Treatment outcome and pattern of failure in hepatoblastoma treated with a consensus protocol in Hong Kong.


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.


Pediatr Blood Cancer


Pediatric blood & cancer


Liu APY,Ip JJK,Leung AWK,Luk CW,Li CH,Ho KKH,Lo R,Chan EKW,Chan ACY,Chung PHY,Chiang AKS




Has Abstract


2019-01-01 00:00:00












  • Retinoic acid therapy in "degenerative-like" neuro-langerhans cell histiocytosis: a prospective pilot study.

    abstract:BACKGROUND:Degenerative-like neuro-Langerhans cell histiocytosis (DN-LCH) is a rare complication of LCH marked by progressive cerebellar ataxia. No treatment has so far been shown to slow this progression. PROCEDURE:All-trans retinoic acid (ATRA) was administered orally at a dose of 45 mg/m(2) daily for 6 weeks and th...

    journal_title:Pediatric blood & cancer

    pub_type: 临床试验,杂志文章,多中心研究


    authors: Idbaih A,Donadieu J,Barthez MA,Geissmann F,Bertrand Y,Hermine O,Brugières L,Genereau T,Thomas C,Hoang-Xuan K

    更新日期:2004-07-01 00:00:00

  • Sirolimus therapy in the treatment of pseudomyogenic hemangioendothelioma.

    abstract::Pseudomyogenic hemangioendothelioma (PMH) is a rare, mostly indolent vascular tumor. Extensive cases are treated with amputation as chemotherapy seems to be ineffective. Recently, promising results were published using mammalian target of rapamycin (mTOR) inhibitors in tumors of vascular origin. Here, we present a cas...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章


    authors: Gabor KM,Sapi Z,Tiszlavicz LG,Fige A,Bereczki C,Bartyik K

    更新日期:2018-02-01 00:00:00

  • Adolescent and young adult oncology: transition of care.

    abstract::The fundamental purposes underlying formal health care transition from the pediatric to adult setting for young adult survivors of childhood cancer are to facilitate the continuous, medically and developmentally appropriate implementation of risk-based guidelines for the monitoring and management of late effects of ch...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章,评审


    authors: Freyer DR,Brugieres L

    更新日期:2008-05-01 00:00:00

  • Characterization of karyotypic events and evolution in neuroblastoma.

    abstract:BACKGROUND:Neuroblastoma (NB) is cytogenetically characterized by a number of non-random events. However, knowledge is limited concerning the timing of occurrence and inter-action of many of these events. METHODS:Karyotypic patterns were obtained from a study group of 49 NB tumors that had been analyzed by conventiona...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章


    authors: Betts DR,Cohen N,Leibundgut KE,Kühne T,Caflisch U,Greiner J,Traktenbrot L,Niggli FK

    更新日期:2005-02-01 00:00:00

  • Phase I trial and pharmacokinetic study of sorafenib in children with neurofibromatosis type I and plexiform neurofibromas.

    abstract:BACKGROUND:Sorafenib targets multiple pathways thought to be crucial in growth of plexiform neurofibroma (PN) in children with neurofibromatosis type 1 (NF1). Sorafenib has been tolerated with manageable toxicities in adults and children with refractory cancer. We conducted a separate study in this population. Monitori...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章


    authors: Kim A,Dombi E,Tepas K,Fox E,Martin S,Wolters P,Balis FM,Jayaprakash N,Turkbey B,Muradyan N,Choyke PL,Reddy A,Korf B,Widemann BC

    更新日期:2013-03-01 00:00:00

  • Optic pathway gliomas.

    abstract::Optic pathway gliomas represent approximately 5% of all pediatric intracranial tumors. While these tumors are most frequently low grade astrocytomas, they follow a highly variable clinical course, and accordingly, there is much debate regarding their optimal management. Their propensity to occur in very young children...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章,评审


    authors: Jahraus CD,Tarbell NJ

    更新日期:2006-05-01 00:00:00

  • A family with Hoyeraal-Hreidarsson syndrome and four variants in two genes of the telomerase core complex.

    abstract::We describe an African American family with Hoyeraal-Hreidarrson syndrome (HHS) in which 2 TERT mutations (causing P530L and A880T amino acid changes) and two in the DKC1 variants (G486R and A487A) were segregating. Both genes are associated with dyskeratosis congenita and HHS. It was important to determine the import...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章


    authors: Vogiatzi P,Perdigones N,Mason PJ,Wilson DB,Bessler M

    更新日期:2013-06-01 00:00:00

  • Effect of transfusion therapy on transcranial Doppler ultrasonography velocities in children with sickle cell disease.

    abstract:BACKGROUND:Children with sickle cell disease (SCD) and abnormal transcranial Doppler (TCD) ultrasonography have a high risk of stroke, but this risk is greatly reduced when chronic transfusion therapy is administered. The change in TCD velocities during chronic transfusion therapy and rate and frequency of normalizatio...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章


    authors: Kwiatkowski JL,Yim E,Miller S,Adams RJ,STOP 2 Study Investigators.

