Living donor liver transplantation for hepatoblastoma with Beckwith-Wiedemann syndrome.
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.
Sasaki
Kazunari
K
Department of Transplantation Surgery, National Center for Child Health and Development, Tokyo, Japan.
Kasahara
Mureo
M
Fukuda
Akinari
A
Shigeta
Takanobu
T
Tanaka
Hideaki
H
Nakagawa
Satoshi
S
Mitsui
Kazumasa
K
Harada
Ryoko
R
Nakagawa
Atsuko
A
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
D007166:Immunosuppressive Agents; D000509:alpha-Fetoproteins; D009173:Mycophenolic Acid; D016559:Tacrolimus; D008775:Methylprednisolone