Popivanov Georgi, Tabakov Mihail, Mantese George, Cirocchi Roberto, Piccinini Irene, D'Andrea Vito, Covarelli Piero, Boselli Carlo, Barberini Francesco, Tabola Renata, Pietro Ursi, Cavaliere Davide
Military Medical Academy, Clinic of Endoscopic, Endocrine surgery and Coloproctology, Sofia, Bulgaria.
University Hospital Sv. Ivan Rilski, Surgical Clinic, Sofia, Bulgaria.
Transl Gastroenterol Hepatol. 2018 Sep 21;3:71. doi: 10.21037/tgh.2018.09.04. eCollection 2018.
Gastrointestinal stromal tumors (GIST) are the most frequent mesenchymal tumours in the digestive tract. The duodenal GIST (dGIST) is the rarest subtype, representing only 4-5% of all GIST, but up to 21% of the resected ones. The diagnostic and therapeutic management of dGIST may be difficult due to the rarity of this tumor, its anatomical location, and the clinical behavior that often mimic a variety of conditions; moreover, there is lack of consent for their treatment. This study has evaluated the scientific literature to provide consensus on the diagnosis of dGIST and to outline possible options for surgical treatment.
An extensive research has been carried out on the electronic databases MEDLINE, Scopus, EMBASE and Cochrane to identify all clinical trials that report an event or case series of dGIST.
Eighty-six studies that met the inclusion criteria were identified with five hundred forty-nine patients with dGIST: twenty-seven patients were treated with pancreatoduodenectomy and ninety-six with only local resection (segmental/wedge resections); in four hundred twenty-six patients it is not possible identify the type of treatment performed (pancreatoduodenectomy or segmental/wedge resections).
dGISTs are a very rare subset of GISTs. They may be asymptomatic or may involve symptoms of upper GI bleeding and abdominal pain at presentation. Because of the misleading clinical presentation the differential diagnosis may be difficult. Tumours smaller than 2 cm have a low biological aggressiveness and can be followed annually by endoscopic ultrasound. The biggest ones should undergo radical surgical resection (R0). In dGIST there is no uniformly adopted surgical strategy because of the low incidence, lack of experience, and the complex anatomy of the duodenum. Therefore, individually tailored surgical approach is recommended. R0 resection with 1-2 cm clear margin is required. Lymph node dissection is not recommended due to the low incidence of lymphatic metastases. Tumor rupture should be avoided.
胃肠道间质瘤（GIST）是消化道最常见的间叶组织肿瘤。十二指肠GIST（dGIST）是最罕见的亚型，仅占所有GIST的4%-5%，但在所有切除的GIST中占比高达21%。由于这种肿瘤罕见、解剖位置特殊以及临床表现常与多种疾病相似，dGIST的诊断和治疗管理可能具有挑战性；此外，对于其治疗也缺乏共识。本研究评估了科学文献，以就dGIST的诊断达成共识，并概述手术治疗的可能选择。
对电子数据库MEDLINE、Scopus、EMBASE和Cochrane进行了广泛检索，以识别所有报告dGIST事件或病例系列的临床试验。
确定了86项符合纳入标准的研究，共549例dGIST患者：27例接受了胰十二指肠切除术，96例仅接受了局部切除（节段性/楔形切除）；426例患者无法确定所进行的治疗类型（胰十二指肠切除术或节段性/楔形切除）。
dGIST是GIST中非常罕见的一个子集。它们可能无症状，也可能在出现时表现为上消化道出血和腹痛症状。由于临床表现具有误导性，鉴别诊断可能困难。小于2 cm的肿瘤生物学侵袭性低，可每年通过内镜超声进行随访。较大的肿瘤应接受根治性手术切除（R0）。由于发病率低、经验不足以及十二指肠解剖结构复杂，dGIST尚无统一采用的手术策略。因此，建议采用个体化的手术方法。需要进行切缘1-2 cm的R0切除。由于淋巴转移发生率低，不建议进行淋巴结清扫。应避免肿瘤破裂。
