Singhal Sanjeev, Singhal Anu, Tugnait Rahul, Varghese Vineet, Tiwari Bishwanath, Arora Pankaj K, Malik Pawan, Bharali Mriganka Deuri, Dhuria Ankur Subhash, Chauhan Pushkar, Singh Chandrakant, Ballani Amit, Panwar Vishnu
Department of Surgery, Northern Railway Central Hospital, New Delhi, India.
Case Rep Gastrointest Med. 2013;2013:934875. doi: 10.1155/2013/934875. Epub 2013 Mar 25.
Gastrointestinal stromal tumors or "GIST" are mesenchymal neoplasms expressing KIT(CD117) tyrosine kinase and showing the presence of activating mutations in KIT or PDGFR α (platelet-derived growth factor alpha). GIST of anal canal is an extremely rare tumor, accounting for only 3% of all anorectal mesenchymal tumors and 0.1-0.4% of all GIST. GIST with large tumor size and high mitotic activity are highly malignant, but the biological behavior of anorectal GIST is less clear. Abdominoperineal resection (APR) or conservative surgery is the best treatment option. Imatinib mesylate, a tyrosine kinase inhibitor, has shown promising results in its management. We present a case of anorectal GIST diagnosed by computed tomography (CT) scan, magnetic resonance imaging (MRI), and colonoscopy with biopsy. The patient underwent abdominoperineal resection (APR) and was confirmed on histopathology to have anal canal GIST with tumor size more than 5 cm in maximum dimension and mitotic figures more than 5/50 high power field (HPF). The CD117-immunoreactive score-was 3+ in spindled cells. Therefore the patient was put on adjuvant imatinib mesylate 400 mg daily.
胃肠道间质瘤(GIST)是一种间叶组织肿瘤,表达KIT(CD117)酪氨酸激酶,且KIT或血小板衍生生长因子α(PDGFRα)存在激活突变。肛管GIST是一种极其罕见的肿瘤,仅占所有肛肠间叶组织肿瘤的3%,占所有GIST的0.1 - 0.4%。肿瘤体积大且有丝分裂活性高的GIST具有高度恶性,但肛肠GIST的生物学行为尚不清楚。腹会阴联合切除术(APR)或保守手术是最佳治疗选择。甲磺酸伊马替尼,一种酪氨酸激酶抑制剂,在其治疗中已显示出有前景的结果。我们报告一例经计算机断层扫描(CT)、磁共振成像(MRI)及结肠镜活检确诊的肛管GIST病例。该患者接受了腹会阴联合切除术(APR),组织病理学证实为肛管GIST,最大径超过5 cm,有丝分裂象超过5/50高倍视野(HPF)。梭形细胞中CD117免疫反应评分为3 +。因此,该患者接受了每日400 mg甲磺酸伊马替尼辅助治疗。