Synchronous multiple colonic adenocarcinomas arising in patient with neurofibromatosis type 1

ABSTRACT

We report a case of synchronous multiple colon adenocarcinomas in a patient with neurofibromatosis type 1 (NF1). NF1 is an autosomal dominant inherited disorder and patients with NF1 have high risk for both benign and malignant tumors. However, adenocarcinomas involving the colon have rarely been reported in patients with NF1. A 61-year-old man was referred for generalized peritonitis due to descending colon perforation. Left hemicolectomy was performed and pathologic examination showed four adenocarcinomas. Peritoneal nodules were confirmed as metastatic adenocarcinoma (pT4N1M1). The patient also had clinical features compatible with NF1 such as cafe au lait macules, axillary freckles, neurofibromas across the body, and Lisch nodules. Upon review of the literature, colon adenocarcinoma in patients with NF1 tends to occur in males and relatively young age groups, and is associated with advanced tumor stages and multiple colon cancers. To improve treatment outcome, early colonoscopic surveillance should be considered in patients with NF1.