Liver and intrahepatic bile ducts - tumor
Other malignancies
Hepatoblastoma

Author: Deepali Jain, M.D. (see Authors page)

Revised: 5 December 2017, last major update February 2012

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Hepatoblastoma[TI] liver[TI]

See also: Epithelial typeMixed epithelial mesenchymal type
Cite this page: Jain, D. Hepatoblastoma. PathologyOutlines.com website. http://pathologyoutlines.com/topic/livertumorhepatoblastoma.html. Accessed December 15th, 2017.
Definition / general
  • Malignant liver neoplasm almost exclusively in infants, composed exclusively of immature hepatocytic elements (IARC: 8970 / 3 Hepatoblastoma [Accessed 5 December 2017])
  • Most common primary liver tumor in children (50% of liver malignancies in children)
  • Existence in adults is controversial (J Hepatol 2012;56:1392)
  • Can be congenital; 90% occur by age of 5 years, 70% by age of 2 years; 2/3 male, prevalence of 1 per 120,000 (1 per million children under age 15 years)
  • Associated with familial colonic polyposis (APC gene, 500x risk), glycogen storage disease, hemihypertrophy (Beckwith-Wiedemann syndrome), Li-Fraumeni syndrome, low birth weight infants, Simpson-Golabi-Behmel syndrome, trisomy 18, Wilms tumor; not associated with cirrhosis
  • Also associated with adenomatoid transformation of Bowman capsular epithelium in kidney
  • Metastases to adrenal glands, bone marrow, brain, lung, lymph nodes (regional)
  • Variable virilization due to hCG production by multinucleated giant cells
  • Laboratory: thrombocytosis (Arch Dis Child 1993;69:171), elevated serum AFP (75%)
  • Note: diagnosis difficult on needle biopsy; must sample generously
Staging / staging classifications
Based on Children's Cancer Study Group:
  • Stage I: completely resected
  • Stage II: microscopic residual disease only
  • Stage III: gross residual disease or positive lymph nodes or spilled tumor
  • Stage IV: metastases

  • Note: can also stage using TNM
Prognostic factors
  • Stage, age and sex
  • Favorable: presence of osteoid
  • Unfavorable: small cell / undifferentiated (Pediatr Blood Cancer 2009;52:328) and macrotrabecular patterns, increased mitotic activity; also AFP level < 100, nuclear beta catenin staining, low p27 / kip1, high cyclin D1, 4q deletions
Treatment
  • Preoperative chemotherapy and surgery; resect lung metastases; liver transplant if unresectable
  • Long term survival now 60 - 70% with most recurrences detected within 3 years
Gross description
  • Usually right lobe
  • Usually solid, variegated, tan green, solitary (70%), variable hemorrhage and cysts
  • Mean 10 cm (range 3 - 20 cm), well circumscribed; often partially encapsulated
  • May be calcified or ossified in prominent mesenchymal component
Microscopic (histologic) description
  • Epithelial and mesenchymal elements in varying proportions and at variable stages of differentiation
  • Pseudocapsule, canaliculi with bile formation, cords 2 - 3 cells thick with alternating light and dark pattern due to glycogen and fat
  • Cells smaller than normal hepatocytes
  • Extramedullary hematopoiesis common in fetal and embryonal subtypes
  • Usually no pleomorphism, no intranuclear inclusions, no hyaline globules, rare / no tumor giant cells, no mitotic figures and no associated cirrhosis
  • Rare rhabdoid cells