Zhang Cai-Qing, Lu DE-Gan, Liu Qing-Fa, Xiao Wei
Department of Respiratory Medicine, Shandong Provincial Qianfoshan Hospital Affiliated to Shandong University, Jinan, Shandong 250014, P.R. China.
Department of Respiratory Medicine, Qilu Hospital of Shandong University, Jinan, Shandong 250012, P.R. China.
Oncol Lett. 2016 May;11(5):3135-3138. doi: 10.3892/ol.2016.4344. Epub 2016 Mar 17.
Gastrointestinal stromal tumors (GISTs) are the most common type of mesenchymal tumor of the gastrointestinal tract. The stomach and small intestine are the most common sites of occurrence. GISTs are mesenchymal neoplasms originating from the interstitial cells of Cajal (ICCs), and are characterized by positivity for cluster of differentiation (CD) 117, also known as proto-oncogene c-Kit. While the majority of GISTs develop in the alimentary tract, in rare cases they may also be found in extragastrointestinal tissues. This type of GIST is known as an extragastrointestinal stromal tumor (EGIST). Despite the fact that EGISTs have been reported in the mesentery, omentum and retroperitoneum, primary intrathoracic EGISTs, arising from the pleura or lungs, are rare. The patient presented in the current study was a 40-year-old man, who presented with a cough and pyrexia, with pleural effusion on the left side. Multiple nodules throughout the parietal pleura were identified by thoracoscopy and a diagnosis of primary GIST of pleura was established, since they were positive for CD117 and discovered on GIST-1 and there was no evidence of gastrointestinal tumors. Subsequently, the patient was administered with imatinib and had no signs of disease recurrence 2 years later.
胃肠道间质瘤(GISTs)是胃肠道最常见的间叶性肿瘤类型。胃和小肠是最常见的发病部位。GISTs是起源于 Cajal 间质细胞(ICC)的间叶性肿瘤,其特征为对分化簇(CD)117呈阳性,CD117也被称为原癌基因c-Kit。虽然大多数GISTs发生于消化道,但在罕见情况下也可在胃肠道外组织中发现。这种类型的GIST被称为胃肠道外间质瘤(EGIST)。尽管已有报道EGISTs可发生于肠系膜、网膜和腹膜后,但起源于胸膜或肺的原发性胸腔内EGISTs却很罕见。本研究中的患者是一名40岁男性,表现为咳嗽和发热,左侧有胸腔积液。通过胸腔镜检查发现壁层胸膜上有多个结节,由于这些结节对CD117呈阳性且在GIST-1检测中被发现,并且没有胃肠道肿瘤的证据,因此确诊为原发性胸膜GIST。随后,该患者接受了伊马替尼治疗,两年后无疾病复发迹象。