Abstract
A 55-year-old woman with type 1 neurofibromatosis (NF-1) was referred to our hospital for a liver tumor that was observed during a medical check-up. Abdominal computed tomography (CT) showed a multilocular 22 × 20-cm cystic tumor compressing the diaphragm and kidney with a poorly defined hepatic border. A 3-cm hypervascular tumor was also noted in the duodenum. Upper gastrointestinal tract endoscopy showed a submucosal tumor in the anterior wall of the second portion of the duodenum, which was diagnosed as a gastrointestinal stromal tumor (GIST). The site corresponded to the hypervascular tumor seen on CT. The positional relationship between the duodenum and cystic tumor seen on CT suggested that the latter was a retroperitoneal tumor. While a definitive diagnosis was not made preoperatively, the possibility of hepatic metastases of the GIST was ruled out based on differences in characteristics. Intraoperatively, the retroperitoneal tumor showed extensive adhesion to the right hepatic lobe, and the posterior segment of the liver was concurrently resected. Partial duodenectomy was performed for the duodenal GIST. The retroperitoneal tumor was pathologically diagnosed as pheochromocytoma. There are few case reports of NF-1 with pheochromocytoma and GIST, and we report this patient with a discussion of the relevant literature.
