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Table 2 Hepatic carcinomas reported with similar morphology (HCC, CC, and NE)

From: Pediatric combined hepatocellular-cholangiocarcinoma (cHCC-CC) with neuroendocrine features: distinguishing genetic alterations detected by chromosomal microarray

Reference

Age/Sex

Morphology

Serum Markers

IHC (+) 

IHC (–)

Molecular

Diagnosis

Beard et al. [11]

19/M

Tubulocystic, trabecular, glandular

CA 19–9

CA19.9, CK19, CK7,

Hep-Par1 (few cells), Arginase-1, Syn

NA

Copy number imbalances (e.g., 5q and 7p). NE mutations not identified in the HCC/CC components indicating separate cell line lineages

Malignant epithelial neoplasm with multiple lineages including HCC, CC, and NE carcinoma

aNodular/solid

 

aCK19 (focal), Hep-Par1 (patchy), Syn, CD56

aCK7

Braxton et al. [12]

17/F

Microcystic, trabeculae, pseudoglandular, solid/hepatoid, blastemal

None

CK19, CK7, Syn & CgA (weak), InhibinA

Hep-Par1, AFP,

b-Catenin

Cytogenomic alterations consistent with primary liver carcinomas (not NE carcinomas)

Cholangioblastic Cholangiocarcinoma

44/F

Trabecular, solid/hepatoid

CgA (mild)

CK7 (weak), CK19, pCEA (non-canalicular), AFP (weak, patchy), Glypican-3, CDX2 (focal), Syn & CgA (weak), InhibinA

Ca19.9, Hep-Par1

24/F

Microcystic, trabeculae, pseudoglandular, solid/hepatoid, blastemal

None

CK7, CK19, SOX9, pCEA (non-canalicular), CgA (weak), InhibinA

b-Catenin, Glypican-3, Syn

Dimopoulos et al. [13]

65/F

Tubuloglandular, solid, nested

AFP

AFP-L3%

CgA

CK19 and focally ( +) for CK7, CK20, AFP, Hep-Par1, Arginase 1, pCEA, Syn, NSE, CDX2, p53

CA19.9, CgA

NGS: Mutations in

CDKN2A exon 2 and TP53 exon 7

HCC with biliary and NE differentiation

aSyn (strong, diffuse), CgA (focal, weak)

aAFP, Hep-Par1, Arginase 1, CD56

This case

16/M

Tubuloglandular, biliary-like, trabecular, solid

None

CA19.9 (focal), CK19 (biliary areas), pCEA (non-canalicular, focal), Syn & CgA (weak)

AFP, b-catenin, Hep-Par1, Glypican 3

CMA: HCC-like and CC-like areas share common LOH pattern with additional losses detected in the CC-like areas. Findings indicate same cell line lineage with subsequent genetic diversion giving rise to CC-like phenotype

cHCC-CC with NE-like features

  1. AFP Alpha-fetoprotein, CC Cholangiocarcinoma, CgA Chromogranin, CMA Chromosomal microarray, HCC Hepatocellular carcinoma, IHC Immunohistochemistry, LOH Loss of heterozygosity, NA Not applicable, NE Neuroendocrine, NGS Next generation sequencing, Syn Synaptophysin
  2. aFeatures describe a subset or a metastatic lesion of the primary tumor