Abstract:
BACKGROUND AND AIM:We reviewed the results and pattern of failure of the consensus HB/HCC 1996 treatment protocol for pediatric hepatoblastoma (HB) in Hong Kong. The role of SIOPEL and Children's Hepatic tumors International Collaboration (CHIC) risk stratification was evaluated. METHODS:Patients enrolled on the protocol from 1996 to 2014 were included. PRETEXT staging, SIOPEL, and CHIC risk groups were retrospectively assigned. RESULTS:Sixty patients were enrolled with median age at diagnosis of 1.1 years and median follow-up time of 6.8 years. Alpha-fetoprotein (AFP) was raised (>100 ng/mL) in 58 (97%) patients. Five (8%) had metastases at presentation and 7 (12%) experienced tumor rupture prior to or during treatment. Twenty-nine patients (48%) received a first-line cisplatin, 5-fluorouracil, and vincristine regimen only while 23 (38%) also had alternative chemotherapeutic agents. Hepatic resection could be performed in 48 (80%) patients. Three (5%) patients underwent upfront liver transplantation. Five-year event-free survival and overall survival rates were 69.2% ± 6.1% and 77.6% ± 5.5% respectively. Among the 16 patients with relapse/progression, 9 had intrahepatic failure only, 5 had distant failure only, and 2 had combined local and distant failure. Predictors of inferior outcome included advanced Evans staging, disease involving both lobes, rupture, low AFP, and suboptimal response to first-line chemotherapy. Assigned in 44 patients, PRETEXT staging, SIOPEL, and CHIC risk groups significantly predicted EFS and OS. CONCLUSIONS:Although the consensus HB/HCC 1996 protocol led to cure in three-quarters of pediatric HB patients, an upfront risk stratification system is required to identify and improve the outcome of high-risk patients.
journal_name
Pediatr Blood Cancerjournal_title
Pediatric blood & cancerauthors
Liu APY,Ip JJK,Leung AWK,Luk CW,Li CH,Ho KKH,Lo R,Chan EKW,Chan ACY,Chung PHY,Chiang AKSdoi
10.1002/pbc.27482subject
Has Abstractpub_date
2019-01-01 00:00:00pages
e27482issue
1eissn
1545-5009issn
1545-5017journal_volume
66pub_type
杂志文章abstract:BACKGROUND:Various projects dedicated specifically to adolescents and young adults (AYA) with cancer have been developed in recent years. A critical aspect of such programs is the ability to demonstrate its value, and therefore how to measure desired outcomes. METHODS:A list of metrics to consider for demonstrating th...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
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abstract::Hemophagocytic lymphohistioytosis (HLH) is a severe, life-threatening hyperinflammatory disorder that requires prompt diagnosis and treatment. Approximately, 25-50% of patients with HLH fail to achieve remission with established regimens that include dexamethasone and etoposide, or methylprednisolone and antithymocyte...
journal_title:Pediatric blood & cancer
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journal_title:Pediatric blood & cancer
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更新日期:2011-01-01 00:00:00
abstract::2-Chlorodeoxyadenosine (2-CdA) has been successfully used in children to treat refractory Langerhans cell histiocytosis and juvenile xanthogranuloma (JXG) as salvage therapy. Although 2-CdA is generally well-tolerated, with temporary myelosuppression as the primary dose-limiting toxicity, prolonged myelosuppressive, a...
journal_title:Pediatric blood & cancer
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更新日期:2012-02-01 00:00:00
abstract:BACKGROUND:The efficacy of combination chemotherapy with methotrexate (MTX) and asparaginase is not well known in relapsed and refractory acute leukemia after contemporary therapy. PROCEDURE:A retrospective study of pediatric patients with relapsed or refractory acute myeloid leukemia (AML) who received MTX and aspara...
journal_title:Pediatric blood & cancer
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journal_title:Pediatric blood & cancer
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journal_title:Pediatric blood & cancer
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journal_title:Pediatric blood & cancer
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更新日期:2015-12-01 00:00:00
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journal_title:Pediatric blood & cancer
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journal_title:Pediatric blood & cancer
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journal_title:Pediatric blood & cancer
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journal_title:Pediatric blood & cancer
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journal_title:Pediatric blood & cancer
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journal_title:Pediatric blood & cancer
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更新日期:2010-12-01 00:00:00
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journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.20623
更新日期:2007-07-01 00:00:00
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journal_title:Pediatric blood & cancer
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更新日期:2006-10-15 00:00:00
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journal_title:Pediatric blood & cancer
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journal_title:Pediatric blood & cancer
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journal_title:Pediatric blood & cancer
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journal_title:Pediatric blood & cancer
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更新日期:2008-05-01 00:00:00
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journal_title:Pediatric blood & cancer
pub_type: 杂志文章
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更新日期:2007-11-01 00:00:00
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journal_title:Pediatric blood & cancer
pub_type: 杂志文章,多中心研究
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journal_title:Pediatric blood & cancer
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更新日期:2011-08-01 00:00:00
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journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章,多中心研究
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更新日期:2020-08-01 00:00:00
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journal_title:Pediatric blood & cancer
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journal_title:Pediatric blood & cancer
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journal_title:Pediatric blood & cancer
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