Abstract:
BACKGROUND:Partial nephrectomy (NSS) for unilateral nephroblastoma may be beneficial, although in case of regional lymph node (LN) involvement, radiotherapy counteracts the functional benefit of NSS. The aim is to verify whether decrease of tumor volume under preoperative chemotherapy implies clearance of regional LN. PROCEDURE:SIOP 9301 (1993-2001) collected 1,450 localized nephroblastoma patients of whom 1,360 (93%) had sufficiently available data and were retrospectively reviewed. RESULTS:Histologic subtypes were classically distributed. Patients were divided in those with tumor positive LN (76, 5.5%) and those with tumor negative LN (1,284, 94.5%) at surgery. In the LN(+) group, the tumor volume changed from a median of 554 (318-772) to 192 (63-458) ml = 67% (27-88%) during preoperative ChT. In the LN(-) group-377 (200-612) to 130 (44-294) ml = 62% (28-83%) (NS). Increase of tumor volume was observed in 16% of patients with LN(+), and 11% of those with LN(-) (NS); ranges are interquartile. Initial tumor volume was significantly larger in the LN(+) patients (P = 0.00091) but not different (NS) at surgery; patients with initial tumor volume under 318 ml had the regional LN involved significantly less frequently (P = 0.00751). CONCLUSIONS:Change in tumor volume under preoperative chemotherapy is not a predictor for LN status at surgery, although larger initial volume is associated with a higher risk of LN invasion. The decrease of tumor volume is not a good criterion for the safety of NSS. The low rate of LN(+) (5.5%) indicates that this risk is low.
journal_name
Pediatr Blood Cancerjournal_title
Pediatric blood & cancerauthors
Godzinski J,van Tinteren H,de Kraker J,Graf N,Bergeron C,Heij H,von Schweinitz D,Fuchs J,Cecchetto G,Audry G,Gauthier F,Sandstedt B,SIOP Nephroblastoma Trial & Study Committee.doi
10.1002/pbc.23147subject
Has Abstractpub_date
2011-12-15 00:00:00pages
1266-9issue
7eissn
1545-5009issn
1545-5017journal_volume
57pub_type
杂志文章abstract:BACKGROUND:Sickle cell anaemia (SCA) is the leading genetic disorder in Nigeria. Elevated velocities ≥170 cm/sec occur in about a third of Nigerian children with SCA. Chronic blood transfusion for stroke prevention is faced with a myriad of challenges in our practice. OBJECTIVES:To evaluate the effectiveness of hydrox...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25529
更新日期:2015-09-01 00:00:00
abstract:BACKGROUND:Children with hepatoblastoma (HB) are at risk of sarcopenia due to immobility, chemotherapy, and malnutrition. We hypothesized that children with HB have a low preoperative total psoas muscle area (tPMA), reflecting sarcopenia, which negatively impacts outcome. PROCEDURE:Retrospective study of children (1-1...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.28862
更新日期:2021-01-12 00:00:00
abstract:BACKGROUND:This study investigates the effect of using patient reported outcomes (PROs) about health-related quality of life (HRQOL) in clinical practice on the type and amount of psychosocial topics discussed during a paediatric oncology consultation. PROCEDURE:Children (N = 193) with cancer participated in a sequent...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/pbc.24089
更新日期:2012-07-15 00:00:00
abstract:BACKGROUND:There is a need for more knowledge about how survivors of childhood cancer perceive their lives and what influence current health status has on their quality of life. The purpose was to describe this among a group of long-term survivors and among a comparison group. PROCEDURE:Telephone interviews were perfo...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22492
更新日期:2010-08-01 00:00:00
abstract::Hepatoblastoma (HB) and biliary atresia (BA) are both rare conditions that occurred in the patient described. This is the second such case in the literature. An explanation for this apparent coincidence could possibly be found in the existance of pluripotent liver stem cells. In humans, small epithelial cells (SEC) be...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.20115
更新日期:2004-10-01 00:00:00
