow-up data. Anal function was evaluated by the low anterior resection syndrome (LARS) score. RESULTS: Among 88 rectal GIST patients over the 10-year study period, 17 who underwent the TSC approach were analysed. The median age was 55 (range, 26-73) years. In total, 15 patients received preoperative imatinib neoadjuvant therapy for 232 (30-690) days. The tumours were exogenous in 14 patients and intramural in 3 patients. The mean initial tumour size and preoperative tumour size were 6.4±2.2 and 4.2±1.7 cm, respectively. The operative time and blood loss were 130.2±47.4 min and 44.6±36.0 mL, respectively. Of the 17 patients 7 had postoperative complications (within 30 days postoperatively), and the complications of 5 patients were cured by conservative treatment. Only 1 patient was lost to follow-up, and the others had a good oncological prognosis at recent follow-up evaluations. All patients had LARS scores ≤9 points at 1 year after the operation. CONCLUSIONS: The TSC approach can result in a good oncological prognosis, usually does not affect anal function, and is particularly suitable for exogenous middle and low rectal GISTs. However, it might cause some controlled complications. Hence, careful patient selection is necessary for this operation.

Comparison of prognostic prediction models for rectal gastrointestinal stromal tumor.

BACKGROUND: Rectal gastrointestinal stromal tumors (RGISTs) are biologically characterized tumors that are relatively rare. Thus, few studies have reported a specific prognostic system for this subset of tumors but integrated it into parallel systems, such as small intestine. Our aim is to develop a new predictive staging system nomogram (named FD-ZS system) for RGISTs. RESULTS: Tumor size and mitotic rate were independent risk factors for tumor recurrence in RGISTs according to univariate and multivariate survival analyses. A prognostic predictive nomogram was developed, and a cut-off value of 65 points was calculated by X-tile to discriminate risk based on tumor size and mitotic rate. The C-indices for the FD-ZS, FD-Hou, NIH, and WHO systems were 0.706, 0.693, 0.687, and 0.680, respectively. CONCLUSION: In the present study, a concise two-tier grading system (FD-ZS) for prognostic prediction of RGISTs that is simpler to several reported systems was developed, and a cut-off value was established to help RGIST patients determine whether to undergo adjuvant imatinib treatment. METHODS: A nomogram was employed, and its predictive accuracy and discriminative ability were determined by concordance index (C-index) and calibration curve analyses. The nomogram was then compared with three stratification systems used for GISTs (FD-Hou, NIH, and WHO).

Treatment of gastrointestinal tumor (GIST) of the rectum requiring abdominoperineal resection following neoadjuvant imatinib: a cost-effectiveness analysis.

BACKGROUND: Neoadjuvant imatinib for gastrointestinal stromal tumors (GIST) of the rectum can reduce, but may not eliminate, risk of surgical morbidity from permanent bowel diversion. We sought to evaluate the cost-effectiveness of alternative strategies in rectal GIST patients requiring abdominoperineal resection following neoadjuvant imatinib. METHODS: We developed a Markov model using a healthcare payers' perspective to estimate costs in 2017 Singapore dollars (SGD) and quality adjusted life years (QALYs) for upfront abdominoperineal resection (UAPR) versus continued imatinib until progression (CIUP) following 1 year of neoadjuvant imatinib. Transition probabilities and utilities were obtained from published data, and costs were estimated using data from the National Cancer Centre Singapore. Deterministic and probabilistic sensitivity analyses were conducted to probe model uncertainty. Incremental cost-effectiveness ratio below SGD 50,000 per QALY gained was considered cost-effective. RESULTS: In the base case, UAPR dominates CIUP being both more effective (8.66 QALYS vs 5.43 QALYs) and less expensive (SGD 312,627 vs SGD 339,011). These estimates were most sensitive to 2 variables, utility of abdominoperineal resection and annual recurrence probability post-abdominoperineal resection. However, simultaneously varying the values of these variables to maximally favor CIUP did not render it the more cost effective strategy at willingness to pay (WTP) of SGD 50,000. In probabilistic sensitivity analysis, UAPR had probability of being cost-effective compared with CIUP greater than 95%, reaching 100% at WTP SGD 10,000. CONCLUSION: UAPR is more effective and less costly than CIUP for patients with rectal GIST requiring abdominoperineal resection following neoadjuvant imatinib, and is the strategy of choice in this setting.

