TY - JOUR
T1 - Multifocal hepatoblastoma
T2 - What is the risk of recurrent disease in the remnant liver?
AU - Fahy, Aodhnait S.
AU - Shaikh, Furqan
AU - Gerstle, Justin T.
N1 - Publisher Copyright:
© 2019 Elsevier Inc.
PY - 2019/5
Y1 - 2019/5
N2 - Purpose: Multifocal hepatoblastoma (HB)is often treated with total hepatectomy and transplantation owing to concerns of surgical resectability, local recurrence, and/or metachronous tumor in the remnant liver. We aimed to review HB patients to determine the risk of local recurrence in multifocal disease. Methods: We undertook retrospective cohort analysis of all HB patients at a single tertiary referral center between 2001 and 2015. Demographics, diagnostic features, operative details, and outcomes were analyzed. Results: Sixty patients underwent surgical management of HB. 39 had unifocal, and 21 had multifocal disease. Of multifocal patients, 9 underwent liver transplantation, 10 anatomic resections, and 2 nonanatomic resections. Overall, two patients had recurrence in the remnant liver — both from the unifocal group. There were equivalent distant (lung)recurrences between the groups (8% for unifocal versus 14% for multifocal), p = 0.89. At a mean of 75 months of follow-up, overall survival was 97% for unifocal patients and 86% for multifocal patients, p = 0.12. Conclusion: Multifocal HB was not associated with increased local recurrence in the setting of R0 resection and chemotherapy. These data do not support the contention that all patients with multifocal HB require a total hepatectomy and transplantation to reduce the incidence of local recurrence and/or metachronous tumor development. Level of evidence: Level III — Limited cohort analysis.
AB - Purpose: Multifocal hepatoblastoma (HB)is often treated with total hepatectomy and transplantation owing to concerns of surgical resectability, local recurrence, and/or metachronous tumor in the remnant liver. We aimed to review HB patients to determine the risk of local recurrence in multifocal disease. Methods: We undertook retrospective cohort analysis of all HB patients at a single tertiary referral center between 2001 and 2015. Demographics, diagnostic features, operative details, and outcomes were analyzed. Results: Sixty patients underwent surgical management of HB. 39 had unifocal, and 21 had multifocal disease. Of multifocal patients, 9 underwent liver transplantation, 10 anatomic resections, and 2 nonanatomic resections. Overall, two patients had recurrence in the remnant liver — both from the unifocal group. There were equivalent distant (lung)recurrences between the groups (8% for unifocal versus 14% for multifocal), p = 0.89. At a mean of 75 months of follow-up, overall survival was 97% for unifocal patients and 86% for multifocal patients, p = 0.12. Conclusion: Multifocal HB was not associated with increased local recurrence in the setting of R0 resection and chemotherapy. These data do not support the contention that all patients with multifocal HB require a total hepatectomy and transplantation to reduce the incidence of local recurrence and/or metachronous tumor development. Level of evidence: Level III — Limited cohort analysis.
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U2 - 10.1016/j.jpedsurg.2019.01.036
DO - 10.1016/j.jpedsurg.2019.01.036
M3 - Article
C2 - 30819543
AN - SCOPUS:85061965961
SN - 0022-3468
VL - 54
SP - 1035
EP - 1040
JO - Journal of pediatric surgery
JF - Journal of pediatric surgery
IS - 5
ER -