Teratoid hepatoblastoma with abundant cholangioblastic component in a child with full trisomy 13.
Teratoid hepatoblastoma is a rare histological subtype of hepatoblastoma. A 15-month-old girl with full trisomy 13 presented with a liver mass (6 × 4.4 × 3.4 cm). Histological examination showed a teratoid hepatoblastoma with very different differentiation patterns intermixed with each other. Approximately 30% of the tumor demonstrated a primitive glandular epithelium component, which had a moderate to well-differentiated adenocarcinoma-like morphology, and features of mucinous epithelium with a biliary immunophenotype. We designated it as cholangioblastic component. The child received 4 cycles of monotherapy with doxorubicin after complete resection and showed no evidence of residual tumor 8 months after surgery. Our case is not only the 1st report of hepatoblastoma in trisomy 13 but also represents a unique example with a large glandular epithelium component with cholangioblastic features. Increased awareness of this entity and further molecular studies are needed for better understanding of the pathogenesis of teratoid hepatoblastoma.
1  Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles, Keck School of Medicine of the University of Southern California, Los Angeles, CA, USA.
Shengmei
S
Zhou
Sarangarajan
S
Ranganathan
Rajkumar
R
Venkatramani
Ellen
E
Gomulia
Larry
L
Wang
D000970:Antineoplastic Agents / Q000627:therapeutic use; D025063:Chromosome Disorders / Q000150:complications*; D002882:Chromosomes, Human, Pair 13; D004317:Doxorubicin / Q000627:therapeutic use; D005260:Female; D018197:Hepatoblastoma / Q000150:complications / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology*; D006801:Humans; D007223:Infant; D008113:Liver Neoplasms / Q000150:complications / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology*; D014314:Trisomy; D000073839:Trisomy 13 Syndrome
D000970:Antineoplastic Agents; D004317:Doxorubicin