abstract:BACKGROUND:Platelet counts below normal values define thrombocytopenia. However, platelet counts alone do not reveal the underlying pathomechanism. New blood cell counters provide additional information on platelet size and volume, and enable the distinction of sub-populations. In this preliminary study, we evaluate wh...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22907
更新日期:2011-10-01 00:00:00
abstract::Phytosterolemia is a rare autosomal recessive sterol storage disease caused by mutations in ABCG5 and ABCG8 genes. A 9-year-old Turkish boy who was presented with exclusively hematologic abnormalities had elevated plant sterol levels. Sequencing of ABCG5 and ABCG8 genes revealed a novel homozygous IVS10-1 G>T mutation...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24934
更新日期:2014-08-01 00:00:00
abstract:BACKGROUND:Treatment abandonment (TxA) is a primary cause of therapy failure in children with cancer in low-/middle-income countries. We explored the absence of social support network (SSN), among other predictive factors, and TxA in children with cancer in Cali, Colombia. PROCEDURE:In this prospective cohort study, w...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25919
更新日期:2016-05-01 00:00:00
abstract:BACKGROUND:Conflicting data exist regarding whether low bone mineral density (BMD) is associated with Fanconi anemia (FA). The current study identified the frequency of low BMD in FA, expecting low BMD even in childhood and before HCT. PROCEDURE:Thirty-seven FA patients (18 prior HCT, 19 no prior HCT), participating i...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22956
更新日期:2011-12-01 00:00:00
abstract:BACKGROUND:Severe congenital neutropenia is a rare disease, and autosomal dominantly inherited ELANE mutation is the most frequently observed genetic defect in the registries from North America and Western Europe. However, in eastern countries where consanguineous marriages are common, autosomal recessive forms might b...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27923
更新日期:2019-10-01 00:00:00
abstract::We present here a case of MIRAGE syndrome due to novel variant (c.2318T>C) in the sterile α motif domain-containing protein 9 (SAMD9) gene. Previous reports have described the clinical phenotype, which includes myelodysplasia, recurrent infections, restriction of growth and development, adrenal insufficiency, genitour...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.27726
更新日期:2019-07-01 00:00:00
abstract::Inherited bone marrow failure syndromes (IBMFS) are rare cancer predisposition syndromes with an especially high risk of transformation to myelodysplastic syndrome (MDS) and/or acute myeloid leukemia (AML). We performed a retrospective systematic review of reported MDS/AML arising in the eight most common IBMFS to det...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.26714
更新日期:2017-12-01 00:00:00
abstract:BACKGROUND:Specialized pediatric cancer centers (PCCs) are thought to be essential to obtain state-of-the-art care for children and adolescents. We determined the proportion of childhood cancer patients not treated in a PCC, and described their characteristics and place of treatment. PROCEDURE:The Swiss Childhood Canc...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,多中心研究
doi:10.1002/pbc.22426
更新日期:2010-05-01 00:00:00
abstract::Familial hemophagocytic lymphohistiocytosis (FHLH) is an autosomal recessive disorder of cytotoxic cell function that results in abnormal proliferation of benign lymphocytes and histiocytes in response to infectious stimuli. FHLH generally occurs in very young children, and typically presents with fever, cytopenias, c...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21438
更新日期:2008-05-01 00:00:00
abstract::Pediatric cancer programs in low-income countries (LIC) can improve outcomes. However, treatment must be tailored to the patient's living conditions and the availability of supportive care. In some cases, a more intense regimen will decrease survival since the increase in death from toxicity may exceed any decrease in...
