National guidelines for the diagnosis and treatment of hilar cholangiocarcinoma

Abstract

A consensus meeting of national experts from all major national hepatobiliary centres in the country was held on May 26, 2023, at the Pakistan Kidney and Liver Institute & Research Centre (PKLI & RC) after initial consultations with the experts. The Pakistan Society for the Study of Liver Diseases (PSSLD) and PKLI & RC jointly organised this meeting. This effort was based on a comprehensive literature review to establish national practice guidelines for hilar cholangiocarcinoma (hCCA). The consensus was that hCCA is a complex disease and requires a multidisciplinary team approach to best manage these patients. This coordinated effort can minimise delays and give patients a chance for curative treatment and effective palliation. The diagnostic and staging workup includes high-quality computed tomography, magnetic resonance imaging, and magnetic resonance cholangiopancreatography. Brush cytology or biopsy utilizing endoscopic retrograde cholangiopancreatography is a mainstay for diagnosis. However, histopathologic confirmation is not always required before resection. Endoscopic ultrasound with fine needle aspiration of regional lymph nodes and positron emission tomography scan are valuable adjuncts for staging. The only curative treatment is the surgical resection of the biliary tree based on the Bismuth-Corlette classification. Selected patients with unresectable hCCA can be considered for liver transplantation. Adjuvant chemotherapy should be offered to patients with a high risk of recurrence. The use of preoperative biliary drainage and the need for portal vein embolisation should be based on local multidisciplinary discussions. Patients with acute cholangitis can be drained with endoscopic or percutaneous biliary drainage. Palliative chemotherapy with cisplatin and gemcitabine has shown improved survival in patients with irresectable and recurrent hCCA.

Keywords: Hilar cholangiocarcinoma; Bismuth-Corlette classification; Memorial Sloan Kettering Cancer Centre Staging; Preoperative biliary drainage; Portal vein embolisation; Surgical resection; Hepatectomy

Core Tip: Consensus among national hepatobiliary experts convened at the Pakistan Kidney and Liver Institute & Research Centre emphasized the complexity of hilar cholangiocarcinoma, advocating a multidisciplinary approach for optimal patient management. Diagnostic protocol includes imaging like computed tomography, magnetic resonance imaging, and magnetic resonance cholangiopancreatography, while endoscopic retrograde cholangiopancreatography plays an important role in tissue acquisition. Surgical resection remains the best curative treatment option. For unresectable cases, liver transplantation is considered under strict selection criteria. Preoperative biliary drainage and portal vein embolisation decisions may be needed for selective cases. Adjuvant chemotherapy addresses the risk of recurrence. The role of Immunotherapy is emerging and offers improved survival for irresectable hilar cholangiocarcinoma.