Abstract:
BACKGROUND:Hepatoblastoma is a rare malignancy of childhood. The scarcity of adequate cell models has limited our understanding of this tumor. Here we describe and characterize a new human liver tumor cell line, Hep293TT, derived from an aggressive childhood hepatoblastoma. PROCEDURES:Hep293TT cells were established using primary tumor tissues from a 5-year-old Caucasian female child. This cell line has been maintained for more than 34 months and over 20 subcultures, and was characterized by histopathology, ELISA, genotype, cytogenetics, CGH array, immunohistochemistry, and molecular sequence analyses. RESULTS:Cells were confirmed to originate from parental tumor cells, secrete alpha-fetoprotein, and express hepatic markers and beta-catenin. Hep293TT cells were able to form colonies in soft agar. Tumorigenicity was demonstrated by induction of solid tumors after subrenal capsule injection in immunodeficient mice. Hep293TT cells demonstrated a highly aneuploid karyotype, and a whole genome CGH analysis revealed chromosomal imbalances in every chromosome. Allelotype analysis demonstrated loss of alleles at distal 11p15.5 as is typical of embryonal tumors. Both Hep293TT cells and the primary tumor contain a deletion of 351 nucleotides in beta-catenin, as has been seen in other hepatoblastoma tumors. The cell line expressed beta-catenin protein in both full-length and partially deleted forms, and expressed NOTCH2 protein characteristic of hepatoblasts. No mutation was detected in the APC, MYH, MLH1, or MSH2 genes. CONCLUSION:This cell line, Hep293TT, is a valuable resource for the study of childhood liver cancer and may potentially provide a tool in the development of new agents.
journal_name
Pediatr Blood Cancerjournal_title
Pediatric blood & cancerauthors
Chen TT,Rakheja D,Hung JY,Hornsby PJ,Tabaczewski P,Malogolowkin M,Feusner J,Miskevich F,Schultz R,Tomlinson GEdoi
10.1002/pbc.22187subject
Has Abstractpub_date
2009-12-01 00:00:00pages
1040-7issue
6eissn
1545-5009issn
1545-5017journal_volume
53pub_type
杂志文章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
doi:10.1002/pbc.22951
更新日期:2011-05-01 00:00:00
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
doi:10.1002/pbc.25098
更新日期:2014-09-01 00:00:00
abstract:PURPOSE:To report the final analysis of survival outcomes for children with newly diagnosed high-grade glioma (HGG) treated on the "Head Start" (HS) II and III protocols with chemotherapy and intent to avoid irradiation in children <6 years old. PATIENTS AND METHODS:Between 1997 and 2009, 32 eligible children were enr...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26118
更新日期:2016-10-01 00:00:00
abstract::We report two survivors of Wilms tumor (WT) who developed inflammatory myofibroblastic tumor (IMT). The first patient had IMT in the mainstem bronchus 1 year after WT therapy that included vincristine, dactinomycin, doxorubicin (VDA) and whole lung radiation therapy (XRT). The second patient had IMT in the cecum 7 yea...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25345
更新日期:2015-06-01 00:00:00
abstract:BACKGROUND:Children with sickle cell disease (SCD) are at increased risk of death from invasive bacterial infections. Emergent evaluation of fever allows early treatment of potentially fatal infections. Limited data exist regarding caregiver adherence to physician recommendations of prompt medical evaluation of fever i...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章
doi:10.1002/pbc.25634
更新日期:2015-11-01 00:00:00
abstract::Although awareness of implicit bias and its influence on providers and patients is increasing, the effects of implicit bias on the field of pediatric hematology-oncology are less clear. This Special Report reviews the literature on implicit bias in pediatrics and medical oncology and further provides case examples and...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.28204
更新日期:2020-05-01 00:00:00
abstract:BACKGROUND:Hemophagocytic Lymphohistiocytosis (HLH) is characterized by uncontrolled inflammation that is generally fatal without immune modulating chemotherapy. At Texas Children's Hospital, we have observed significant central nervous system (CNS) toxicity in several patients treated for HLH according to the Histiocy...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21838
更新日期:2009-05-01 00:00:00
