RESUMO
We report a 67-year-old woman who had stiff shoulders and anemia. Upper gastrointestinal endoscopy revealed a pedunclated nodular submucosal tumor with erosions and surface ulcers in the second portion of the duodenum. These endoscopic findings were thought to be characteristic of gangliocytic paraganglioma. CT scan revealed suspected lymph node metastasis, thus the tumor was resected with pancreaticoduodenectomy and was found to be a gangliocytic paraganglioma associated with lymph nodal metastasis. Due to the rarity of the disease there is no consensus regarding treatment. Although this tumor is considered benign, the possibility exists for regional lymph nodal spread. The treatment should be well planned with continuous careful evaluation.