Sawaki Akira
Department of Medical Oncology, Fujita Health University, Toyoake, Japan.
Transl Gastroenterol Hepatol. 2017 Dec 19;2:116. doi: 10.21037/tgh.2017.12.07. eCollection 2017.
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms that arise in the gastrointestinal tract and rarely elsewhere in the abdomen. GISTs that develop outside the digestive tract are called extra-GISTs (EGISTs). The incidence of EGISTs is reported to be approximately 10% of all GISTs, and the median age is younger than that of conventional GISTs. EGISTs have similar histology and immunohistochemical features as conventional GISTs, with the majority of them in the omentum and mesentery. Most GISTs harbor a kinase-activating mutation in either KIT or PDGFRA. For EGISTs, the incidence of this type of mutation is 40-50%, which is somewhat lower than for conventional GISTs. EGISTs may have a worse prognosis compared with conventional GISTs with high mitotic indices, large size, and distant metastasis including lymph node involvement. In large abdominal tumors, the visceral origin is almost impossible to discern.
胃肠道间质瘤（GISTs）是最常见的起源于胃肠道的间叶性肿瘤，很少发生于腹部其他部位。发生于消化道外的GISTs称为消化道外胃肠道间质瘤（EGISTs）。据报道，EGISTs的发病率约占所有GISTs的10%，其年龄中位数比传统GISTs小。EGISTs与传统GISTs具有相似的组织学和免疫组化特征，大多数位于大网膜和肠系膜。大多数GISTs在KIT或PDGFRA基因中存在激酶激活突变。对于EGISTs，这种类型突变的发生率为40%-50%，略低于传统GISTs。与具有高有丝分裂指数、体积大以及包括淋巴结受累在内的远处转移的传统GISTs相比，EGISTs的预后可能更差。在较大的腹部肿瘤中，几乎无法辨别其起源于内脏。
