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a, b The resected tumor was 3.5 × 3.5 × 2.5 cm in size. The rubbery-hard tumor with widespread central necrosis was completely capsulized. c Microscopic examination (hematoxylin-eosin staining, original magnification: ×400) revealed a formation of spindle-shaped cells. d Immunohistochemical staining of the tumor cells for c-kit revealed strong positive findings
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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 presentati...
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Background
Gastrointestinal stromal tumors (GIST) are rare abdominal tumors. Pretreatment biopsies may be used to diagnose a GIST and enable tailored treatment. Some experts are skeptical about biopsies because they fear tumor cell seeding. The objective of this study was to determine if pretreatment biopsy is associated with increased tumor recurr...
Citations
... The primary goal of surgery is to obtain negative microscopic margins without causing bleeding or rupture of the pseudocapsule [5]. Transanal resection is one of the most minimally invasive methods but is limited by the distance from the dentate line [6]. Transcoccygeal excision is adequate for lower rectal GIST but has high postoperative morbidity, with fistulae occurring in 21% of patients [7]. ...
Tailgut cysts and rectal gastrointestinal stromal tumors (GIST) are rare rectal tumors found in the retrorectal space. Tailgut cysts are congenital malformations resulting from incomplete regression of the hindgut during embryogenesis and they mostly affect women in the third to sixth decade of life; rectal GIST account for 5% of all GIST. Both conditions may present with nonspecific symptoms such as pelvic or anal pain, gastrointestinal bleeding or weight loss. Tailgut cysts are usually benign, but malignant lesions tend to be symptomatic and are only detected at a later stage of development. Rectal GIST have a poor overall prognosis and the rate of tumor rupture is more than four times that of nonrectal GIST. There are several treatment options, including radical resection with en-bloc excision of the mass, local excision, low anterior resection, abdominoperineal excision of the rectum (APER) and pelvic exenteration. The text discusses the advantages of robotic surgery compared to traditional open and laparoscopic methods, such as shorter length of hospital stay and faster recovery time. In addition, robotic surgery offers a particularly useful minimally invasive option for the removal of these tumors in difficult pelvic anatomy.
... The para-sacral or trans-sacral approaches are classic techniques in the treatment of other rectal pathologies, and they have also been used for the resection of rectal GISTs (82,83). They may be of special interest when the tumor is voluminous, posterior, and reaches the superior part of the puborectalis muscle, especially if transanal surgery does not guarantee adequate resection. ...
... Trans-anal resection is one of the most minimally invasive approaches, but is limited by the distance from the dentate line (39). Trans-coccygeal excision is effective for the lower rectal GISTs, but has high postoperative morbidity, with fistulae occurring in 21% of patients (40). ...
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 withKITmutation genotype. However, theKITmutation 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,KITmutation analysis before IM treatment is important. This review summarizes current treatment strategies for rectal GIST.
... In 57 patients who underwent R0 resection, lymph node metastasis was reported as 8.8%. [65] The published case reports [43,48,50,[66][67][68][69] and series [15,23,25,27,[70][71][72][73][74] are summarized in Supplementary tables 1 and 2, respectively, in terms of management modalities used, details of the surgical procedure, radiotherapy treatment doses and fields, and outcomes if available. Taking the literature into consideration, although there is no accepted guideline, elective lymph node irradiation can be omitted in rectal GIST cases. ...
Objective: Gastrointestinal stromal tumors (GISTs) are rare mesenchymal tumors of the gastrointestinal tract, which frequently cause intraabdominal metastases. The current standard of care is surgery for localized cases, and adjuvant imatinib is recommended for tumors with a high risk of recurrence. To date, radiotherapy has not been commonly accepted as a part of multimodality treatment approach other than palliation. However, recently published case reports and some small series suggest that radiotherapy is a valuable option for controlling locally progressive, drug-resistant disease. The aim of this review is to provide a viewpoint from a radiation oncologist concerning the management of GISTs, especially rectal GIST, and clarify the role and technical aspects of radiotherapy in the treatment approach.
Data Sources: A comprehensive search in PubMed using the keywords “radiotherapy for rectal GIST” and “rectal GIST” was undertaken. The literature search included the related articles after 1995.
Study Selection: The main articles including rectal GIST case reports and GIST series containing rectal cases were the primary references.
Results: Surgery is the mainstay of treatment. However, to date, radiotherapy is included in the multidisciplinary treatment strategy of rectal GISTs in some circumstances with palliative, adjuvant, or definitive intent using different treatment doses and fields.
Conclusions: Recently reported long-term local control rates indicate that GIST is a radiosensitive disease. This makes radiotherapy a valuable alternative in GIST management with curative intent, especially in patients who (1) cannot tolerate or are resistant to chemotherapy agents, (2) have an unresectable disease, (3) have a gross or microscopic residual disease after surgery, and (4) have a recurrent disease.
... Gastrointestinal stromal tumors (GISTs) are rare mesenchymal tumors of the gastrointestinal track that express the phenotype of the interstitial cells of Cajal and are commonly associated with mutations of the tyrosine kinase receptors c-KIT [1]. They mainly occur in the stomach (60%) and the small intestine (30%) [2]; other rare locations include the esophagus, colon, rectum (<5% of cases [3]), and extra gastrointestinal areas [4]. ...
We report a case of rectal gastrointestinal stromal tumor (GIST) that was treated with transanal minimally invasive surgery (TAMIS). The patient was a 63-year-old male who was found to have a tumor between the prostate and rectum on US. MRI showed a rectal tumor displacing the prostate ventrally. The submucosal tumor was approximately 2.5 cm in diameter in the left anterior wall of the lower rectum and the distal margin was 2 cm above the dentate line. Under a diagnosis of rectal GIST, local resection was performed by TAMIS, based on the relatively small size of the tumor and preservation of anal function. A transanal access device was inserted, and local resection of the tumor with full-thickness incision of the rectal wall was performed after creating a pneumorectum. The rectal wall defect was surgically closed. Rectal GIST is a relatively rare tumor of the gastrointestinal tract, and a tumor located in the lower rectum requires a conventional extended operation. TAMIS for a rectal GIST with careful consideration of both curative resection and preserving anal function seems safe and feasible using transanal total mesorectal excision (TaTME), which is progressively becoming more common.
A 68-year-old man was pointed out to have a tumor of about 5 cm in diameter in the lower rectum (3 cm above the anal verge) by a CT scan for an external injury. Although a biopsy was not effective for pathological diagnosis, colonoscopy, FDG-PET, and MRI scan strongly indicated that this rectal submucosal tumor might be a rectal gastro-intestinal stromal tumor (GIST) without distant metastasis. The tumor had protruded out of the rectal wall, and appeared to widely come into contact with the levator ani muscle. Robot-assisted rectal intersphincteric resection was performed for this case. Using the multi-joint function specific for the robotic surgery, an anus-preserving surgery was safely performed without breakage of the tumor's capsule. There was no postoperative complication after the surgery. Since the rectal tumor was pathologically proved to be a c-kit-positive GIST by immunohistochemistry, adjuvant therapy with imatinib was started according to the Japanese GIST Guideline. We conclude that the robotic surgery is effective for lower rectal GISTs, especially for the difficult cases (e.g., large tumor, narrow pelvis, male, and obese). It enables us to preserve the anal function and to perform safe and radical resection.