abstract::A 17-year-old girl with B-cell precursor acute lymphoblastic leukemia (BCP-ALL) with persistent minimal residual disease (MRD) who underwent standard chemotherapy was found to have a BCR-ABL1-like gene expression pattern. Genome sequencing revealed a JAK2 mutation not previously described in BCP-ALL and a potential th...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26328
更新日期:2017-05-01 00:00:00
abstract::Under and overweight, usually secondary to energy imbalance, are common complications of childhood chronic disease. A simple energy balance paradigm has been helpful in understanding the etiology of underweight and overweight in chronic disease, including malignant disease, particularly when measurements of total ener...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.21412
更新日期:2008-02-01 00:00:00
abstract::Limited high-quality evidence supports the management of iron deficiency anemia (IDA). To assess our institutional performance in this area, we retrospectively reviewed IDA treatment practices in 195 consecutive children referred to our center from 2006 to mid-2010. The majority of children were ≤4 years old (64%) and...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25861
更新日期:2016-04-01 00:00:00
abstract::We report on an acute myeloid leukemia in a neonate whose mother was exposed to diethylstilboestrol in utero. The newborn presented with leukemia cutis, hemorrhagic skin lesions, hyperleucocytosis and disseminated intravascular coagulation. A bone marrow examination confirmed the diagnosis of acute monocytic leukemia ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22040
更新日期:2009-08-01 00:00:00
abstract:BACKGROUND:Acute lymphoblastic leukemia (ALL) is the most common cancer in children. Because of major improvements in treatment protocols, the survival rate now exceeds 80%. However, ALL treatments can cause long-term neurocognitive sequelae, which negatively impact academic achievement and quality of life. Therefore, ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,多中心研究
doi:10.1002/pbc.27259
更新日期:2018-09-01 00:00:00
abstract:BACKGROUND:Hepatic sinusoidal obstruction syndrome (SOS) is a serious complication of autologous stem cell transplant (ASCT) in children with historically high mortality rates. Defibrotide has shown proven benefit in its treatment and may have a modest role in prevention. We report our experience with SOS in children u...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章
doi:10.1002/pbc.28677
更新日期:2020-11-01 00:00:00
abstract:BACKGROUND:Alveolar soft-part sarcoma (ASPS), a rare vascular sarcoma with a clinically indolent course, frequently presents with metastases. Vascular endothelial growth factor (VEGF) is a promising therapeutic target. In a phase-II trial of the VEGF receptor inhibitor cediranib for adults with ASPS, the partial respon...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,多中心研究
doi:10.1002/pbc.27987
更新日期:2019-12-01 00:00:00
abstract:BACKGROUND:Soft tissue sarcomas (STS) are a heterogeneous group of mesenchymal malignancies that occur throughout the lifespan. The impact of age on disease features and outcome is unclear. METHODS:We analyzed the clinical features and outcome of all STS cases registered between 1973 and 2006 in the SEER database. RE...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.23252
更新日期:2011-12-01 00:00:00
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
doi:10.1002/pbc.22280
更新日期:2010-02-01 00:00:00
abstract:BACKGROUND:Asparaginase (Asp) and corticosteroid (CS) treatment in patients with acute lymphoblastic leukaemia (ALL) is associated with an increased risk of thrombotic events. OBJECTIVE:Characterization of global haemostatic phenotypes of patients with ALL during Asp therapy. PROCEDURE:Thrombin generation (TG) was mo...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26228
更新日期:2017-02-01 00:00:00
abstract:BACKGROUND:Pediatric cancer-induced financial burden is source of stress for parents, particularly mothers, single parents, and parents with lower incomes. This financial burden has been linked to poorer family quality of life (QOL) in terms of new onset material hardships, and could also affect individual QOL in terms...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.28093
更新日期:2020-03-01 00:00:00
