title: Living donor liver transplantation for hepatoblastoma with Beckwith-Wiedemann syndrome.
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.
authors.affiliation: Department of Transplantation Surgery, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Kazunari
authors.initials: K
authors.lastname: Sasaki
authors.forename: Mureo
authors.initials: M
authors.lastname: Kasahara
authors.forename: Akinari
authors.initials: A
authors.lastname: Fukuda
authors.forename: Takanobu
authors.initials: T
authors.lastname: Shigeta
authors.forename: Hideaki
authors.initials: H
authors.lastname: Tanaka
authors.forename: Satoshi
authors.initials: S
authors.lastname: Nakagawa
authors.forename: Kazumasa
authors.initials: K
authors.lastname: Mitsui
authors.forename: Ryoko
authors.initials: R
authors.lastname: Harada
authors.forename: Atsuko
authors.initials: A
authors.lastname: Nakagawa
mesh_terms: D001506:Beckwith-Wiedemann Syndrome / Q000628:therapy*; D005260:Female; D018197:Hepatoblastoma / Q000628:therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D008113:Liver Neoplasms / Q000628:therapy*; D016031:Liver Transplantation / Q000379:methods*; D019520:Living Donors; D008775:Methylprednisolone / Q000627:therapeutic use; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D012008:Recurrence; D016559:Tacrolimus / Q000627:therapeutic use; D013997:Time Factors; D014057:Tomography, X-Ray Computed / Q000379:methods; D000509:alpha-Fetoproteins / Q000378:metabolism
chemical_list: D007166:Immunosuppressive Agents; D000509:alpha-Fetoproteins; D009173:Mycophenolic Acid; D016559:Tacrolimus; D008775:Methylprednisolone