Hepatoblastoma in a child with neurofibromatosis type I


Ucar C., Caliskan U., Toy H., Gunel E.

PEDIATRIC BLOOD & CANCER, vol.49, no.3, pp.357-359, 2007 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 49 Issue: 3
  • Publication Date: 2007
  • Doi Number: 10.1002/pbc.20663
  • Journal Name: PEDIATRIC BLOOD & CANCER
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.357-359
  • Ondokuz Mayıs University Affiliated: No

Abstract

A major hallmark of NF1 is the development of benign tumors, including peripheral neurofibromas, plexiform neurofibromas, gliomas of the optic tract, other low grade gliomas, and pheochromocytomas. Hepatoblastoma have not been previously reported in patients with neurofibromatosis type 1. We present a case of a 9-month-old boy diagnosed with both hepatoblastoma and neurofibromatosis type 1. Hepatoblastoma occurs in association with several well-described cancer predisposition syndromes, including familial adenomatous polyposis, Beckwith-Wiedemann syndrome, Li-Fraumeni syndrome, trisomy 18, and glycogen storage disease type 1. This paper describes a case of hepatoblastoma diagnosed in association with neurofibromatosis type 1.