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) | Lack et al[31] (1983) | Pham et al[17] (2007) | Ismail et al[22] (2009) | Romano et al[18] (2011) | Beaunoyer et 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% |
Staging 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;
Grade 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.