# Perihilar Extrahepatic Cholangiocarcinoma With Typical and Atypical Manifestations: Diagnostic Resolution During Autopsy

## TL;DR

A rare case of bile duct cancer was only diagnosed after death, showing how autopsies can reveal complex diseases missed during life.

## Contribution

Highlights the diagnostic challenges and value of autopsy in atypical cholangiocarcinoma cases.

## Key findings

- The tumor was identified during autopsy after imaging failed to detect it.
- Atypical symptoms like kidney failure and bleeding were explained by multiorgan infiltration.
- Autopsy clarified the role of immunohistochemistry in diagnosis and treatment planning.

## Abstract

Extrahepatic cholangiocarcinoma is a rare malignant neoplasm characterized by aggressive behavior and poor prognosis. It typically presents in the seventh to eighth decades of life, with a slight male predominance, and most commonly manifests with symptoms related to biliary obstruction; however, atypical clinical presentations may occur. We report the case of a 71-year-old man who presented with acute pancreatitis. Imaging studies failed to identify an underlying neoplasm, and the diagnosis was established only during the autopsy, which revealed a perihilar extrahepatic cholangiocarcinoma involving the proximal extrahepatic bile ducts, with extensive multiorgan infiltration. These findings accounted for the patient’s atypical manifestations, including renal dysfunction and gastrointestinal bleeding, which were fully elucidated through postmortem examination. Extrahepatic cholangiocarcinoma represents a diagnostic challenge due to its nonspecific clinical features and its anatomically difficult location for biopsy, particularly when atypical manifestations predominate. This case highlights the value of autopsy in clarifying complex clinicopathologic correlations and provides observations regarding the role of immunohistochemistry in the diagnosis, prognostic assessment, and therapeutic management of these tumors.