Figures (0)  Tables (3)
    • Author Age, years/sex Clinical presentation AFP, ng/mL Lobe/size, cm Pre-chemo pathology diagnoses Neoadjuvant chemotherapy Outcome
      Prokurat et al. [3] 9/M nr 21 K Right/nr HBL – Died after 22 months
      17/F nr 200 K Right/15 HBL – Died 22 months
      14/F nr Very high Right/15 HBL – Alive after 50 months
      8/F nr 53 K Left/15 nr – Died about 1 year after 27 months
      5/M nr 100 K Right/10 nr – Alive after 60 months
      11/M nr >300 Right/5 HBL – Died on day 13 post-surgery
      6/F nr 200 K na – Poor follow-up
      Ozcan et al. [7] 8/M Abdominal pain 37.2 K Right/10 HEM, NOS – Died after 2 months
      Ertel et al. [8] 23/F Abdominal pain 47.7 K Right/17 HEM, NOS – Alive
      Katzilakis et al. [9] 8/M nr nr nr nr – Died after 2 months
      Seng et al. [5] 8/M Abdominal distention 550 K Multiple HEM, NOS + Alive after 3 years
      Zhou et al. [2] 4.5/M Acute abdomen 25 K Right/11.3 HBL + focal HCC-like + Alive after 11 years
      12.5/M Abdominal pain 980 K Right/10 HEM, NOS + Alive after 8.8 years
      4/M Abdominal pain 301 K Right/5 HEM, NOS + Alive after 7.5 years
      9/M Abdominal pain 439 K Right/4.5 HEM, NOS + Alive after 9 years
      5/M Abdominal pain 80 K Right/9 HBL + focal HCC-like + Alive after 7.9 years
      7/M Abdominal pain 70 K Right/4.3 HEM, NOS + Alive after 6.1 years
      4/M Abdominal pain 182 K Right/12.1 HBL + Alive after 4.6 years
      15/F Abdominal pain 617 Left/10 HBL + focal HCC-like + Alive after 4.5 years
      14/M Abdominal pain 198 K Left/4.5 HEM, NOS + Alive after 5.1 years
      7/M Abdominal pain 1,280 K Left/6 HBL + focal HCC-like + Alive after 2.4 years
      11/M Acute abdomen 26.9 K Multifocal/11.3 HEM, NOS – Alive after 1.2 years
      nr, not reported; HBL, hepatoblastoma; HEM, NOS, hepatocellular malignant tumor, not otherwise specified; HCC, hepatocellular carcinoma; OLT, orthotopic liver transplant.

      Table 1. 

      Clinicopathologic characteristics of 22 reported cases of hepatocellular malignant tumor, NOS, in the literature

    • Marker Reported cases Positive Percentage
      β-Catenin 20 20 100
      Glypican-3 13 13 100
      SALL4 11 11 100
      Nuclear staining of TERT 11 9 81.8
      HepPar1 2 2 100
      AFP 8 7 87.5
      Cam 5.2 1 1 100
      EMA 7 1 14.3
      CK19 7 1 14.3
      P53 7 1 14.3
      HEM, NOS, hepatocellular malignant tumor, not otherwise specified; TERT, telomerase reverse transcriptase; AFP, alphafetoprotein.

      Table 2. 

      Immunohistochemical findings of the reported cases of HEM, NOS

    • Characteristic Most common findings
      Sex (F/M) 6/16
      Age (range), years 4–23
      Most common clinical presentation RUQ pain
      Most common imaging characteristic A large solid tumor of the liver with peripheral enhancement and central necrosis
      AFP (range), ng/mL 21,000–1,280,000
      Pre-chemo pathology diagnosis HEM, NOS: 13
      HBL: 5
      nr: 4
      Post-chemo pathology diagnosis HEM, NOS: 16
      HCC-like: 2
      HBL: 1
      nr: 3
      Histological findings Heterogeneous and complex with areas similar to HBL and areas similar to HCC
      Positive immunohistochemical markers β-Catenin and glypican-3
      Outcome (free of disease/died) 14 (63.7%)
      HEM, NOS, hepatocellular malignant tumor, not otherwise specified; AFP, alpha-fetoprotein; HBL, hepatoblastoma; nr, not reported; HCC, hepatocellular carcinoma.

      Table 3. 

      Summary of the presentation of 22 reported cases of HEM, NOS