Xu Jian-Xia, Gu Meng-Yin, Wang Yi-Hua, Huang Xiao-Shan, Duan Jia-Qi, Zheng Xiao-Zhong, Chen Xi-Xi, Yu Ri-Sheng, Xiang Jun-Yi
Department of Radiology, The Second Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou, Zhejiang, China.
Department of Radiology, The First People's Hospital of Wenling, Wenling, Zhejiang, China.
Front Oncol. 2025 Jul 29;15:1638850. doi: 10.3389/fonc.2025.1638850. eCollection 2025.
Pancreatic extra-gastrointestinal stromal tumors (pEGISTs) are exceedingly rare, accounting for less than 5% of all extra-gastrointestinal stromal tumors (EGISTs). We report a case involving a 62-year-old woman who presented with a hypervascular pancreatic mass. Postoperative histopathological examination confirmed the diagnosis of a gastrointestinal stromal tumor (GIST). The neoplastic cells exhibited a spindle-shaped morphology without evident necrosis on histologic sections. Immunohistochemical analysis demonstrated strong, diffuse positivity for CD117, DOG-1, and CD34. Notably, the imaging characteristics of this pEGISTs displayed several distinctive features, including intralesional "island-like" enhancement, peripheral nodular enhancement, and prominent vascular "staghorn" patterns. Although histopathology remains the gold standard for definitive diagnosis of pEGISTs, correlating clinical presentation with these unique radiologic features can substantially improve diagnostic accuracy for radiologists. Additionally, we provide a comprehensive review and discussion of the current literature on the clinical and imaging hallmarks of pEGISTs.
胰腺外胃肠道间质瘤(pEGISTs)极为罕见,占所有胃肠道外间质瘤(EGISTs)的比例不到5%。我们报告一例病例,患者为一名62岁女性,表现为胰腺富血供肿块。术后组织病理学检查确诊为胃肠道间质瘤(GIST)。肿瘤细胞在组织学切片上呈梭形形态,无明显坏死。免疫组化分析显示CD117、DOG-1和CD34呈强弥漫阳性。值得注意的是,该pEGISTs的影像学特征表现出几个独特特点,包括瘤内“岛状”强化、周边结节状强化以及显著血管“鹿角”样形态。虽然组织病理学仍然是pEGISTs确诊的金标准,但将临床表现与这些独特的放射学特征相结合,可显著提高放射科医生的诊断准确性。此外,我们对目前关于pEGISTs临床和影像学特征的文献进行了全面综述和讨论。