Review of incidence and outcomes of treatment of cholangiocarcinoma in patients with primary sclerosing cholangitis

Table 1 Studies on the incidence and prevalence of primary sclerosing cholangitis-associated cholangiocarcinoma

Ref.	Year	Country	No of PSC patients	Median/mean age (yr) of PSC patients	Percentage of male PSC patients	No of PSC patients with CCA1	Incidence of PSC-associated CCA (%)	Prevalence of PSC-associated CCA (%)	Median/mean age (yr) of the CCA cohort	Percentage of males in the CCA cohort	Study design
Stieber et al[171]	1989	United States	111	NS	NS	10	N/A	9	45	70	Retrospective (Tx cohort)
Broome et al[39]	1996	Sweden	305	39	63.9	24	1.5	7.8	NS	NS	Retrospective
Goss et al[173]	1997	United States	127	47	68.5	14	3.4	11	NS	NS	Retrospective (Tx cohort)
Ahrendt et al[8]	1999	United States	139	47	64.2	25	N/A	18	46	48	Retrospective
Björnsson et al[36]	2002	Sweden	260	41	72.3	28	1.2	10.8	NS	NS	Retrospective
Ponsioen et al[38]	2002	Netherlands	174	40	60.3	18	1.6	10.3	NS	NS	Retrospective
Brandsaeter et al[77]	2004	Scandinavian	255a	45	68.6	37	N/A	14.5	NS	Male preponderance	Multicentre retrospective
Burak et al[42]	2004	United States	161	41	67.7	11	0.6	6.8	NS	NS	Retrospective
Tischendorf et al[19]	2007	Germany	273	32	71.4	36	2.1	13.2	NS	NS	Retrospective
Bjornsson et al[35]	2008	International	176	38	55.1	20	1.1	11.4	NS	NS	Retrospective
Tanaka et al[92]	2008	Japan	391	50	58b	14	0.7	3.6	NS	57.1	Retrospective
Charatcharoenwitthaya et al[136]	2008	United States	230	42	59.6	23	1.2	10	51	73.9	Retrospective
Morris et al[87]	2008	United Kingdom	370	49	71.6	48	2.7	13	50	75	Retrospective
Claessen et al[74]	2009	Netherlands	211	35	67.8	15	0.8	7.1	NS	NS	Retrospective
Ataseven et al[51]	2009	Turkey	35	42	60	2	1.2	5.7	72	0	Retrospective
Chapman et al[5]	2012	United Kingdom	128	49	63.2	21	1.7	16.4	NS	67	Retrospective
de Valle et al[88]	2012	Sweden	199	39	71.3	17	1.3	8.5	NS	NS	Retrospective
Fevery et al[43]	2012	Belgium	200	39	59	13	0.5	6.5	41	46.1	Retrospective
Ngu et al[46]	2012	New Zealand	79	49	62	7	1.1	8.9	NS	NS	Retrospective + Prospective
Boonstra et al[33]	2013	Netherlands	590	39	63.5	41	0.9	6.9	47	NS	Retrospective
Geramizadeh et al[78]	2015	Iran	181	37	67.2	16	N/A	8.8	49	75	Retrospective (Tx cohort)
Liang et al[58]	2017	United Kingdom	244	57	63.5	10	0.8	4.1	NS	NS	Retrospective
Liu et al[91]	2017	Australia	208	41	60.5	16	0.7	7.7	NS	NS	Retrospective
Weismuller et al[32]	2017	International	7121	39	65.4	594	1.1	8.3	NS	NS	Retrospective
Saffioti et al[47]	2018	United Kingdom	281	48	64	13	N/A	4.6	NS	NS	Retrospective
Ali et al[158]	2018	United States	830	NS	NS	56	N/A	6.7	55	66	Retrospective
Freeman et al[21]	2019	Australia	39	45	69.2	3	1.0	7.7	NS	100	Retrospective

¹Incidences have been estimated by number of cholangiocarcinoma (CCA)/total person years, or number of CCA/[number of primary sclerosing cholangitis (PSC) × mean or median follow-up time (yr)].

^alisted for transplantation.

^bavailable data for 345 patients. Appropriate calculations were made where required. No studies with exclusively small duct PSC or studies in paediatric cohorts were included. PSA: Primary sclerosing cholangitis; CCA: Cholangiocarcinoma; N/A: Not applicable (where estimation is not feasible); NS: No significance; Tx: Transplant.