Division of Gastroenterology and Hepatology, Department of Internal Medicine, Institute of Gastrointestinal Medical Instrument Research, Korea University College of Medicine, Seoul 02841, South Korea.
Division of HBP Surgery and Liver Transplantation, Department of Surgery, Korea University College of Medicine, Seoul 02841, South Korea.
World J Gastroenterol. 2018 Jan 28;24(4):537-542. doi: 10.3748/wjg.v24.i4.537.
We report our experience with a synchronous case of gastrointestinal stromal tumor (GIST) and intraductal papillary neoplasm of the bile duct (IPNB) in an elderly woman with neurofibromatosis type 1 (NF-1). A 72-year-old woman presented with a 2-mo history of right upper abdominal pain unrelated to diet and indigestion. Fourteen years earlier, she had been diagnosed with NF-1, which manifested as café au lait spots and multiple nodules on the skin. Computed tomography (CT) revealed a multilocular low-density mass with septation, and mural nodules in the right hepatic lobe, as well as a 1.7-cm-sized well-demarcated enhancing mass in the third portion of the duodenum. The patient subsequently underwent right hepatectomy and duodenal wedge resection. We present here the first report of a case involving a synchronous IPNB and GIST in a patient with NF-1. Our findings demonstrate the possibility of various tumors in NF-1 patients and the importance of diagnosis at an early stage.
我们报告了一例老年女性神经纤维瘤病 1 型（NF-1）合并胃肠道间质瘤（GIST）和胆管内乳头状肿瘤（IPNB）的同步病例。一名 72 岁女性因右上腹痛就诊，与饮食和消化不良无关，病程为 2 个月。14 年前，她被诊断为 NF-1，表现为咖啡牛奶斑和皮肤多发结节。计算机断层扫描（CT）显示右肝叶有多房低密度肿块伴分隔和壁结节，以及十二指肠第三段 1.7 厘米大小的边界清楚的强化肿块。随后患者接受了右半肝切除术和十二指肠楔形切除术。我们在此报告首例 NF-1 患者合并 IPNB 和 GIST 的病例。我们的发现表明 NF-1 患者可能存在多种肿瘤，早期诊断非常重要。
