Ampullary gangliocytic paraganglioma with lymph node metastasis

Abstract Rationale: Gangliocytic paraganglioma (GP) is a rare tumor that mostly develops in the duodenum and is composed of the following 3 cell types: epithelioid endocrine, spindle-like, and ganglion-like cells. It manifests as symptoms such as abdominal pain, gastrointestinal bleeding, and weight loss; however, occasionally, it is incidentally detected on endoscopic or radiologic examinations. Although GP is usually benign, it can metastasize to the lymph nodes, and distant metastases have been reported in some cases. Patient concerns: A 46-year-old woman presented with anemia on health surveillance examination. She had no other specific symptoms, and her physical examination did not reveal any abnormal finding. Diagnosis: Endoscopic ultrasound-guided fine-needle aspiration biopsy was performed, and the endoscopist obtained samples from the inner side of the ampullary mass. Pathological examination suggested GP or a neuroendocrine tumor. Interventions: Initially, we planned transduodenal ampullectomy with lymph node excision. However, there was severe fibrosis around the duodenum, and an examination of a frozen biopsy sample from the periduodenal lymph node showed atypical cells in the lymph node. Therefore, we performed pylorus-preserving pancreaticoduodenectomy with lymph node dissection. Outcomes: The final pathological diagnosis was GP located in the ampulla of Vater. The GP showed lymphovascular and perineural invasion and invaded the duodenal wall. Furthermore, 4 out of 18 harvested lymph nodes showed metastasis. Lessons: We described a case of GP confined to the ampulla with regional lymph node metastasis and reviewed published literature on ampullary GP with lymph node metastasis.