Tomihara Hideo, Hashimoto Kazuhiko, Wakasa Tomoko, Ishikawa Hajime, Tsujimoto Tomoyuki, Gakuhara Atsushi, Fukuda Shuichi, Ohta Katsuya, Kitani Kotaro, Hida Jin-Ichi, Ohta Yoshio, Yukawa Masao
Department of Surgery, Kindai University Nara Hospital, Otoda-cho 1248-1, Ikoma, Nara, 630-0293, Japan.
Department of Pathology and Laboratory, Kindai University Nara Hospital, Otoda-cho 1248-1, Ikoma, Nara, 630-0293, Japan.
Surg Case Rep. 2020 Oct 27;6(1):274. doi: 10.1186/s40792-020-01055-w.
Gallbladder neuroendocrine tumors (GB-NETs) are extremely rare, representing only 0.5% of all NETs because no neuroectodermal cells are present in the gallbladder. In 2019, the World Health Organization updated the classification of NETs based on their molecular differences. The mutation status of DAXX and ATRX has been added to the criteria for well-differentiated NETs.
A 50-year-old man presented to our hospital for further examination of a gallbladder polyp. He had no right quadrant pain, fever, jaundice, weight loss, or carcinoid syndrome-related symptoms. The patient hoped to avoid cholecystectomy. During the 3-year observation period, the polyp gradually increased in size from 8.3 to 9.9 mm. He decided to undergo surgery, and whole cholecystectomy was successfully performed. Immunohistochemical staining revealed positivity for chromogranin A, synaptophysin, and CD56. The Ki-67 index was < 3%. Taken together, these results led to a diagnosis of a grade 1 GB-NET. We also performed immunohistochemical staining of DAXX and ATRX, which revealed that DAXX protein expression was negative. The patient's postoperative course was uneventful, and he developed no recurrence for 8 years after surgery.
We experienced a very rare case of GB-NET. Obtaining a correct preoperative diagnosis is quite difficult at the first evaluation. A GB-NET should be considered as a differential diagnosis of gallbladder tumors.
胆囊神经内分泌肿瘤(GB-NETs)极为罕见,仅占所有神经内分泌肿瘤的0.5%,因为胆囊中不存在神经外胚层细胞。2019年,世界卫生组织根据分子差异更新了神经内分泌肿瘤的分类。DAXX和ATRX的突变状态已被纳入高分化神经内分泌肿瘤的诊断标准。
一名50岁男性因胆囊息肉来我院进一步检查。他没有右下腹疼痛、发热、黄疸、体重减轻或类癌综合征相关症状。患者希望避免胆囊切除术。在3年的观察期内,息肉大小从8.3毫米逐渐增加到9.9毫米。他决定接受手术,成功进行了全胆囊切除术。免疫组化染色显示嗜铬粒蛋白A、突触素和CD56呈阳性。Ki-67指数<3%。综合这些结果,诊断为1级GB-NET。我们还对DAXX和ATRX进行了免疫组化染色,结果显示DAXX蛋白表达为阴性。患者术后恢复顺利,术后8年未复发。
我们遇到了一例非常罕见的GB-NET病例。在首次评估时很难获得正确的术前诊断。GB-NET应被视为胆囊肿瘤的鉴别诊断之一。