Primary linitis plastica of the rectum: Report of four cases and review of the literature

Primary linitis plastica of the rectum is a rare form of large bowel carcinoma, with only 30 cases having been reported in the English literature to date. Of significance is the fact that it carries a prognosis that is poor compared to other types of colorectal cancers. We report herein the cases of four patients with primary linitis plastica of the rectum who underwent surgery, followed by intensive postoperative chemotherapy using 5-fluorouracil (5-FU) derivatives in our institution. A review of the literature is presented with special reference to the diverse treatment and prognosis of this unusual disease. Although three of the patients died of recurrent cancer, one has survived for 5 years and 10 months despite advanced disease. This is only the third reported case of a patient with linitis plastica of the rectum achieving 5-year survival. We believe that this unexpected long survival could be related to the patient's extensive lymphadenectomy and intensive postoperative chemotherapy using cisplatin and 5-FU derivatives.