A 67-year-old male presenting with right lower quadrant abdominal pain was referred to our hospital. On physical examination, an approximately 10 cm mass was palpated in the right lower quadrant area with mild tenderness. A computed tomography of his abdomen revealed a well-demarcated and enhanced tumor in the appendix, measuring approximately 5.6x5.0x8.0 cm. At laparotomy, a solid appendiceal mass was removed by right hemicolectomy. The cut section of the appendix showed that the tumor was a 6.0x4.0x3.0 cm polypoid lesion with hemorrhage from the midportion of the appendix (Figure 1). Histological examination revealed that the tumor was composed of spindle cells with a mitotic count of <5 mitoses/50 high power fields. Immunohistochemical study revealed positive staining for CD117 (Figure 2). Based on the above findings, the tumor was diagnosed as a gastrointestinal stromal tumor (GIST) with intermediate-grade malignancy originating from the appendix. The patient had an uneventful postoperative course, and there was no evidence of recurrence in the six months after his surgery.Gastrointestinal stromal tumors can occur anywhere throughout the GI tract, but are most commonly found in the stomach or small intestine (1-3). Appendiceal GISTs are extremely rare, with only eight cases reported to date (3-6). Based on the previously reported eight cases and our one case, the patients with appendiceal GISTs included six (67%) males and three (33%) females, with a median age of 66 years (range: 56-78 years). Among the five patients who had clinical symptoms, four patients had appendicitis-like symptoms in the absence of histologic evidence of acute appendicitis, suggesting that the symptoms were caused by the tumor. The asymptomatic tumors were detected incidentally during surgery for other diseases or at autopsy. Five patients were affected by other malignant tumors. The presenting malignancies included pulmonary adenocarcinoma, malignant gastric GIST, endometrial carcinoma, urinary bladder carcinoma, and mantle cell lymphoma. One patient had neurofibromatosis type 2. The tumors were located in the proximal (n=1, 11%), mid (n=6, 67%) and tip (n=2, 22%) por-
Gastrointestinal stromal tumor of the appendix
Apendiksin gastrointestinal stromal tümörü
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