Perihilar cholangiocarcinoma

Perihilar cholangiocarcinoma, also known as Klatskin tumor, is a rare malignant neoplasm arising from the epithelial cells of the bile ducts at or near the confluence of the right and left hepatic ducts (the hepatic hilum). It is the most common subtype of cholangiocarcinoma, accounting for approximately 50-60% of all bile duct cancers. The tumor primarily affects the hepatobiliary system and can obstruct bile flow, leading to progressive jaundice, which is often the earliest and most prominent symptom. Patients typically present with painless jaundice, pruritus (itching), pale stools, dark urine, unintentional weight loss, and abdominal discomfort. As the disease progresses, fatigue, loss of appetite, and signs of cholangitis (bile duct infection) may develop. Risk factors include primary sclerosing cholangitis, hepatolithiasis, parasitic infections of the bile ducts (such as liver flukes), and chronic biliary inflammation. The disease predominantly affects adults, with a peak incidence in the sixth to seventh decades of life. Diagnosis involves a combination of imaging studies (MRI/MRCP, CT, and endoscopic ultrasound), tumor markers (CA 19-9 and CEA), and sometimes tissue biopsy. The only potentially curative treatment is surgical resection, which may include hepatectomy with bile duct excision and lymph node dissection. In selected cases, liver transplantation following neoadjuvant chemoradiation has shown promising results. For unresectable disease, palliative options include biliary stenting, chemotherapy (typically gemcitabine-based regimens combined with cisplatin), and radiation therapy. The prognosis remains poor overall, with five-year survival rates varying significantly depending on the stage at diagnosis and resectability of the tumor.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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