The surgical challenge of papillary neoplasia of the biliary tract

This study is a case report and literature review of the surgical approach to papillary lesions of the biliary tract exclusive of the ampulla of Vater. Papillary lesions of the bile ducts, exclusive of the ampulla of Vater, are distinctly uncommon but, because of their unpredictable and aggressive behavior, pose challenging problems for the surgeon. Including the present illustrative case description, an English language literature review was conducted to determine the number and clinical behavior of papillary lesions of the bile ducts, particularly the propensity for malignant transformation, and the most favorable surgical approach. In addition to the present case, 29 patients with papillary biliary lesions were found in the literature. Twenty-two patients had tumors in multiple locations in the biliary tract, 6 had isolated lesions in one hepatic duct, and 3 had diffuse papillomatosis. Overall, 15 of 30 patients (50%) died of their disease. Patients with solitary tumors faired best (5 of 6 long-term survivors), and patients with diffuse papillomatosis did worse (all 3 died). Characteristics of these lesions include a propensity for recurrence (15 of 30 patients), an abundant mucin production, and a tendency toward malignant change (eight of 30 patients). Papillary lesions prove difficult to treat because of their frequent multifocality, propensity to recur after surgical extirpation, and malignant potential. Surgical strategy should include liver resection for isolated tumors, close monitoring for recurrence, and reoperations as required to control tumor growth.