Sugimoto Shinya, Takahashi Masazumi, Nakagawa Kazuya, Yabuno Taichi, Kito Fumihiko, Yoshida Sachiko
Department of Gastroenterological Surgery, Yokohama Municipal Citizen's Hospital.
Nihon Shokakibyo Gakkai Zasshi. 2011 Jan;108(1):68-73.
A 67-year-old man with von Recklinghausen disease (VRD) was found to have an ill-defined large mass in the ileocecal area on abdominal computed tomography when he was examined for abdominal pain in the right lower quadrant. Because of high serum C-reactive protein level and leukocytosis appendiceal tumor complicated by appendicitis was diagnosed. Due to the possibility of malignancy, standard cancer surgery was carried out to remove the mass, i.e., ileocecal resection together with dissection of the regional lymph nodes. The cecum was displaced by a remarkably enlarged appendix with a firm and thickened wall. The distal portion of the appendix was cystically dilated, forming an abscess. The pathological diagnosis was neurofibromatosis with mucus hyperplasia and abscess formation in the appendix. Neurofibroma of the appendix is very rare, even in patients with VRD. Although it is benign, prompt resection is recommended because of a high risk of appendicitis and malignant transformation.
一名67岁患有冯雷克林霍增氏病（VRD）的男性，因右下腹腹痛接受腹部计算机断层扫描检查时，发现回盲部有一个边界不清的大肿块。由于血清C反应蛋白水平升高和白细胞增多，诊断为阑尾肿瘤合并阑尾炎。鉴于存在恶性可能性，遂进行标准癌症手术切除肿块，即回盲部切除术并清扫区域淋巴结。盲肠被明显肿大、壁坚硬且增厚的阑尾推移。阑尾远端呈囊性扩张，形成脓肿。病理诊断为神经纤维瘤病，伴有阑尾黏液增生和脓肿形成。阑尾神经纤维瘤非常罕见，即使在VRD患者中也是如此。尽管它是良性的，但由于阑尾炎和恶性转化风险高，建议及时切除。