    更新日期:2011-05-01 00:00:00

  • Thiotepa/topotecan/carboplatin with autologous stem cell rescue in recurrent/refractory/poor prognosis pediatric malignancies of the central nervous system.

    abstract:BACKGROUND:Thiotepa and carboplatin are known to be active in central nervous system tumors. Topotecan potentiates the anti-cancer effects of alkylators and crosses the blood-brain barrier. We present ten patients with recurrent or progressive central nervous system malignancies treated on a myeloablative regimen using...

    journal_title:Pediatric blood & cancer

    pub_type: 临床试验,杂志文章


    authors: Gilheeney SW,Khakoo Y,Souweidane M,Wolden S,Boulad F,Dunkel IJ

    更新日期:2010-04-01 00:00:00

  • Leydig cell tumor after treatment for Ewing's sarcoma.

    abstract::Leydig cell tumors account for 3% of testicular tumors and have never been reported after treatment for Ewing's sarcoma. We report the unusual occurrence of a patient who developed a Leydig cell tumor of the testis 18 years after successful treatment for Ewing's sarcoma. Additional monitoring for second malignancies m...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章


    authors: Butros LJ,Phillip S,Chou A,Meyers PA,Huvos AG,Healey JH,Russo P,Gorlick RG

    更新日期:2007-12-01 00:00:00

  • Phase I study of oral cyclophosphamide and oral topotecan for children with recurrent or refractory solid tumors.

    abstract:BACKGROUND:To determine the maximum-tolerated duration and dose-limiting toxicity of a daily schedule of orally administered cyclophosphamide and topotecan in pediatric patients with recurrent or refractory malignant solid tumors. METHODS:Patients received oral cyclophosphamide (50 mg/m2/dose) in the morning followed ...

    journal_title:Pediatric blood & cancer

    pub_type: 临床试验,杂志文章


    authors: Bowers DC,Aquino VM,Leavey PJ,Bash RO,Journeycake JM,Tomlinson G,Mulne AF,Haynes HJ,Winick NJ

    更新日期:2004-01-01 00:00:00

  • Subcutaneous immunoglobulin replacement following CD19-specific chimeric antigen receptor T-cell therapy for B-cell acute lymphoblastic leukemia in pediatric patients.

    abstract::Twenty-eight patients were maintained on subcutaneous immunoglobulin replacement for persistent B-cell aplasia and agammaglobulinemia following CD19-targeted chimeric antigen receptor T-cell therapy for B-cell lymphoblastic leukemia. Patients were transitioned from intravenous to subcutaneous immunoglobulin replacemen...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章


    authors: Arnold DE,Maude SL,Callahan CA,DiNofia AM,Grupp SA,Heimall JR

    更新日期:2020-03-01 00:00:00

  • Identifying patient-centered outcomes for children with cancer and their caregivers when they seek care in the emergency department.

    abstract:BACKGROUND:Children with cancer have high utilization of the emergency department (ED), but little is known about which outcomes are most important to them and their caregivers when they seek care in the ED. PROCEDURE:A qualitative evaluation of ED experience for children with cancer and their caregivers was performed...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章


    authors: Mueller EL,Cochrane AR,Lynch DO,Cockrum BP,Wiehe SE

    更新日期:2019-10-01 00:00:00

  • Risk for CMV reactivation in children undergoing allogeneic hematopoietic stem cell transplantation.

    abstract::Tailoring pre-emptive CMV therapy to hematopoietic cell transplant recipients' risk for reactivation could make this approach more cost-effective. To determine the feasibility of creating a risk classification system for this purpose, we analyzed 169 pediatric HCTs involving seropositive recipients or donors. Using ri...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章


    authors: Qayed M,Khurana M,Hilinski J,Gillespie S,McCracken C,Applegate K,Chiang KY,Horan J

    更新日期:2015-02-01 00:00:00

  • Increased prevalence of risk factors for cardiovascular disease in long-term survivors of acute lymphoblastic leukemia and Wilms tumor treated with radiotherapy.

