Machado Norman Oneil, Chopra Pradeep J, Al-Haddabi Ibrahim Hassan, Al-Qadhi Hani
Department of Surgery, Sultan Qaboos University Hospital. Muscat, Oman.
JOP. 2011 Mar 9;12(2):194-9.
Duodenal gastrointestinal stromal tumors (GISTs) are uncommon and constitute a relatively small subset of GISTs which presents a unique dilemma having various surgical options. A case of a large ulcerating duodenal GIST arising from the second and third parts of the duodenum and involving the pancreas which was managed by a Whipple resection is presented. The literature is also reviewed to present the current status on surgical options, outcome, prognostic indicators and the role of imatinib mesylate in its management.
A 58-year-old patient presented with acute gastrointestinal bleeding which was diagnosed to be due to a duodenal GIST following CT scan and endoscopic biopsy. The mass which measured about 10x9 cm originated from the 2nd part and extended into the 3rd part of the duodenum. He underwent a Whipple resection, and histopathology confirmed a duodenal GIST having a greater than 10 mitotic count per fifty high power field and areas of necrosis. Postoperatively, he received imatinib mesylate 400 mg bid; however, 4 months later, he presented with multiple disseminated peritoneal metastases and succumbed to the disease 2 months later.
GISTs of the duodenum which are small in size and do not involve the papilla of Vater are better resolved using a limited resection of the duodenum since the outcome in terms of operative risk or disease recurrence is not influenced in these cases. However, large tumors with more extensive involvement would require a pancreaticoduodenectomy to achieve adequate tumor clearance. Even though duodenal GISTs have a relatively better prognosis as compared to GISTs at other sites, their aggressiveness ranges from small indolent tumors to aggressive sarcomas. Following tumor resection, a recurrence rate of about 40% has been reported. A more favorable prognosis in duodenal GISTs is attributed to a lower prevalence of P53 loss, the duodenal location of the tumor, a smaller size of the lesion and a low mitotic count. Imatinib mesylate is reported to play a role in neoadjuvant therapy as well as in the management of metastatic and recurrent disease, although some of these tumors may fail to respond.
十二指肠胃肠道间质瘤（GIST）较为罕见，在GIST中所占比例相对较小，其治疗面临多种手术选择的独特困境。本文报道一例起源于十二指肠第二和第三部分、累及胰腺的巨大溃疡性十二指肠GIST，采用惠普尔手术进行治疗。同时对相关文献进行综述，以阐述手术选择、预后、预后指标以及甲磺酸伊马替尼在其治疗中的作用等方面的现状。
一名58岁患者因急性胃肠道出血就诊，经CT扫描和内镜活检诊断为十二指肠GIST。肿块大小约为10×9 cm，起源于十二指肠第二部并延伸至第三部。患者接受了惠普尔手术，组织病理学证实为十二指肠GIST，每50个高倍视野有超过10个有丝分裂象且存在坏死区域。术后，患者接受甲磺酸伊马替尼400 mg，每日两次治疗；然而，4个月后，患者出现多处弥漫性腹膜转移，2个月后死于该疾病。
对于体积较小且未累及十二指肠乳头的十二指肠GIST，采用有限的十二指肠切除术可取得较好疗效，因为在这些病例中，手术风险或疾病复发方面的结果不受影响。然而，对于累及范围更广的大肿瘤，则需要进行胰十二指肠切除术以实现充分的肿瘤清除。尽管与其他部位的GIST相比，十二指肠GIST的预后相对较好，但其侵袭性范围从惰性小肿瘤到侵袭性肉瘤不等。据报道，肿瘤切除后复发率约为40%。十二指肠GIST预后较好归因于P53缺失发生率较低、肿瘤位于十二指肠、病变体积较小以及有丝分裂象计数较低。据报道，甲磺酸伊马替尼在新辅助治疗以及转移性和复发性疾病的治疗中发挥作用，尽管其中一些肿瘤可能对此无反应。
