Hassan I, You N, Dozois EJ, Shayyan R, Smyrk TC, Okuno SH, Donohue JHClinical, pathologic, and immunohistochemical characteristics of gastrointestinal stromal tumors of the colon and rectum: implications for surgical management and adjuvant therapies. Dis Colon Rectum 49: 609-615
This study was designed to review the clinical characteristics of surgically treated gastrointestinal stromal tumors of the colon and rectum, evaluate their immunohistochemical and pathologic features based on the current National Institutes of Health criteria, and correlate clinicopathologic findings with the subsequent clinical course.
Patient and disease characteristics at presentation, pathologic features, surgical management, and clinical outcomes of 18 patients with gastrointestinal stromal tumors (4 colon and 14 rectum) diagnosed and primarily treated at our institution between 1979 and 2004 were evaluated.
Tumors were classified on basis of size and mitotic rate according to current National Institutes of Health recommendations: 67 percent (n = 12) were high-risk, 5 percent (n = 1) were intermediate-risk, 17 percent (n = 3) were low-risk, and 11 percent (n = 2) were very low-risk gastrointestinal stromal tumors. Fifteen of 18 tumors were KIT-positive. The three KIT-negative tumors were platelet-derived growth factor receptor alpha positive. All patients with colonic gastrointestinal stromal tumors (n = 4) underwent segmental resection, whereas patients with rectal gastrointestinal stromal tumors had local excision (n = 5) or radical resection (n = 9). Sixty-six percent (8/12) of patients with high-risk colorectal gastrointestinal stromal tumors developed metastases. None of the patients (n = 6) with intermediate-risk, low-risk, or very low-risk gastrointestinal stromal tumors died of their disease after a median follow-up of 65 (range, 15-266) months.
The majority of gastrointestinal stromal tumors of the colon and rectum are high-risk. Patients with high-risk colorectal gastrointestinal stromal tumors have a significant likelihood of developing metastases that is associated with poor prognosis. These patients need to be closely followed for an extended period and should be considered for adjuvant therapy with tyrosine kinase inhibitors.
"Firstly, metastases are extremely rare in the locoregional lymph nodes, and secondly, GISTs typically show a tendency to grow away from the intestinal lumen (2). So for the surgical management of a large GIST arising in the lower rectum, radical surgery, including LAR and APR with TME, may have little benefit (15). A previous study found that GISTs >5 cm in diameter that were removed by APR, LAR or local excision demonstrated no significant differences with regard to survival. "
[Show abstract][Hide abstract] 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.
"The sigmoid colon was also the most common site in a study by Chen et al. . However, in a study by Hassan et al , three of four cases were found in the right-sided colon. Shonaka et al. , by analyzing case reports of Japanese patients with colonic GISTs, found that six of 14 cases of colonic GIST developed in the sigmoid colon. "
[Show abstract][Hide abstract] ABSTRACT: Gastrointestinal stromal tumors (GISTs) developing in the colon are rare, accounting for <5 % of all GISTs. There are few data on the clinical efficacy of tyrosine kinase inhibitors in colonic GISTs. We report here on an 80-year-old male patient with advanced GIST of the transverse colon. The patient underwent palliative resection of the primary tumor because the disease was associated with multiple liver metastases and peritoneal dissemination. Immunohistochemical analysis of the surgical specimens showed KIT and CD34 expression. Sequence analysis revealed that the tumor harbored deletion mutation at codons 557-558 in exon 11 of the c-kit gene. A diagnosis of colonic GIST was made. The patient postoperatively underwent imatinib therapy for the remaining metastatic tumors. Imatinib therapy induced a cyst-like appearance of the liver metastases and stabilized the disease. In the present case, c-kit gene analysis was found to be clinically helpful for validating the diagnosis and therapeutic decision making for this rare disease.
Clinical Journal of Gastroenterology 04/2013; 6(2):116-121. DOI:10.1007/s12328-013-0365-2
"The commonest symptoms of gastric GISTs are hemorrhage and pain [7, 10]. Most colon GISTs are asymptomatic and detected incidentally . It is difficult to predict their metastatic potential because malignity does not have any obvious clinical and pathological findings . "
[Show abstract][Hide abstract] ABSTRACT: The most common tumors derived from the mesenchyme of the gastrointestinal system are stromal tumors. These tumors are typically seen in the stomach and small intestine and less frequently in the colon, rectum and esophagus and are very rarely located outside the gastrointestinal system. Cure is provided with complete surgical resection with resection borders free of tumor. Tumor size, mitotic index, localization, CD117 and CD34 negativity in immunohistochemical studies, mucosal ulceration and presence of necrosis help to predict recurrence of the illness and patient survival. In high-risk gastrointestinal stromal tumors (GISTs) there is an increased rate of recurrence and shortened survival despite complete surgical resection. Thus patients with a high-risk GIST should be given adjuvant therapy with imatinib mesylate. Sunitinib maleate is another FDA-approved agent only for cases who cannot tolerate imatinib or who are resistant to it. Herein we present three cases with GISTs in different locations of the gastrointestinal system with a review of the relevant literature.
Case Reports in Gastroenterology 07/2010; 4(2):250-260. DOI:10.1159/000319167
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