Sato Ayami, Sato Yu, Hiruta Nobuyuki, Oshiro Takashi, Yoshida Yutaka, Urita Tasuku, Kitahara Tomoaki, Kadoya Kengo, Nabekura Taiki, Moriyama Yuki, Okazumi Shinichi
Department of Surgery, Toho University Sakura Medical Center, 564-1 Shimoshizu, Sakura, Chiba, 285-8741, Japan.
Department of Surgical Pathology, Toho University Sakura Medical Center, Chiba, Japan.
Surg Case Rep. 2022 Aug 5;8(1):151. doi: 10.1186/s40792-022-01509-3.
Primary cancer of the appendix, especially signet-ring cell carcinoma, is an uncommon disease, and it is rarely suspected before surgery. Diffuse intestinal ganglioneuromatosis that is not associated with neurofibromatosis-1 or multiple endocrine neoplasia 2b is also rare. The most frequent symptoms caused by it are changes in bowel habits, abdominal pain, and occlusive episodes.
The patient was a 48-year-old woman who had a month-long history of chronic abdominal pain, fullness, constipation, and diarrhoea. Enhanced computed tomography showed a 100-mm irregular swelling in the appendix and thickening of the appendiceal wall with cystic dilatation. Based on a preoperative diagnosis of appendiceal cancer, the patient underwent laparoscopic ileocecal resection with D3 lymph node dissection. Pathological diagnosis revealed a signet-ring cell carcinoma of the appendix with ganglioneuromatosis. The patient completed four courses of capecitabine plus oxaliplatin (CAPEOX) as postoperative adjuvant chemotherapy, and 23-month postoperative outcome was noneventful without recurrence.
We report a signet-ring cell carcinoma of the appendix that was detected early because of its presence with ganglioneuromatosis.
阑尾原发性癌，尤其是印戒细胞癌，是一种罕见疾病，术前很少被怀疑。不伴有1型神经纤维瘤病或2b型多发性内分泌肿瘤的弥漫性肠道神经节瘤病也很罕见。其最常见的症状是排便习惯改变、腹痛和梗阻发作。
患者为一名48岁女性，有长达一个月的慢性腹痛、腹胀、便秘和腹泻病史。增强计算机断层扫描显示阑尾有一个100毫米的不规则肿胀，阑尾壁增厚并伴有囊性扩张。基于术前阑尾癌的诊断，患者接受了腹腔镜回盲部切除术及D3淋巴结清扫术。病理诊断显示为阑尾印戒细胞癌伴神经节瘤病。患者完成了四个疗程的卡培他滨加奥沙利铂（CAPEOX）术后辅助化疗，术后23个月情况良好，无复发。
我们报告了一例因伴有神经节瘤病而早期被发现的阑尾印戒细胞癌。
