Carney-Stratakis syndrome (CSS) is a familial syndrome characterized by gastrointestinal stromal tumors (GISTs) and paragangliomas, often at multiple sites. A 34-year-old woman who had undergone resection of gastric GISTs, liver metastases, and a retroperitoneal paraganglioma in her previous hospital was referred to our hospital due to recurrence after 5 years. She presented with two gastric GISTs, a liver tumor, and a peritoneal tumor. As molecular-targeted agents are reported to be ineffective against CSS-related GISTs, we selected surgical resection for the recurrence. We performed laparoscopic local gastrectomy, liver S7 subsegmentectomy, and peritoneal tumor resection. Pathological findings revealed multiple gastric GISTs with liver metastasis and a paraganglioma. The laparoscopic approach could be performed safely, less invasively, and it could be more effective in such cases. This is the first case report of laparoscopic resection for recurrent CSS-related GISTs and paragangliomas.
Keywords: Carney-Stratakis syndrome; laparoscopic surgery; recurrence of gastrointestinal stromal tumors.
© 2022 Asia Endosurgery Task Force and Japan Society of Endoscopic Surgery and John Wiley & Sons Australia, Ltd.