Cholangiocarcinoma: Current opinion on clinical practice diagnostic and therapeutic algorithms. A review of the literature and a long-standing experience of a referral center

In the oncology landscape, cholangiocarcinoma is a challenging disease in terms of both diagnosis and treatment. Besides anamnesis and clinical examination, a definitive diagnosis of cholangiocarcinoma should be supported by imaging techniques (US, CT, MRI) and invasive investigations (ERC or EUS with brushing and FNA or US or CT-guided biopsy) followed by pathological confirmation. Surgery is the main curative option, so resectability of the tumour should be promptly assessed. Moreover, jaundice must be evaluated at the outset because biliary tract decompression with drainage and stent placement may be required. If the patient is resectable, pre-operative assessment of postoperative liver function is mandatory. After a curative resection, an adjuvant therapy may be administered. Otherwise, in cases with macroscopic residual disease after surgery or locally recurrent or unresectable cholangiocarcinoma at the diagnosis, first-line chemotherapy is the preferred strategy, possibly associated with radiotherapy and/or locoregional treatments. As the diagnostic and therapeutic pathway for cholangiocarcinoma can be declined in different modalities, patients should be promptly referred to a multidisciplinary team in a tertiary centre, familiar with this rare but lethal disease. Hence, the aim of the present paper is to focus on diagnostic and therapeutic algorithms based on the common guidelines and also on the clinical practice of multispecialist expert groups.