Periportal Paraganglioma: A Rare Cause of Obstructive Jaundice and Gastroesophageal Varices.

Paragangliomas are rare neuroendocrine tumors arising from extra-adrenal chromaffin cells. Biliary tropism is exceptionally rare and can mimic primary biliary tract malignancies, thus presenting a diagnostic challenge. We describe a rare case of periportal paraganglioma presenting with obstructive jaundice and gastric and duodenal varices. Similar to other reported primary hepatic paragangliomas, this tumor was nonfunctioning, and there was no evidence of metastatic disease. The patient underwent portal vein stenting to relieve portal hypertension after failed laparoscopic resection because of high vascularity. He continues to follow-up with our clinic for serial blood tests and imaging and remains asymptomatic. This case highlights the importance of considering paraganglioma in the differential diagnosis of biliary tract masses.