Open transanal resection of low rectal stromal tumor following neoadjuvant therapy of imatinib mesylate: Report of 11 cases and review of literature.

OBJECTIVE: The global burden of rectal gastrointestinal stromal tumor (GIST) is increasing. However, a limited number of reports hinder our ability to reach a definitive conclusion regarding the current treatment and prognosis. In this study, we outline our experience with open transanal resection of rectal GIST following neoadjuvant therapy with imatinib mesylate (IM). PATIENTS AND METHODS: We retrospectively analyzed 11 patients with rectal GISTs treated with neoadjuvant IM therapy and open transanal resection between April 2011 and April 2017 in Shanghai Changzheng Hospital. RESULTS: The patients had 400-600 mg/day IM once daily for a median of 7 months (range: 3-9 months). Tumor size, distance from the lower margin of the tumor to the anal verge (AV), mitotic rates and mutation analysis were assessed on pretreatment biopsy. After reassessment, all 11 patients underwent transanal R0 resection. With median follow-up of 28 months (range: 8-80 months), there was no tumor recurrence or metastasis. CONCLUSION: Open transanal resection of rectal GIST after neoadjuvant treatment with IM has good surgical and survival outcomes.

Management of rectal gastrointestinal stromal tumor.

Gastrointestinal stromal tumor (GIST) is the most common mesenchymal neoplasm of the gastrointestinal tract. However, rectal GIST is rare, the incident rate of it is approximately 5% of all GISTs. Rectal GIST symptoms generally include bleeding and/or pain and occasionally, urinary symptoms. Immunohistochemical evaluation finds that most rectal GIST tumors are CD117 (KIT) positive, and are sometimes CD34, platelet-derived growth factor receptor alpha (PDGFRA), smooth muscle actin, S-100, or vimentin positive. The National Institutes of Health (NIH) classifies rectal GIST as very-low risk, low risk, intermediate risk, or high risk, and the frequencies have been estimated as 0-23.8% for very-low risk, 0-45% for low risk, 0-34% for intermediate risk, and 21-100% for high risk tumors. The first-line treatment for localized GIST is curative resection, but is difficult in rectal GIST because of anatomical characteristics such as the deep, narrow pelvis and proximity to the sphincter muscle or other organs. Several studies noted the efficacy of the minimally invasive surgery, such as trans-anal, trans-sacral, trans-vaginal resection, or laparoscopic resection. The appropriate surgical procedure should be selected depending on the case. Imatinib mesylate (IM) is indicated as first-line treatment of metastatic or unresectable GIST, and clinical outcomes are correlated with KIT mutation genotype. However, the KIT mutation genotypes in rectal GIST are not well known. In this review, as in other GISTs, a large proportion (59-100%) of rectal GISTs carry exon 11 mutations. Although curative resection is indicated for localized rectal GIST, a high rate of local recurrence is a problem. Multimodal therapy including perioperative IM may improve postoperative outcomes, contributing to anus-preserving surgery. Moreover, KIT mutation analysis before IM treatment is important. This review summarizes current treatment strategies for rectal GIST.

Rectal gastrointestinal stromal tumour: What do we know in 2017? A systematic review protocol.

