INTRADUCTAL PAPILLARY NEOPLASM OF THE BILE DUCT (IPNB): A RARE CASE FROM A WESTERN COUNTRY rita.banza@chleiria.min-saude.pt

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INTRADUCTAL PAPILLARY NEOPLASM OF THE BILE DUCT (IPNB): A RARE CASE FROM A WESTERN COUNTRY

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Mónica Laureano monica.laureano@ulsrl.min-saude.pt Unidade Local de Saúde da Região de Leiria Cirurgia Geral Leiria Portugal

Rui Caetano Oliveira rui.caetano@germanodesousa.com Centro de Anatomia Patológica Germano de Sousa Anatomia Patológica Coimbra Portugal

Ricardo Martins ricardo.martins@ulsrl.min-saude.pt Unidade Local de Saúde da Região de Leiria Cirurgia Geral Leiria Portugal

Miguel Coelho dos Santos Miguel.Coelho@ulsrl.min-saude.pt Unidade Local de Saúde da Região de Leiria Cirurgia Geral Leiria Portugal

Portugal

Poster Exhibition only

Intraductal Papillary Neoplasm of the Bile Duct (IPNB) is a rare bile duct neoplasm, more commonly observed in Far Eastern countries, where it is associated with hepatolithiasis and clonorchiasis infections. In contrast, it is very rare in Western countries. The most common clinical manifestations include intermittent abdominal pain, acute cholangitis, and jaundice.

This report describes a rare case of IPNB in a Western country. A 75-year-old woman presented with abdominal pain, cholestatic jaundice, anorexia, and weight loss of approximately one month’s duration. She denied any other symptoms. Clinical examination on admission revealed jaundice and abdominal pain in the right upper quadrant without tenderness. Computed tomography (CT) and magnetic resonance cholangiopancreatography (MRCP) demonstrated findings consistent with a type IIIA Klatskin tumor. The case was discussed at a multidisciplinary team meeting (MDTM), and the patient underwent resection of the main bile duct, right hepatectomy with cholecystectomy, lymphadenectomy of the hepatic pedicle and common hepatic artery, followed by Roux-en-Y hepaticojejunostomy to the left hepatic duct.

The postoperative course was uneventful and the patient was discharged on the 9th postoperative day. The postoperative histologic exam revealed an IPNB with high-grade dysplasia.

IPNB is a rare disease that can involve both the intrahepatic and extrahepatic biliary tract. It occurs most commonly in men, usually during the 6th and 7th decades of life. Because of the high risk of malignant transformation, surgical resection is the recommended treatment, with options including pancreaticoduodenectomy, hemihepatectomy and bile duct resection. However, the pathogenesis, clinical features, and outcomes remain poorly documented.