Pediatric hepatocellular carcinoma

Table 2 Clinical characteristics of children with hepatocellular carcinoma in series from West

Study→Ref. (yr)	SEER[8,15,30] (2013, 2013, 2014)	POG[16](2002)	SIOPEL 1[27] (2002)	Lacket al[31](1983)	Phamet al[17](2007)	Ismailet al[22](2009)	Romanoet al[18](2011)	Beaunoyeret al[23](2007)
Region	United States	United States	Europe	United States	United States	Poland	Italy	United States
Number of subjects	238, 80, 218	46	39	32	22	21	10	10
Males (%)	58	57	72	63	50	-	40	60
Age (yr)	12.9 ± 5.2	-	Median 12 (4-15)	Mean 9.7 (0.5-21)	13.1 ± 1.1 (2-18 )	Median 11.1 (2-18)	Median 1.8 (0.5-7.2)	Median 10.1 (4.4-16.3)
Age-group (%)	0-4 (8-9) 5-9 (14-16) 10-14 (27-29) 15-19 (48-49)	< 1 y (6.5) 1-9 y (37) ≥ 10 y (56.5)	-	-	-	≤ 2 (5) 2-10 (38) ≥ 10 (57)	< 2 (50) 2-5 (30) ≥ 5 (20)	3-12 (70) ≥ 13 (30)
Size (cm)	0-5 (21%) 5.1-10 (24%) 10.1-15 (36%) > 15 (19%)	-	Median 14 (7-26) cm	-	11.8 ± 0.6 (5-25) cm	-	-	Median 5.8 (2-10.5) cm > 5 cm in 60%
Multifocality	Satellite lesions 28%	-	56% (4 diffuse)	-	-	-	50% (2-10 in No.)	70%
Etiology	-	-	13 HBV 1 Tyr 1 Biliary	-	-	4 Tyr 2 HBV 1 HCV 1 A1ATD 1 AIH 1 PFIC 11 no liver ds	3 BA 3 PFIC2 2 Tyr 1 CC 1 GSD4	4 Viral (3HBV) 1 Tyr 1 Alagille’s 1 PFIC 3 No Liver ds
Histology (n or %)	HCC NOS 58%-74% FLHCC 24%-41% Clear cell 1%-2%	HCC 78% FLHCC 22%	Epithelial 75% FLHCC 15% Poorly diff or clear cell 10%	HCC 84% FLHCC 16%	-	High grade2 (29%) Low grade (52%) FLHCC (19%)	G1 (1) G2 (6) G3 (3)2 No FLHCC	HCC in 9 FLHCC in 1
Cirrhosis	-	-	38%	16%	-	52%	100%	50%
AFP	-	≥ 20 ng/mL in 67%	Median 9677 (1-1400000) ng/mL > 10 ng/mL in 69%	-	-	1.7-713000 IU/mL	Median 2322 (3-35000) ng/mL	Median 446927 ng/mL
Extent or Stage (n or %)	Local 27%-28% Regional 35%-37% Distant 34%-35%	Stage I (8), II (0), III (25), IV (13)1	PRETEXT I (1), II (14), III (11), IV (13)	-	CCG/POG Stage I (60%), II (11.5%), III (17%), IV (11.5%)1	PRETEXT I (4), II (7), III (5), IV (5)	PRETEXT I (4), II (1), III (1), IV (4)	TNM Stage I (0), II (3), III (7), IV (0) T2 (3), T3 (6), T4 (1)
Vascular invasion	48%	-	21%	-	-	29%	20%	30%
Metastases	35%	-	Lung 31% Other 18%		-	0	-	-
Resectability	25%	22%	36%	22%	67%	48%	NA (All LT)	NA
Outside Milan	-	-	-	-	-	86%	60%	70%

¹Staging done as I, complete gross resection with pathologically negative margins; II, gross total resection with microscopic residual disease at margins; III, gross total resection with nodal involvement or tumor spill; and IV, metastatic disease with either complete or incomplete resection or biopsy;

²Grade as per Edmondson and Steiner. A1ATD: Alpha-1 antitrypsin deficiency; AFP: Alpha-fetoprotein; AIH: Autoimmune hepatitis; BA: Biliary atresia; CC: Choledochal cyst; CCG: Children’s cancer group; diff: Differentiated; FLHCC: Fibrolamellar variant of HCC; GSD: Glycogen storage disease; HBV: Hepatitis-B virus; HCV: Hepatitis-C virus; LT: Liver transplantation; NA: Not assessed; PFIC: Progressive familial intrahepatic cholestasis; PRETEXT: Pretreatment tumor extent evaluation; POG: Pediatric oncology group; SEER: Surveillance epidemiology and end-results; SIOPEL: Group for epithelial liver tumors of the international society of pediatric oncology; TNM: Tumor node metastasis stage; Tyr: Tyrosinemia.