Perihilar clear cell cholangiocarcinoma: an often-diagnostic challenge

Cholangiocarcinomas are rare and often diagnosed late. Clear cell histology is a rare variant of such cancers. We report one such case of a man in his late 60s, with a history of excess alcohol intake, who was found to have deranged liver biochemical tests incidentally during an admission for an allergic reaction. Subsequent imaging to investigate this suggested a diagnosis of perihilar cholangiocarcinoma (ultrasound, CT, MRI, cholangiogram). Biopsy confirmed this to be of clear cell type on histology and immunohistochemistry. Diagnosis and further management of this rare entity was conducted in multidisciplinary meetings with the regional hepatobiliary centre. The patient was deemed unsuitable for surgical resection, underwent chemotherapy but died 1 year later.