Early onset colon cancer affected by Lynch syndrome

Abstract

Lynch syndrome is an autosomal dominant disorder in which one's DNA mismatch repair mechanism is impaired, resulting in an increased risk of developing colon and endometrial cancers, as well as other malignancies. With such an increased risk of colorectal cancer, patients diagnosed with Lynch syndrome should undergo colonoscopy screening every 1–2 years beginning at ages 20 to 25 or two to five years prior to earliest diagnosis of colorectal cancer in their family members. In patients with Lynch syndrome, colorectal cancer usually presents after the fourth decade of life. Few cases have been reported in pediatric patients with early onset colorectal cancer. We report here a 16-year-old patient who presented initially with symptoms characteristic of appendicitis. Following a CT scan suggesting perforated appendicitis, the patient was treated with intravenous antibiotics with a scheduled interval appendectomy three months later. Pathology reports from the interval appendectomy showed that the specimen contained a well-to-moderately differentiated adenocarcinoma. The patient then underwent robot assisted right hemicolectomy as definitive management, which demonstrated that the tumor originated in the cecum with invasion into the submucosa and focally infiltrating the muscularis propria. This case illustrates the need to be cognizant that cancer can present similarly to appendicitis, to be aware of the increasing incidence of colon cancer in younger patients, and to catalyze the acceptability of robotics for minimally invasive surgery. Keywords: Lynch syndrome, Pediatric colon cancer, Robotic colectomy