Intrahepatic Cholangiocarcinoma: The Weight of History

Cholangiocarcinoma (CCA) is a malignant tumor arising from the epithelial cells of the bile ducts. Its incidence is increasing, and it is associated with a high mortality rate due to its aggressive behavior and frequent late diagnosis. We report the case of a 52-year-old man with a history of dyslipidemia and active smoking who was brought to the emergency department following a suicide attempt. The patient suspected he had a neoplastic disease, similar to the CCA that had affected his mother. He reported a three-month history of weight loss, asthenia, fever, and night sweats. Laboratory evaluation revealed inflammatory anemia. He also reported consumption of unpasteurized cheese at symptom onset, prompting hospital admission for etiological investigation. Computed tomography scan (CT scan) of the abdomen and pelvis demonstrated calcified hilar lymphadenopathy and other slightly globular, nonspecific lymph nodes. Extensive laboratory testing, such as bone marrow aspirate, bone biopsy, and gastrointestinal endoscopic studies, revealed no primary tumor. After discharge, an 18F-FDG PET-CT scan showed findings compatible with metastatic disease - hepatic uptake (single lesion), multifocal bone involvement, and intestinal uptake. Biopsy of a bone lesion confirmed metastasis from an undifferentiated carcinoma of unknown primary, and treatment was initiated. The clinical course was unfavourable. Clinical autopsy revealed a high-grade intrahepatic CCA with bone metastases. This case illustrates an atypical presentation of intrahepatic CCA manifesting as metastatic disease of an unknown primary tumor and underscores the diagnostic challenges associated with this malignancy.