Living donor liver transplantation for hepatoblastoma with Beckwith-Wiedemann syndrome

Pediatr Transplant. 2010 Nov;14(7):E89-92. doi: 10.1111/j.1399-3046.2009.01198.x. Epub 2010 Aug 5.

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.

Publication types

  • Case Reports

MeSH terms

  • Beckwith-Wiedemann Syndrome / therapy*
  • Female
  • Hepatoblastoma / therapy*
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Infant
  • Liver Neoplasms / therapy*
  • Liver Transplantation / methods*
  • Living Donors
  • Methylprednisolone / therapeutic use
  • Mycophenolic Acid / analogs & derivatives
  • Mycophenolic Acid / therapeutic use
  • Recurrence
  • Tacrolimus / therapeutic use
  • Time Factors
  • Tomography, X-Ray Computed / methods
  • alpha-Fetoproteins / metabolism

Substances

  • Immunosuppressive Agents
  • alpha-Fetoproteins
  • Mycophenolic Acid
  • Tacrolimus
  • Methylprednisolone