Abstract:
BACKGROUND:Low expression of HLA class II antigens has been associated with more aggressive disease in several human malignancies including adult adrenocortical tumors (ACT), but their clinical relevance in pediatric ACT needs to be investigated. PROCEDURE:This study analyzed the expression profile of three class II histocompatibility genes (HLA-DRA, HLA-DPA1, and HLA-DPB1) in 58 consecutive pediatric ACT (13 adenomas and 45 carcinomas) by quantitative real time PCR and their association with clinical and biological features. HLA-DPA1 protein level was determined by immunohistochemistry. RESULTS:A significant association (P < 0.01) was observed between lower expression levels of the three genes analyzed and poor prognostic factors such as age ≥ 4 years, tumor size ≥ 200 cm(3), tumor weight ≥ 100 g, and metastatic disease; the presence of an unfavorable event and death. Underexpression of the HLA-DRA, HLA-DPA1, and HLA-DPB1 genes were associated with lower 5-year event-free survival (EFS) (P = 0.017, P < 0.001, and P = 0.017, respectively). Cox multivariate analysis showed that HLA-DPA1 was an independent prognostic factor (P = 0.029) when analyzed in association with stage IV, age and tumor size. Significantly lower EFS was also observed in patients with negative/weak immunostaining for HLA-DPA1 (P = 0.002). Similar results were observed when only patients classified as having carcinomas were analyzed. CONCLUSION:Our results suggest that lower expression of HLA-DRA, HLA-DPA1, and HLA-DPB1 genes may contribute to more aggressive disease in pediatric ACT. HLA-DPA1 immunostaining may represent potential aggressiveness marker in this tumor.
journal_name
Pediatr Blood Cancerjournal_title
Pediatric blood & cancerauthors
Leite FA,Lira RC,Fedatto PF,Antonini SR,Martinelli CE Jr,de Castro M,Neder L,Ramalho LN,Tucci S Jr,Mastelaro MJ,Seidinger AL,Cardinalli IA,Yunes JA,Brandalise SR,Tone LG,Scrideli CAdoi
10.1002/pbc.25118subject
Has Abstractpub_date
2014-11-01 00:00:00pages
1940-8issue
11eissn
1545-5009issn
1545-5017journal_volume
61pub_type
杂志文章abstract:PURPOSE:Chemotherapy during radiation and/or bone-seeking radioisotope therapy (153-samarium; 1 mCi/kg) during radiation may improve osteosarcoma cancer control. PATIENTS AND METHODS:We analyzed our preliminary radiation experience in high-risk, metastatic, and/or recurrent patients during a consecutive period of 20 m...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21451
更新日期:2008-05-01 00:00:00
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
doi:10.1002/pbc.24281
更新日期:2013-03-01 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:BACKGROUND:The aim of this study is to evaluate the relationship between the latency to diagnosis (LD) and the time to completion of chemotherapy (TCC) with clinical outcomes in children with osteosarcoma. METHODS:We performed a retrospective analysis of all patients who received treatment for osteosarcoma in two tert...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/pbc.28180
更新日期:2020-04-01 00:00:00
abstract::Most publications report the adverse (negative) health issues in childhood cancer survivors. Presenting information to the newly diagnosed patient in a positive manner is advocated, while noting that recurrence is the most likely adverse event. Re-analysis of population-based studies on life-threatening toxicities fro...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.22975
更新日期:2011-12-15 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::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: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::Hemophilia is an excellent example in medicine where clinical translation of basic science discoveries has transformed the gloomy outlook of the disease. This review provides an overview of clinical advances in hemophilia management with a specific focus on the molecular heterogeneity of the disease and progress in ma...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.23193
更新日期:2011-12-01 00:00:00
abstract::Developing countries with an uncontrolled AIDS epidemic have new challenges to meet in ITP. Secondary ITP, HIV related, becomes an increasing problem, which has many aspects that need addressing, including medical, effective counseling, psychosocial and unresolved management issues. Assistance in developing treatment ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21020
更新日期:2006-10-15 00:00:00
abstract:BACKGROUND:We investigated the spectrum, frequency, and risk factors for renal, gastrointestinal, and hepatic late adverse effects in survivors of childhood acute myeloid leukemia (AML) without relapse treated with chemotherapy alone according to three consecutive AML trials by the Nordic Society of Pediatric Hematolog...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25069
更新日期:2014-09-01 00:00:00
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
doi:10.1002/pbc.20825
更新日期:2007-12-01 00:00:00
abstract:BACKGROUND:Radiotherapy (RT) is well established in the management of intracranial ependymoma (EP) and post-operative RT is employed for the majority of patients. There are no randomised trials of RT in EP and evidence for dose and volume relies on retrospective single institution series, usually comprising a heterogen...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.10470
更新日期:2004-05-01 00:00:00
abstract:BACKGROUND:Transcranial Doppler ultrasound (TCD) has been demonstrated to be a powerful predictor of stroke risk due to sickle cell disease (SCD) in pediatric populations. Little is known about how this healthcare innovation has disseminated into preventive care for SCD. The objective of this study was to determine TCD...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21677
更新日期:2008-11-01 00:00:00