INTRODUCTION: Gastrointestinal stromal tumour is a pathology that originates from the interstitial cells of Cajal and differentiates from other mesenchymal neoplasm by expression of CD117 oncogene on Immunohistochemistry test. Colon and Rectal GISTs constitutes of approximately 5% of all gastrointestinal GISTs. The past decade has witnessed a dramatic change in the treatment of rectal cancer. Preoperative, perioperative and postoperative, management has changed thanks to new chemotherapy regimens and emergence of novel surgical techniques. Our aim is to investigate if same change can be implemented for rectal GISTs management. METHODS AND ANALYSIS: This protocol is compliant with the Preferred Reporting Items for Systematic Review and Meta-Analysis protocols (PRISMA-P) guidelines. Exclusion and inclusion criteria are outlined within this protocol. Points of interest and objectives are described within this protocol. The search strategy, aims to identify all articles on "Rectal GISTs". DISCUSSION: The choice of resection type surgery depends upon the location and size of rectal GIST. Neoadjuvant Imatinib therapy yields tumour shrinkage in at least 50% and is associated with a prolonged disease-free survival for intermediate and high-risk patients. This review will also allow a summary clinicopathological features and prognostic factors of rectal GISTs. ETHICS AND DISSEMINATION: The Centre for Reviews and Dissemination, University of York acknowledged that this systematic review is within the register scope. This review will be published in a peer-reviewed journal and will be presented at various national and international conferences.

Sphincter-saving resection by cluneal arched skin incision for a gastrointestinal stromal tumor (GIST) of the lower rectum: a case report.

BACKGROUND: Planning the surgical strategy for a gastrointestinal stromal tumor (GIST) at the posterior wall of the lower rectum is difficult, as the procedures for the lower rectum are hampered by poor visualization and may cause anal dysfunction or discomfort. We report a novel procedure to resect a submucosal tumor of the rectum. CASE PRESENTATION: A 75-year-old woman presented with metrorrhagia. Endovaginal ultrasonography showed a low echoic tumor. Computed tomography showed an enhanced tumor, measuring 5.3 × 4.2 cm, behind the rectum. Magnetic resonance imaging revealed a submucosal tumor of the rectum, measuring 5.3 cm at its greatest dimension. Colonoscopy showed that the distal tumor margin was 1 cm above the dentate line. Core needle biopsy of the tumor revealed the rectal GIST. After receiving neoadjuvant imatinib treatment, the tumor size decreased to 3.5 cm. During the operation, we approached the rectum and resected the posterior rectal wall, including the 3.5 × 3.5 cm tumor with a safety margin, making an arched incision at the buttocks to form a skin flap with the patient in a jackknife position. The histopathological diagnosis was GIST of the rectum. Her anorectal sphincter function was well preserved. No recurrence was seen during the 2-year follow-up. CONCLUSIONS: This novel approach improves the operative field visibility in resecting a tumor with a safety margin and preserves a patient's anorectal sphincter function.

Case report of diffusely metastatic rectal GIST.

This is a case report of an aggressive, diffusely disseminated Stage IV rectal gastrointestinal stromal tumor (GIST) in a 57-year-old male that presented for symptoms of malaise, constipation, and twenty pound weight loss in 2 months. Upon rectal examination, a hard 4centimeter submucosal mass was found at the 9-12 o'clock position. Liver and lung metastases were visualized on computerized tomography (CT) of the chest, abdomen, and pelvis on metastatic work-up. He was deemed a poor surgical candidate due to diffuse metastatic disease and referred for palliative chemotherapy. The patient had suffered a perforation of his rectal wall two weeks after his initial presentation and passed away shortly thereafter. He never received palliative chemotherapy. We present a case report as a unique case of an extremely aggressive and quickly fatal GIST tumor.

Rectal GIST-Outcomes and viewpoint from a tertiary cancer center.