abstract:BACKGROUND:Pheochromocytomas (PCs) are neuroendocrine tumors arising from the chromaffin cells of the adrenal gland, and paragangliomas (PGLs) are their extra-adrenal counterparts arising from ganglia along the sympathetic/parasympathetic chain. Surgery is the cornerstone of treatment. A sporatic or inherited germline ...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/pbc.28332
更新日期:2020-08-01 00:00:00
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
doi:10.1002/pbc.27980
更新日期:2019-12-01 00:00:00
abstract::Undifferentiated embryonal sarcoma of the liver (UESL) is a rare aggressive mesenchymal pediatric tumor. Previously, reported outcomes have been very poor. Here, we report a single-center experience of five patients with UESL treated with upfront gross total resection and adjuvant chemotherapy. We have a median follow...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26154
更新日期:2016-12-01 00:00:00
abstract:BACKGROUND:Nephrogenic rests (NRs) are abnormally persistent foci of embryonal cells, thought to be the precursor lesion of Wilms tumors (WTs). To date, their presence has not been systematically examined in WTs treated with preoperative chemotherapy. METHODS:A systematic analysis of the data on NRs in WTs treated wit...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,多中心研究
doi:10.1002/pbc.26547
更新日期:2017-11-01 00:00:00
abstract:BACKGROUND:Survivors of childhood brain tumors are at increased risk for neurocognitive impairments, including deficits in abilities supported by frontal brain regions. Catechol-O-methyltransferase (COMT) metabolizes dopamine in the prefrontal cortex, with the Met allele resulting in greater dopamine availability and b...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24649
更新日期:2014-01-01 00:00:00
abstract::The youngest siblings may be both emotionally vulnerable and often neglected members of the family of a childhood cancer patient. The prompt identification of signs of distress in these subjects allows trained caregivers to intervene with personalized, age-appropriate, attention, and care. A narrative approach, based ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21056
更新日期:2008-03-01 00:00:00
abstract::The provision of tele-practice symptom management is often without the provision of evidence-based guidelines. Under the auspices of the Pediatric Oncology Group of Ontario, a nursing task force was established to appraise the evidence and develop guidelines. Promising new efforts to enhance symptom management through...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,实务指引,评审
doi:10.1002/pbc.22993
更新日期:2011-10-01 00:00:00
abstract:BACKGROUND:SIOPEL protocols have recommended liver transplantation for unresectable hepatoblastoma (HBL) after chemotherapy in absence of visible extrahepatic disease. METHODS:This retrospective single center study includes 13 children treated following SIOPEL 3 or 4 protocols who underwent orthotopic liver transplant...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.23301
更新日期:2011-12-15 00:00:00
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
doi:10.1002/pbc.24526
更新日期:2013-09-01 00:00:00
abstract:BACKGROUND:Thyroid dysfunction has been reported in up to 52% of patients 1.4 years after treatment with (131) I-Metaiodobenzylguanidine (MIBG) in children with neuroblastoma (NBL), despite the use of potassium-iodide (KI). Our aim was to investigate if the incidence and severity of thyroid damage increases in time. M...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24681
更新日期:2013-11-01 00:00:00
abstract::We report the results of a protocol for the diagnosis and treatment of pediatric non-Hodgkin lymphomas (NHL) conducted in Nicaragua in the context of an international collaborative program. Fifty-three children with NHL treated between 1996 and 2003 were retrospectively evaluated. Therapy was designed based on local d...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21046
更新日期:2008-01-01 00:00:00
abstract:BACKGROUND:Extramedullary infiltration (EMI) is an occasional clinical symptom in childhood acute myelogenous leukemia (AML), but there is considerable controversy regarding the prognostic significance of EMI in AML. PROCEDURE:We evaluated the frequency and prognostic significance of EMI at diagnosis of AML in childre...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.20824
更新日期:2007-04-01 00:00:00