Abstract
The patient was an 18-year-old woman. At the age of 9 years, upper gastrointestinal endoscopy for anemia showed a submucosal gastric tumor, and partial gastrectomy was performed. The submucosal gastric tumor was diagnosed as a gastrointestinal stromal tumor (GIST). Positron emission tomography (PET) computed tomography (CT) showed novel tumors in the stomach and metastases to other organs. Partial gastrectomy at the age of 10 years, and partial resections of the stomach, liver, and duodenum at the age of 13 years were performed. PET-CT at the age of 18 years showed multiple new lesions in the stomach. At the same time, chest CT showed multiple circumscribed calcified tumors in the upper and middle lobes. Although no evidence of an extra-adrenal paraganglioma could be identified, two criteria of Carney's triad were satisfied. A third partial gastrectomy was performed, and the tumor was diagnosed as a GIST. In consideration of the multiple recurrences and her age, imatinib was started as adjuvant chemotherapy after surgery. Two years after surgery, the patient is under observation without recurrence or metastases.
