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  • Surgery and targeted molecular therapy

    2018-10-22

    Surgery and targeted molecular therapy are the two gold standards for GISTs. Surgery is the first choice if a complete resection is possible. Imatinib is indicated for treating patients with KIT-positive (i.e., CD117-positive) unresectable and/or metastatic GISTs. For KIT-mutant proteins, imatinib is the choice drug and the response rate is nearly 70%, even in advanced disease. In our patient, the leiomyoma specimen obtained 20 years before was not available and the recurrence rate in this type of GIST would decrease as time progresses. Recurrent GIST in our patient is thus an uncertified diagnosis. With prolonged follow-up, it appears that almost all GISTs presenting with clinical symptoms or signs leading to treatment have the potential to behave in a malignant fashion. Based on tumor size and mitotic count in a consensus approach to GISTs, our GIST case appears to have a low risk of malignancy. The role of adjuvant treatment with imatinib after the complete resection of primary GIST tumors is unknown. The American College of Surgeons Oncology Group (ACOSOG) Z9001 Trial showed that imatinib significantly reduces the recurrence risk of a primary GIST that measures at least 3 cm (3% recurrence on imatinib vs. 17% without therapy at 12 months). We are inclined to think that the adjuvant imatinib treatment should be prescribed for patients with a tumor measuring 3.5 cm × 2.5 cm × 2.0 cm, although the drug is not sponsored by the national health insurance in Taiwan. Our patient preferred long-term follow-up rather than taking medications.
    Introduction Intussusception in adults is an unusual condition that contributes to about 5% of all cases of intestinal intussusception. Most cases are related to an identifiable lesion that is either benign or malignant. Surgery is the therapeutic method used. Almost all patients present with abdominal pain, and some of them have acute symptoms that need an emergent surgical intervention. Inflammatory fibroid Protease Inhibitor Library (IFP) as the cause of intussusception was first reported in six cases by Vanek in 1949, and so it is also called Vanek\'s tumor. IFP is a benign but rare tumor of the gastrointestinal tract. The most frequent anatomical site is the stomach, followed by the small intestine. Here we describe an adult intussusception secondary to an ileal IFP initially presenting with bloody stools.
    Case report A 55-year-old Taiwanese male was referred following bloody stools for 5 days. Dark-red blood mixing with loose stools was observed. Tracing his history, we found that he had experienced intermittent abdominal dullness in the past 3 weeks but he still had a fair appetite. At our hospital, abdominal physical examination did not reveal any masses or tenderness. He had stable blood pressure and normal body temperature. Gastroendoscopy and colonoscopy were both performed, but no obvious cause of bleeding was found. Laboratory examinations demonstrated a normal complete blood count. On abdomen computed tomography (CT) imaging, an ileoileal intussusception was suspected on account of the characteristic “bull\'s-eye sign” (Fig. 1). During exploratory laparotomy, an ileoileal intussusception was identified 50 cm proximal to the ileocecal valve (Fig. 2). Segmental resection of the affected ileum was performed followed by end-to-end anastomosis. The excised ileum was 14 cm in length. The intestinal wall was congested and edematous. A 5.1 × 2.4 × 1.4 cm ulcerative sessile polypoid mass was confined to the excised ileum (Fig. 3). On microscopic examination, the tumor was characterized by vascular and fibroblastic proliferation in a loosely-textured or whorl-like arrangement around the vessels, demonstrating a polymorphic inflammatory response, and prominent eosinophilia (Fig. 4). Marked coagulation necrosis was also present. The spindle cells were positive for CD34 and bcl-2 on immunostaining. The morphology and immunohistochemical appearance taken together, the diagnosis was consistent with IFP.