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连接蛋白43阳性胃肠道间质瘤(GIST)的首次报道。

First report of connexin 43-positive gastrointestinal stromal tumor (GIST).

作者信息

Nikolić Marko, Boban Marko, Ljubicić Neven, Bekavac-Beslin Miroslav, Tomas Davor, Pezo-Nikolić Borka, Kulis Tomislav, Matejcić Aljosa, Hrabar Davor, Zokvić Edita

机构信息

Sestre milosrdnice University Hospital, Zagreb, Croatia.

出版信息

Acta Clin Croat. 2010 Sep;49(3):359-63.

Abstract

Gastrointestinal stromal tumors (GISTs) are mesenchymal neoplasms most frequently found in the stomach and presenting without symptoms or with unspecific ones such as hemorrhage and abdominal pain. The malignant potential of GIST is variable and there are several prognostic indexes for treatment and follow up. The superior diagnostic method is endoscopic ultrasound combined with fine needle aspiration biopsy (EUS-FNA) immunocytochemistry. Surgery is the preferable treatment option, while recurrent or advanced disease is best managed with thyrosine kinase inhibitors. We report a case of a 20-year-old man that presented with gastrointestinal bleeding and anemia. Upper gastrointestinal endoscopy detected a submucosal lesion and computerized tomography revealed a node near the liver. EUS-FNA immunocytochemistry found CD-117 positive cells suggestive of GIST and the patient was operated on. The diagnosis was confirmed by histopathology with immunostaining. Prognostic assessment was done according to tumor size and Ki-67 index with mitosis count on microscopy. Many studies have shown that tumors demonstrate an abnormal number, structure and occurrence of connexin proteins with altered connexin-mediated intercellular communication. Since a great deal of gastroenterological tumors express connexin 43, our aim was to find out whether it was present in GIST. Immunohistochemically, the tumor was positive for connexin 43, which was not previously described as typical. The early postoperative course was uneventful, free from complications. At three-year postoperative follow-up, the patient was subjectively well and without clinical signs of disease recurrence.

摘要

胃肠道间质瘤(GISTs)是一种间叶性肿瘤,最常见于胃部,通常无症状或仅有诸如出血和腹痛等非特异性症状。GIST的恶性潜能各异,有多种用于治疗和随访的预后指标。最佳诊断方法是内镜超声联合细针穿刺活检(EUS - FNA)免疫细胞化学检查。手术是首选的治疗方式,而复发性或晚期疾病则最好用酪氨酸激酶抑制剂进行治疗。我们报告一例20岁男性患者,其表现为胃肠道出血和贫血。上消化道内镜检查发现一个黏膜下病变,计算机断层扫描显示肝脏附近有一个结节。EUS - FNA免疫细胞化学检查发现CD - 117阳性细胞,提示为GIST,该患者接受了手术治疗。组织病理学免疫染色确诊了该诊断。根据肿瘤大小、Ki - 67指数以及显微镜下的有丝分裂计数进行预后评估。许多研究表明,肿瘤中连接蛋白的数量、结构和出现情况异常,连接蛋白介导的细胞间通讯也发生改变。由于大量胃肠肿瘤表达连接蛋白43,我们的目的是查明它是否存在于GIST中。免疫组织化学检查显示,该肿瘤连接蛋白43呈阳性,此前未将其描述为典型表现。术后早期过程顺利,无并发症。术后三年随访时,患者主观感觉良好,无疾病复发的临床迹象。

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