Harhar Marouane, Harouachi Abdelhakim, Akouh Nada, Atmani Abderrahmane, Aabdi Houssam, Bouhout Tariq, Bennani Amal, Serji Badr, El Harroudi Tijani
Surgical Oncology Department, Regional Oncology Center, Mohammed VI University Hospital, Oujda, Morocco.
Mohammed First University Oujda, Faculty of Medicine and Pharmacy Oujda, Oujda, Morocco.
Ann Med Surg (Lond). 2022 Mar 29;77:103560. doi: 10.1016/j.amsu.2022.103560. eCollection 2022 May.
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the digestive tract.
54-year-old woman with a history of phyllodes tumor of the left breast he patient was admitted to our hospital for management of retroperitoneal soft tissue tumor, attached to the fourth portion of the duodenum, opposite the head of the pancreas. The patient underwent a large excision of the tumor, the duodenojejunal flexure, and the third and fourth portions of the duodenum along with the head of the pancreas. The histopathological examination confirmed the presence of a spindlecell mesenchymal proliferation. These cells do not express CD117, but they express DOG1. A PDGFRA mutation was identified later. The final diagnosis was duodenal GIST.
few cases of GIST in the fourth portion of the duodenum had been reported in the literature. PDGFRA mutation is identified as GISTs tumorigenesis to 15% of cases, and the diagnosis of GISTs is not based solely on the expression of the protein Kit.
the molecular biology examinations are very helpful in the direction of the correct diagnosis in case of negative staining for CD117.
胃肠道间质瘤（GISTs）是消化道最常见的间叶组织肿瘤。
一名54岁女性，有左乳腺叶状肿瘤病史。该患者因腹膜后软组织肿瘤入院治疗，肿瘤附着于十二指肠第四部分，位于胰头对面。患者接受了肿瘤、十二指肠空肠曲、十二指肠第三和第四部分以及胰头的大范围切除。组织病理学检查证实存在梭形细胞间叶组织增生。这些细胞不表达CD117，但表达DOG1。后来发现了血小板衍生生长因子受体α（PDGFRA）突变。最终诊断为十二指肠GIST。
文献中报道的十二指肠第四部分GIST病例很少。PDGFRA突变在15%的GIST病例中被确定为肿瘤发生的原因，GIST的诊断不能仅基于蛋白KIT的表达。
在CD117染色阴性的情况下，分子生物学检查对正确诊断非常有帮助。