    abstract:BACKGROUND:Only a few studies have assessed cardiovascular risk factors (CRFs) in childhood cancer survivors. We determined the prevalence of CRFs in long-term survivors of acute lymphoblastic leukemia (ALL) and Wilms tumor. PROCEDURE:Adult survivors of ALL and Wilms tumor treated with radiotherapy and chemotherapy (R...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章


    authors: Geenen MM,Bakker PJ,Kremer LC,Kastelein JJ,van Leeuwen FE

    更新日期:2010-10-01 00:00:00

  • Resolution of chronic hypoxemia in pediatric sickle cell patients after treatment with hydroxyurea.

    abstract::Chronic hypoxemia is a common manifestation among patients with sickle cell anemia (SCA) who develop chronic lung disease. We report the beneficial effect of hydroxyurea on chronic hypoxemia in three pediatric patients with SCA and recurrent episodes of acute chest syndrome (ACS). All three patients improved rapidly a...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章


    authors: Singh SA,Koumbourlis AC,Aygun B

    更新日期:2008-06-01 00:00:00

  • Pharmacologic inhibition of epigenetic modification reveals targets of aberrant promoter methylation in Ewing sarcoma.

    abstract:BACKGROUND:Ewing sarcoma (ES), a highly aggressive tumor of children and young adults, is characterized most commonly by an 11;22 chromosomal translocation that fuses EWSR1 located at 22q12 with FLI1, coding for a member of the ETS family of transcription factors. Although genetic changes in ES have been extensively re...

    journal_title:Pediatric blood & cancer

    pub_type: 临床试验,杂志文章,多中心研究


    authors: Nestheide S,Bridge JA,Barnes M,Frayer R,Sumegi J

    更新日期:2013-09-01 00:00:00

  • Guidance regarding COVID-19 for survivors of childhood, adolescent, and young adult cancer: A statement from the International Late Effects of Childhood Cancer Guideline Harmonization Group.

    abstract::Childhood, adolescent, and young adult (CAYA) cancer survivors may be at risk for a severe course of COVID-19. Little is known about the clinical course of COVID-19 in CAYA cancer survivors, or if additional preventive measures are warranted. We established a working group within the International Late Effects of Chil...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章,多中心研究


    authors: Verbruggen LC,Wang Y,Armenian SH,Ehrhardt MJ,van der Pal HJH,van Dalen EC,van As JW,Bardi E,Baust K,Berger C,Castagnola E,Devine KA,Gebauer J,Marchak JG,Glaser AW,Groll AH,Haeusler GM,den Hartogh J,Haupt R,Hjorth L

    更新日期:2020-12-01 00:00:00

  • Successful treatment of refractory donor lymphocyte infusion-induced immune-mediated pancytopenia with rituximab.

    abstract::A 6-year-old male with chronic granulomatous disease, who was transplanted with bone marrow and exhibited increasing mixed chimerism, subsequently received two donor lymphocyte infusions (DLI). Two weeks after the second DLI, the patient developed acute graft-versus-host disease (GVHD) and progressive pancytopenia tha...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章


    authors: Kato I,Umeda K,Awaya T,Yui Y,Niwa A,Fujino H,Matsubara H,Watanabe K,Heike T,Adachi N,Endo F,Mizukami T,Nunoi H,Nakahata T,Adachi S

    更新日期:2010-02-01 00:00:00

  • Pulmonary function abnormalities in childhood cancer survivors treated with bleomycin.

    abstract:BACKGROUND:Bleomycin is associated with pulmonary toxic side effects including pneumonitis and pulmonary fibrosis. We evaluated the prevalence of long-term pulmonary function abnormalities in children receiving bleomycin therapy in the context of current chemotherapeutic regimens. METHODS:A retrospective review of pat...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章


    authors: De A,Guryev I,LaRiviere A,Kato R,Wee CP,Mascarenhas L,Keens TG,Venkatramani R

    更新日期:2014-09-01 00:00:00

  • Children with low-risk acute lymphoblastic leukemia are at highest risk of second cancers.

    abstract:BACKGROUND:The improved survival rates for childhood acute lymphoblastic leukemia (ALL) may be jeopardized by the development of a second cancer, which has been associated with thiopurine therapy. PROCEDURE:We retrospectively analyzed three sequential Nordic Society of Paediatric Haematology and Oncology's protocols c...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章


    authors: Nielsen SN,Eriksson F,Rosthoej S,Andersen MK,Forestier E,Hasle H,Hjalgrim LL,Aasberg A,Abrahamsson J,Heyman M,Jónsson ÓG,Pruunsild K,Vaitkeviciené GE,Vettenranta K,Schmiegelow K

    更新日期:2017-10-01 00:00:00

  • Prevalence of abnormal bone density of pediatric patients prior to blood or marrow transplant.

