Table 1. Summary of patients with pediatric cholangiocarcinoma presenting in the first decade of life described in the literature.

Ref.	Age (years)/ Sex	Comorbidities	Presenting symptoms	Tumor markers	Site	Treatment	Status/ Follow up
Scheimann et al.8	4/F	PFIC	Fever	CA 19-9 132 IU/mL	ICCA	Chemo	Death within 5 months from diagnosis
		Giant cell hepatitis
		Biliary cirrhosis		AFP-61.5 IU/mL
Scheimann et al.8	7/F	PFIC	Pruritis	Not available	ICCA	None	Death within 4 months from diagnosis
		Giant cell hepatitis
		Biliary cirrhosis
Saikusa et al.6	3/M	Congenital biliary dilatation with pancreaticobiliary maljunction	Abdominal pain	Not available	ECCA	Resection	Alive/ 1 year
Index case	5/M	No comorbidities	Abdominal distension	CA 19-9- 136 IU/mL	ICCA	Chemo	Death within 2 months from diagnosis
				AFP (–) 2.19IU/mL

AFP: Alpha-fetoprotein. CA: Carbohydrate antigen; Chemo: chemotherapy; ECCA: Extrahepatic cholangiocarcinoma; ICCA: Intrahepatic cholangiocarcinoma; PFIC: Progressive familial intrahepatic cholestasis.