Department of General and Thoracic Surgery, University Hospital of Giessen, Rudolf-Buchheim-Street 7, 35392 Giessen, Germany.
World J Surg Oncol. 2010 Jun 2;8:47. doi: 10.1186/1477-7819-8-47.
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the digestive tract. The majority of GISTs is located in the stomach. Only 3-5% of GISTs are located in the duodenum associated with an increased risk of gastrointestinal bleeding as primary manifestation. With response rates of up to 90%, but complications like bleeding due to tumor necrosis in 3%, imatinib mesylate dramatically altered the pre- and postoperative therapy for GIST patients.
A 58-year-old female patient presented with acute upper gastrointestinal bleeding 2 weeks after a giant GIST of the duodenum had been diagnosed. Neoadjuvant imatinib therapy had been initiated to achieve a tumor downsizing prior to surgery. During emergency laparotomy a partial duodenopancreatectomy was performed to achieve a complete resection of the mass. Histology revealed a high-malignancy GIST infiltrating the duodenal wall. Adjuvant imatinib therapy was initiated. At follow-up (19 months) the patient is still alive and healthy.
Giant GISTs of the duodenum are rare and - in contrast to other localizations - harbour a higher risk of serious bleeding as primary manifestation. Tumor necrosis and tumor bleeding are rare but typical adverse effects of imatinib therapy especially during treatment of high-malignancy GIST. In GIST patients with increased risk of tumor bleeding neoadjuvant imatinib therapy should thoroughly be performed during hospitalization. In cases of duodenal GIST primary surgery should be considered as treatment alternative.
胃肠道间质瘤(GIST)是最常见的消化道间叶性肿瘤。大多数 GIST 位于胃内。只有 3-5%的 GIST 位于十二指肠,其以胃肠道出血为主要表现,存在较高的出血风险。甲磺酸伊马替尼的反应率高达 90%,但 3%的患者因肿瘤坏死而发生出血等并发症,这显著改变了 GIST 患者的术前和术后治疗。
一名 58 岁女性患者在诊断为十二指肠巨大 GIST 后 2 周出现急性上消化道出血。在手术前已开始新辅助伊马替尼治疗以实现肿瘤缩小。在紧急剖腹手术中进行了胰十二指肠部分切除术以实现肿块的完全切除。组织学显示高恶性 GIST 浸润十二指肠壁。开始辅助伊马替尼治疗。随访(19 个月)时,患者仍然存活且健康。
十二指肠巨大 GIST 较为罕见,与其他部位相比,其以严重出血为主要表现的风险更高。肿瘤坏死和肿瘤出血是伊马替尼治疗的罕见但典型的不良反应,尤其是在治疗高度恶性 GIST 时。对于有肿瘤出血风险增加的 GIST 患者,应在住院期间彻底进行新辅助伊马替尼治疗。对于十二指肠 GIST,手术应被视为替代治疗方法。