abstract:BACKGROUND:The aim of this study was to evaluate the value of follow-up investigations of T-cell acute lymphoblastic leukemia (T-ALL) and T-cell non-Hodgkin's lymphoma (T-NHL), including cerebrospinal fluid (CSF) examination, bone marrow (BM) aspiration, peripheral blood (PB) count, serum lactate dehydrogenase (LDH) an...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.20813
更新日期:2007-04-01 00:00:00
abstract:OBJECTIVE:Current guidelines recommend high-priority treatment of severe sickle cell disease (SCD) pain with opioids; however, patients with SCD have historically been undertreated. We used mixed methods to assess pediatric residents' perceptions toward opioid use in SCD pain management. METHODS:We distributed a surve...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27535
更新日期:2019-02-01 00:00:00
abstract::Langerhans cell histiocytosis is known to mimic many other conditions. We present two patients where anti-tubercular therapy was instituted when clinical and radiological features suggested tuberculosis. The correct diagnosis of histiocytosis was reached only on further work-up including immunohistochemistry following...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21930
更新日期:2009-07-01 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::Pediatric renal cell carcinoma (RCC) is a rare cancer that can be associated with inherited diseases including tuberous sclerosis complex (TSC) caused by germline mutations in TSC1 or TSC2. Somatic mutations in TSC1 and TSC2 have also been reported in adult RCC, which predict response to mTOR inhibitors. Here, we pres...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26286
更新日期:2017-05-01 00:00:00
abstract::The Standards, Options, and Recommendations (SOR) project undertaken by the French National Federation of Cancer Centers (FNCLCC) to develop and disseminate clinical practice guidelines in oncology has now been taken over by the French National Cancer Institute. In 2007, the SOR updated the information related to the ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.21953
更新日期:2009-07-01 00:00:00
abstract::As increasing numbers of childhood cancer patients are surviving, the long-term complications of the disease and its treatment have become ever more increasingly important. Reduced bone mineral density and increased fracture risk have been reported during and after treatment of children with cancer. The causes of oste...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.21407
更新日期:2008-02-01 00:00:00
abstract::A male patient diagnosed with severe congenital protein C (PC) deficiency during the neonatal period was treated with long-term warfarin but frequently developed purpura fulminans and bleeding. At four years of age, edoxaban was initiated (direct oral anticoagulant [DOAC]). His d-dimer and fibrin/fibrinogen degradatio...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27686
更新日期:2019-06-01 00:00:00
abstract::Obesity is increasing in prevalence in the United States with over 65% of adults considered overweight and 16% of children with BMI > 95 percentile. The heritability of obesity is estimated between 40% and 70%, but the genetics of obesity for most individuals are complex and involve the interaction of multiple genes a...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.23372
更新日期:2012-01-01 00:00:00
abstract:BACKGROUND:Knowledge about their past medical history is central for childhood cancer survivors to ensure informed decisions in their health management. Knowledge about information provision and information needs in this population is still scarce. We thus aimed to assess: (1) the information survivors reported to have...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24762
更新日期:2014-02-01 00:00:00
abstract:BACKGROUND:Malignant ectomesenchymoma (MEM) is a soft tissue tumor with heterologous rhabdomyoblastic components believed to arise from pluripotent migratory neural crest cells. To date merely 50 cases have been published and the knowledge about the course of disease and optimal treatment is limited. METHODS:Six patie...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24174
更新日期:2013-02-01 00:00:00
abstract:INTRODUCTION:Thalassemia major (TM) is an inherited disorder caused by ineffective erythropoiesis. At the present time, allogeneic stem cell transplantation (allo-SCT) is a curative option. Conventional busulfan and cyclophosphamide based myeloablative conditioning regimens are limited by increased toxicity, especially...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27312
更新日期:2018-11-01 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::Malignant degeneration frequently arises from preexisting plexiform neurofibroma in patients with neurofibromatosis type 1 (NF1). Image guided biopsy for diagnostic purposes, such as with CT guidance, can be technically challenging in these patients, as CT cannot distinguish malignant from benign areas within the same...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24668
更新日期:2013-12-01 00:00:00
abstract:BACKGROUND:Neuroblastoma is characterized by heterogeneity of histology, biology, and clinical behavior. Most epidemiology studies are based on Western and Japanese populations; there are very few studies on neuroblastoma from Southeast Asia. PROCEDURE:Cases of Thai children with neuroblastoma were retrospectively rev...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.23325
更新日期:2012-05-01 00:00:00
abstract:BACKGROUND:To evaluate outcomes of Group D eyes of bilateral retinoblastoma patients treated with primary chemoreduction and external beam radiation as salvage. PROCEDURE:Retrospective chart review of patients diagnosed with bilateral retinoblastoma and designated Group D in at least one eye from January 1, 2000 to De...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24303
更新日期:2013-04-01 00:00:00