INTRODUCTION: There is scarce data relating to methods to improve sphincter preservation in rectal gastrointestinal stromal tumor (GIST). Increasing the duration of neoadjuvant (NA) imatinib resulting in improved sphincter preservation rate has not been established. This retrospective analysis looks at the rates of sphincter preservation in rectal GIST with NA imatinib and effect of duration of NA imatinib on the same to find out optimum duration of NA with respect to sphincter preservation in rectal GIST patients. METHODS: Twenty-three cases of GIST of lower third of rectum were treated at our centre from 2005 till 2015. NA imatinib was used in a dose of 400 mg. Response evaluation was done every 3 months with a pelvic magnetic resonance imaging. Surgical management was determined by a team of experienced gastrointestinal oncosurgeons. RESULTS: Five patients underwent upfront surgery which included local resection in four patients and abdominoperineal resection in one patient. NA imatinib was used in 69.5 % (16/23) patients. Median duration of NA imatinib was 15 months (3-84 months). Amongst who underwent a sphincter-salvage surgery median duration of NA imatinib was 13 months whereas 18 months in patients who required a sphincter-sacrificing surgery (p = 0.683). The radiologic response included partial response in 75 % (12/16) patients, stable disease in 18.7 % (3/16) and one with progressive disease. Definitive surgical resection was possible in 13 patients (81.3 %) after NA imatinib. Median progression-free survival (PFS) was 120 months in the whole cohort whereas median overall survival (OS) was not reached. Four-year estimated PFS and OS was 81 % and 100 %, respectively. Median disease-free survival in upfront surgery group vs. neoadjuvant imatinib group was 70 vs. 120 months, respectively (p = 0.039). CONCLUSION: Neoadjuvant imatinib appears to be a useful option in improving chances of sphincter preservation without adversely affecting the outcome. Use of neoadjuvant imatinib leads to improvement in progression-free survival in patients with GIST of lower third of the rectum.

Resección de GIST rectal mediante cirugía transanal mínimamente invasiva (TAMIS) tras neoadyuvancia con imatinib / Resection of a rectal gastrointestinal stromal tumour by transanal minimally invasive surgery (TAMIS) after neoadjuvant treatment with Imatinib

Resumen El tratamiento primario de elección para los pacientes con una tumoración GIST localizada es la extirpación quirúrgica completa con márgenes microscópicos negativos. Sin embargo, en un espacio tan reducido como el de la pelvis, la resección completa de una tumo-ración rectal grande es difícil y necesita en ocasiones una amputación abdomino-perienal. En nuestro caso, con la finalidad de reducir el tamaño del tumor y la morbilidad asociada a procedimientos quirúrgicos más agresivos se introdujo el tratamiento con imatinib, con intención neoadyuvante monitorizando la respuesta mediante ecoendoscopia. La respuesta obtenida, rediciendo el volumen tumoral, modificó la estrategia quirúrgica inicial y fue posible conseguir una resección satisfactoria mediante cirugía transanal mínimamente invasiva (TAMIS), preservando los esfínteres anales y soslayando la morbilidad genitourinaria asociada a la excisión mesorectal.

Abstract The primary treatment of choice for patients with a localised gastro-intestinal stromal tumour (GIST) is complete surgical excision with negative microscopic margins. However, in a space as small as that of the pelvis, complete resection of a large rectal tumour is difficult, and sometimes requires an abdominoperineal amputation. In order to reduce the size of the tumour, as well as the morbidity associated with more aggressive surgical procedures, neoadjuvant treatment with Imatinib was introduced in this case, with the response being monitored by of endoscopic ultrasound. The response obtained by reducing the tumour volume modified the strategy, making it possible to obtain a satisfactory resection using transanal minimally invasive surgery (TAMIS), preserving the anal sphincters and avoiding the genitourinary morbidity associated with the mesorectal excision.

Gastrointestinal tumors of the colon and rectum.

Gastrointestinal stromal tumors (GISTs) of the colon and rectum are the most common mesenchymal tumors of the gastrointestinal tract. GISTs of the colon and rectum constitute ~5% of all cases. Although colorectal GISTs can be small and found incidentally, the majority appear to be high risk and carry a significant likelihood of recurrent and metastatic disease. Surgery remains the mainstay of treatment for primary disease. There is now considerable interest in GISTs because they can be treated effectively with targeted molecular therapies, specifically tyrosine kinase inhibitors (TKIs), such as imatinib mesylate and sunitinib malate. GISTs are best treated by a multidisciplinary team comprised of the surgeon, medical oncologist, pathologist, and radiologist in the initial evaluation, management, and in continued follow-up. Increasing the number of resectable cases through pharmacologic debulking, optimizing the timing of surgery and organ preservation, reducing recurrence and surgical morbidity, prolonging survival, and possibly enhancing response to imatinib through surgical cytoreduction are all potential benefits of multidisciplinary management.