• Beckwith-Wiedemann;
  • transplantation;
  • hepatoblastoma

Sasaki K, Kasahara M, Fukuda A, Shigeta T, Tanaka H, Nakagawa S, Mitsui K, Harada R, Nakagawa A. Living donor liver transplantation for hepatoblastoma with Beckwith–Wiedemann syndrome. Pediatr Transplantation 2010: 14:E89–E92. © 2009 John Wiley & Sons A/S.

Abstract:  BWS is one of the most well-known somatic overgrowth syndromes, which is characterized by macroglossia, organomegaly, abdominal wall defects, and predisposition to embryonal tumors, such as Wilms’ tumor, hepatoblastoma, and adrenocortical carcinoma. We report a case of BWS in a girl with unresectable hepatoblastoma, who received a planned LVDT following neo-adjuvant chemotherapy. This is the first case report of liver transplantation for patients with BWS. Tumor surveillance after transplantation would be necessary to detect possible recurrence of the original disease and development of other malignancies.