journal_title:Pediatric blood & cancer
pub_type:
doi:10.1002/pbc.20989
更新日期:2007-04-01 00:00:00
abstract::We describe a child with dyserythropoietic anemia, thrombocytosis, functional platelet defect, and megakaryocyte dysplasia. We show that (i) this constellation of hematopoietic abnormalities was due to a germline mutation within the 5' untranslated region (5'UTR) of globin transcription factor 1 (GATA1); (ii) the muta...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25871
更新日期:2016-05-01 00:00:00
abstract::Ovarian tumors have a low incidence in childhood, accounting for 1% of malignancies within the ages of 0-17 years. Small cell carcinoma of the ovary is a rare histology and historically has a poor prognosis. We report a case of an 11-year-old female diagnosed with small cell carcinoma of the ovary and hypercalcemia (S...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22184
更新日期:2009-12-15 00:00:00
abstract:BACKGROUND:For children with juvenile myelomonocytic leukemia (JMML) who undergo stem cell transplantation (SCT), the role of immunological interventions including withdrawal of immunosuppressive therapy (IST) and donor lymphocyte infusion (DLI) for treatment of disease recurrence remains uncertain. PROCEDURE:We analy...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24259
更新日期:2013-01-01 00:00:00
abstract:BACKGROUND:Despite the increasing cure rates for children with acute lymphoblastic leukemia (ALL), patients who relapse continue to have poor prognosis. The Children's Oncology Group (COG) conducted a limited institution Phase II trial of Campath-1H, a monoclonal antibody that targets CD52 on leukemic cells, in childre...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,多中心研究
doi:10.1002/pbc.22209
更新日期:2009-12-01 00:00:00
abstract::Survivors of childhood cancer are at risk for dental late effects. This systematic review summarizes associations between treatment exposures and dental late effects among survivors of childhood cancer. We included investigations with at least 20 study participants conducted for 2 or more years after completion of chi...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.24842
更新日期:2014-03-01 00:00:00
abstract:BACKGROUND:Several sickle cell clinical trials have closed due to inability to enroll patients. To limit the early cessation of a proposed clinical trial due to low accrual rates, we sought to better understand barriers and facilitators to enrolling parents of children with sickle cell anemia (SCD) into clinical trials...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,随机对照试验
doi:10.1002/pbc.24486
更新日期:2013-08-01 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
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
doi:10.1002/pbc.26603
更新日期:2017-10-01 00:00:00
abstract::A 4-year-old girl with Noonan syndrome (NS) and constitutive PTPN11 mutation presented with stage 4 neuroblastoma and was treated by intensive chemotherapy. During the treatment, cytogenetic analysis revealed the development of a hyperdiploid clone with duplication of the germline PTPN11 mutation in a morphologically ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.20527
更新日期:2007-01-01 00:00:00
abstract:OBJECTIVES:The aim of this study was to assess glomerular and tubular renal function after HSCT in children in a prospective trial. METHODS:Renal function was assessed prospectively before HSCT (on day -10), on days +30, +100, and at least 6 months after transplantation in 34 patients (21 females/13 males) with a mean...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22030
更新日期:2009-08-01 00:00:00
abstract:BACKGROUND:Alvespimycin (17-DMAG, KOS-1022), a potent small-molecule inhibitor of the protein chaperone Hsp90, is being developed as an anticancer agent because of the multiple Hsp90 client proteins involved in cancer cell growth and survival. PROCEDURES:Alvespimycin was tested against the in vitro panel of the Pediat...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21508
更新日期:2008-07-01 00:00:00
abstract::Post-transplant lymphoproliferative disease (PTLPD), due to the reactivation of Epstein-Barr virus (EBV), is a serious complication. The risk of the disorder increases with T-cell depletion methods, mismatched hematopoietic stem cell transplantation (HSCT), graft-versus-host disease (GVHD), and immunosuppression. In c...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.20710
更新日期:2007-11-01 00:00:00
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
doi:10.1002/pbc.27903
更新日期:2019-10-01 00:00:00
abstract::Non-Hodgkin lymphoma (NHL) is a heterogeneous group of lymphoid malignancies with high incidence in adolescents and young adults (AYAs). The most common diseases include diffuse large B-cell lymphoma, anaplastic large cell lymphoma, Burkitt lymphoma, lymphoblastic lymphoma, and primary mediastinal large B-cell lymphom...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.27073
更新日期:2018-08-01 00:00:00
abstract:BACKGROUND:Childhood cancer survivors (CCS) are at risk of developing long-term morbidity, which is likely to be presented to a primary care physician (PCP). Therefore, insight into CCS's PCP-based health care use is needed. We investigated the volume and underlying health problems of PCP-based health care use and the ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27774
更新日期:2019-08-01 00:00:00