Sugarbaker P H, Kern K, Lack E
Emory University School of Medicine, Atlanta, Georgia 30322.
Dis Colon Rectum. 1987 Oct;30(10):772-9. doi: 10.1007/BF02554625.
Pseudomyxoma peritonei is an unusual malignancy arising from the goblet cells of the large bowel or appendix. Its characteristic clinical features are low histologic grade of malignancy and widespread dissemination throughout the abdominal cavity. In a clinical study of 14 patients, the most common initial symptoms were abdominal distention or right lower quadrant pain suggestive of appendicitis. All patients underwent radical procedures in an attempt to surgically remove all gross disease from the abdomen. Six patients had small-bowel obstruction and five of these had bowel function restored. All of eight patients had relief from bulky intra-abdominal tumors. Six cycles of intraperitoneal 5-FU and three doses of mitomycin C were used following cytoreductive surgery in seven patients. Five of these seven patients are disease free following staging by celiotomy with two- to four-year follow-up. This new treatment strategy, designed to cure some patients with pseudomyxoma peritonei, has given favorable results in a disease that previously had a uniformly lethal outcome.
腹膜假黏液瘤是一种起源于大肠或阑尾杯状细胞的罕见恶性肿瘤。其特征性临床特点是恶性组织学分级低且在腹腔内广泛播散。在一项针对14例患者的临床研究中，最常见的初始症状是提示阑尾炎的腹胀或右下腹疼痛。所有患者均接受了根治性手术，试图通过手术切除腹部所有肉眼可见的病灶。6例患者出现小肠梗阻，其中5例肠道功能恢复。8例患者的腹腔巨大肿瘤均得到缓解。7例患者在减瘤手术后接受了6个周期的腹腔内5-氟尿嘧啶治疗和3剂丝裂霉素C。在接受剖腹探查分期并进行2至4年随访后，这7例患者中有5例无疾病。这种旨在治愈一些腹膜假黏液瘤患者的新治疗策略，在这种以前预后一致很差的疾病中取得了良好效果。
