Risk of malignancy in Caroli disease and syndrome: A systematic review

Table 1 Literature search results and analyzed manuscripts

Ref.	Country	Year of publication	Type of study	n of reported patients	n of cancer (%)
Ammori et al[27]	United Kingdom	2002	Single-center, retrospective	8	3 (37.5)
De Kerckhove et al[19]	Belgium	2005	ELTR registry, retrospective	113	3 (2.7)
Kassahun et al[21]	Germany	2005	Single-center, retrospective	33	3 (9)
Habib et al[17]	United States	2006	Single-center, retrospective	33	1 (3)
Bockhorn et al[30]	Germany	2006	Single-center, retrospective	12	3 (25)
Millwala et al[5]	United States	2008	UNOS registry, retrospective	104	3 (2.9)
Ulrich et al[3]	Germany	2008	Single-center, retrospective	44	4 (9)
Hori et al[24]	Japan	2010	Single-center, retrospective	5	1 (20)
Lendoire et al[13]	Argentina	2011	Multicenter, retrospective	24	1 (4.2)
Mabrut et al[20]	France	2013	Multicenter, retrospective	155	8 (5)
Moslim et al[12]	United States	2015	Single-center, retrospective	9	3 (33.3)
Fahrner et al[25]	Germany	2018	Single-center, retrospective	21	4 (19)

ELTR: European Liver Transplant Registry; UNOS: United Network of Organ Sharing.

Table 2 Diagnostic work flow prior to surgery

Ref.	CT	MRI	ERCP	Ultrasound	Others
Ammori et al[27]	Yes	Yes	100%	Yes	No data
De Kerckhove et al[19]	Yes	Yes	Yes	Yes	Percutaneous cholangiography
Kassahun et al[21]	100%	16.2%	83.9%	100%	No data
Habib et al[17]	No data	No data	No data	No data	No data
Bockhorn et al[30]	100%	No data	92%	100%	No data
Millwala et al[5]	No data	No data	No data	No data	No data
Ulrich et al[3]	Yes	Yes	Yes	Yes	Percutaneous cholangiography
Hori et al[24]	No data	No data	No data	No data	No data
Lendoire et al[13]	Yes	Yes	Yes	Yes	Percutaneous cholangiography
Mabrut et al[20]	No data	74.7%	27.7%	No data	No data
Moslim et al[12]	No data	No data	No data	No data	No data
Fahrner et al[25]	No data	No data	No data	No data	No data

Percentages given indicate frequency of diagnostic tool within the study. CT: Computed tomography; MRI: Magnetic resonance imaging; ERCP: Endoscopic retrograde cholangiopancreatography.

Table 3 Patient demographics, duration of disease and tumor identification

Ref.	Age (yr)	Female sex (%)	Duration of disease	Tumor identification
Ammori et al[27]	35	75	No data	No data
De Kerckhove et al[19]	39.7	52	No data	No data
Kassahun et al[21]	51.5	55	< 1 yr 51%, 1-5 yr 13%, > 5 yr 35%	No data
Habib et al[17]	26	50	No data	No data
Bockhorn et al[30]	39	42	No data	67% incidental finding
Millwala et al[5]	35	55	No data	No data
Ulrich et al[3]	49	55	< 1 yr 55%, > 5 yr 2.5%	100% incidental finding
Hori et al[24]	23.74	No data	6.4 yr	100% puncture of nodule prior surgery
Lendoire et al[13]	48.7	70	2.6 yr	100% incidental finding
Mabrut et al[20]	55.7	43	7 mo	No data
Moslim et al[12]	40	22	No data	100% incidental finding
Fahrner et al[25]	56	74	No data	75% incidental finding 25% suspicious nodules in diagnostics

Table 4 Surgical treatment, tumor characteristics and survival

Ref.	Surgery for all patients (LR vs OLT)1	TNM	1-yr survival cancer patients	Recurrence
Ammori et al[27]	50% vs 50%	No data	33%	0%
De Kerckhove et al[19]	0% vs 100%	No data	66%	33%
Kassahun et al[21]	87% vs 0%	No data	0%	33%
Habib et al[17]	0% vs 100%	No data	0%	No data
Bockhorn et al[30]	83% vs 17%	n = 1 pT1, n = 1 pT2	66%	0%
Millwala et al[5]	0% vs 100%	No data	0%	No data
Ulrich et al[3]	90% vs 10%	No data	0%	No data
Hori et al[24]	0% vs 100%	No data	No data	No data
Lendoire et al[13]	91.6% vs 0%	No data	100%	No data
Mabrut et al[20]	75% vs 18.9%	n = 6 R0, n = 1 peritoneal seeding	33%	50%
Moslim et al[12]	22% vs 78%	No data	0%	33%, 66% tumor progression
Fahrner et al[25]	90% vs 10%	n = 1 pT1N1, n = 2 pT2, n = 1 pT3	100%	75%

¹Besides liver resection and orthotopic liver transplantation, other surgical interventions performed, so that the sum of the percentages does not equal 100%. LR: Liver resection; OLT: Orthotopic liver transplantation; TNM: Tumor-nodes-metastases.