    abstract::Osteoporosis and osteopenia are long-term side effects of bone marrow transplant (BMT). The purpose of this study was to determine the prevalence of bone mineral density (BMD) abnormalities in pediatric patients prior to BMT. Forty-four pediatric patients were evaluated with DEXA scans. The average Z-score was -0.37. ...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章


    authors: Klopfenstein KJ,Clayton J,Rosselet R,Kerlin B,Termuhlen A,Gross T

    更新日期:2009-10-01 00:00:00

  • Loss of antibody titers and effectiveness of revaccination in post-chemotherapy pediatric sarcoma patients.

    abstract:BACKGROUND:Little is known about the effects of chemotherapy on patient antibody titers to vaccine-preventable infectious diseases; thus, there is no standard protocol for revaccinating post-chemotherapy patients. PROCEDURES:To assess losses of detectable antibody titers due to chemotherapy, we retrospectively examine...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章


    authors: Yu J,Chou AJ,Lennox A,Kleiman P,Wexler LH,Meyers PA,Gorlick R

    更新日期:2007-10-15 00:00:00

  • A meta-analysis of the neuropsychological effects of chemotherapy in the treatment of childhood cancer.

    abstract:BACKGROUND:Long-term neuropsychological deficits associated with pediatric cancers and the related treatments have been consistently reported. Whole brain cranial radiation therapy (CRT) is associated with neurocognitive impairment. As a result, physicians are reticent to use CRT in favor of systemic or intrathecal che...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章,meta分析


    authors: Pierson C,Waite E,Pyykkonen B

    更新日期:2016-11-01 00:00:00

  • Validation of an algorithmic nutritional approach in children undergoing chemotherapy for cancer.

    abstract:BACKGROUND:Undernutrition impacts clinical outcome adversely in children with cancer. This study aimed to validate a nutritional algorithm with specific application to the low- and middle-income country (LMIC) setting. PROCEDURE:Fifty children with a new diagnosis of cancer were enrolled in this randomized interventio...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章,随机对照试验


    authors: Totadri S,Trehan A,Mahajan D,Viani K,Barr R,Ladas EJ

    更新日期:2019-12-01 00:00:00

  • Measurement of energy expenditure.

    abstract::Although investigators have been engaged in the study of human energy metabolism for over 100 years, there remain unanswered questions regarding the role that energy expenditure plays in human health and disease. A critical examination of available methods for measurement of the various components of daily energy expe...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章


    authors: DeLany JP

    更新日期:2012-01-01 00:00:00

  • Nausea and vomiting in children and adolescents receiving intrathecal methotrexate: A prospective, observational study.

    abstract:BACKGROUND:The prevalence of nausea and vomiting after receipt of intrathecal methotrexate (IT-MTX) in pediatric oncology patients is unknown. METHODS:Patients (4-18 years) about to receive IT-MTX were eligible to participate in this prospective, observational study. Patients received antiemetics as prescribed by thei...

    journal_title:Pediatric blood & cancer

    pub_type: 评论,杂志文章


    authors: Flank J,Nadeem K,Moledina S,Khanna M,Schindera C,Punnett A,Dupuis LL

    更新日期:2017-10-01 00:00:00

  • Addition of oral iron to plasma transfusion in human congenital hypotransferrinemia: A 10-year observational follow-up with the effects on hematological parameters and growth.

    abstract::Congenital hypotransferrinemia (OMIM 209300) is an extremely rare disorder of inherited iron metabolism. Since its description in 1961, only 16 cases have been reported. The defective gene and molecular defect causing this disorder and clinicolaboratory findings seen in the homozygous and heterozygous states have been...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章


    authors: Aslan D

    更新日期:2018-02-01 00:00:00

  • Time-to-antibiotic administration as a quality of care measure in children with febrile neutropenia: a survey of pediatric oncology centers.

    abstract::Time-to-antibiotic administration (TTA) has been suggested as a quality-of-care (QOC) measure for pediatric oncology patients with febrile neutropenia (FN). Unknown, however, is to what extent pediatric oncology centers utilize TTA. Therefore, we designed and administered an electronic survey (68% response rate) of pr...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章


    authors: McCavit TL,Winick N

    更新日期:2012-02-01 00:00:00

  • Prevalence of daily medication adherence among children with sickle cell disease: a 1-year retrospective cohort analysis.

    abstract::The objective of this study is to determine the prevalence of adherence to daily medications among children with sickle cell disease (SCD). Prescription records for 12 months were obtained from participants who had insurance in a Medicaid-based single health maintenance organization. Adherence was measured as a ratio ...

    journal_title:Pediatric blood & cancer

    pub_type: 杂志文章


    authors: Patel NG,Lindsey T,Strunk RC,DeBaun MR

    更新日期:2010-09-01 00:00:00