Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. These tumors tend to present most frequently in the stomach, followed by the small intestine. GISTs can also arise from the omentum, retroperitoneum, mesentery, or pleura and are termed extragastrointestinal stromal tumors (EGISTs) when they do so. EGISTs arising from the omentum are very rare. Due to the limited incidence of EGISTs in the omentum, the diagnostic criteria are not well established, and making a correct diagnosis may be difficult. In this report, we present a case of an EGIST of the omentum with peritoneal metastasis that was initially suspected to be an appendiceal mucinous carcinoma with carcinomatosis on positron emission tomography/computed tomography imaging.
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Surgery is the standard treatment for a primary gastrointestinal stromal tumor (GIST); however, surgical resection is often not curative, particularly for large GISTs. In the past decade, with imatinib mesylate (IM), management strategies for GISTs have evolved significantly, and now IM is the standard care for patients with locally advanced, recurrent or metastatic GISTs. Adjuvant therapy with imatinib was recently approved for use, and preoperative imatinib is an emerging treatment option for patients who require cytoreductive therapy. IM neoadjuvant therapy for primary GISTs has been reported, but there is no consensus on the dose of the drug, the duration of treatment and the optimal time of surgery. These are critical because drug resistance or tumor progression can develop with a prolonged treatment. This report describes two cases of large rectal malignant GISTs, for which a abdominoperineal resection was initially anticipated. The two patients received IM preoperative treatment; we followed-up with CT or magnetic resonance imaging to access the response. After 9 months of treatment, a multi-disciplinary consensus that maximal benefit from imatinib had been achieved was reached. We determined the best time for surgical intervention and successfully performed sphincter-preserving surgery before resistance to imatinib or tumor progression occurred. We believe that a multidisciplinary team approach, considerating the optimal duration of therapy and the timing of surgery, is required to optimize treatment outcome.
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Tumors of the small bowel are rare, accounting for about 3-6% of all gastrointestinal neoplasms, though they cover more than 90% of the intestinal surface. However, diagnosis and treatment are difficult and present an ongoing challenge for both gastrointestinal surgeons and gastroenterologists. The aim of this study was to investigate the clinical features of small bowel tumors.
Between November 1994 and November 2007, 81 patients underwent treatments for primary tumors in the jejuno-ileal region at the Department of Surgery, Kangnam St. Mary's Hospital, the Catholic University of Korea. A retrospective review of the patients' characteristics and variable tumor factors was performed.
The mean age of the patients was 53.2 years with 48 men and 33 women. The most common symptom was abdominal pain (59.3%), followed by bleeding (22.2%) and an abdominal mass (6.2%). We found that the patients with ileal tumors complained mainly of abdominal pain (72.9%) whereas the patients with jejunal tumors presented with gastrointestinal bleeding (36.4%) (P = 0.048). Seventy-six of the 81 patients (93.8%) had malignant tumors, including 40 (49.4%) gastrointestinal stromal tumors, 26 (32.1%) lymphomas and 5 (6.2%) adenocarcinomas. No postoperative mortalities were observed. The overall 5-year survival rate of the patients with malignant small bowel tumors was 31.8%.
Because the clinical features of a primary tumor of the small bowel are obscure and its diagnosis is difficult, maintaining a high degree of suspicion and recognizing the possibility of a primary small bowel tumor are important.
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