Robotic-assisted total gastrectomy in a teenager with CDH1 mutation: a case report

CDH1 mutation is a rare autosomal dominant mutation. Individuals with this mutation have a lifetime risk of up to 70 % for developing diffuse gastric cancer, often at a young age, and many undergo prophylactic total gastrectomies. We present the case of a teenage girl with a known CDH1 mutation, identified through family screening. Her family history was significant for diffuse gastric carcinoma, with multiple relatives, including her mother, having previously undergone total gastrectomies due to malignancy. On her first surveillance gastroscopy at age 13, multiple small foci of signet ring cells were discovered despite the absence of clinical symptoms. In collaboration with our adult thoracic surgical team, we conducted a 6-h robotic-assisted total gastrectomy, with Roux-en-Y reconstruction using 4 robotic ports (one of which was an umbilical 10 mm camera port) and one laparoscopic assistant port. Both the esophagojejunal and jejunojejunal anastomoses were create with a robotic stapler. The procedure was successful, and the patient had a favorable postoperative course. The patient resumed gradual enteral feeds post-op day 1 and was discharged home on post-op day 8 tolerating her regular diet. Final pathology confirmed the presence of scattered signet ring cell foci, with negative margins and lymph nodes. The patient continues to do well at one year follow-up, with ongoing nutritional and psychological support. This case is unique as, to the best of our knowledge, it represents one of only a handful of robotic-assisted total gastrectomies reported in pediatric patients worldwide. Robotic-assisted surgery seems to be a feasible option for teenagers who require a prophylactic total gastrectomy due to a CDH1 mutation.