Prolonged survival with first-line chemotherapy in advanced extrahepatic cholangiocarcinoma

Biliary tract cancer accounts for roughly 3% of adult malignancies of the gastrointestinal system. First-line treatment with gemcitabine-cisplatin chemotherapy is the standard for the management of metastatic biliary tract cancers. We present the case of a man who presented with abdominal pain, decreased appetite and weight loss for 6 months. Baseline evaluation revealed a liver hilar mass with ascites. Imaging, tumour markers, histopathology and immunohistochemistry revealed the diagnosis of metastatic extrahepatic cholangiocarcinoma. He was treated with gemcitabine-cisplatin chemotherapy followed by maintenance chemotherapy with gemcitabine and demonstrated an exceptionally good response and tolerance to chemotherapy with no long-term toxicity so far on maintenance therapy and progression-free survival exceeding 2.5 years after diagnosis. The rarity of this case is the exhibition of prolonged clinical response with maintenance chemotherapy for an aggressive cancer, thus needing further research into duration and outcomes of maintenance chemotherapy.