Primary sclerosing cholangitis
ICD-10 K83.0 · ICD-11 DB96.2

PSC with Confirmed Cholangiocarcinoma or High-Grade Biliary Dysplasia

This protocol applies to patients with primary sclerosing cholangitis (PSC) in whom cholangiocarcinoma (CCA) or high-grade biliary dysplasia has been confirmed. This diagnosis significantly changes the clinical trajectory and requires prompt specialist involvement.

When cholangiocarcinoma or high-grade dysplasia is confirmed in the setting of PSC, management moves beyond standard disease surveillance. The confirmed malignant or pre-malignant finding calls for referral to a specialised centre, where a multidisciplinary team can evaluate curative options.

Therapeutic considerations at that level may include surgical and transplant-based approaches, as well as combinations of modalities — the appropriate path depends on individual patient and tumour factors assessed by the specialist team.

The complete structured regimen, including all evaluated options and decision criteria, is available via the link below.

DOI: 10.1016/j.jhep.2022.05.011

Referral to a specialised centre is recommended when CCA or high-grade dysplasia is confirmed.

In a multidisciplinary approach, therapeutic options including liver transplantation, liver resection, irradiation, brachy- or systemic therapy or combinations should be considered.

Liver transplantation following neoadjuvant chemoradiation in carefully selected patients, with early cancers without tumour spread, was trialled in the US (Mayo protocol) and was associated with a 5-year overall survival rate of 65%.

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