Low expression of HLA-DRA, HLA-DPA1, and HLA-DPB1 is associated with poor prognosis in pediatric adrenocortical tumors (ACT).

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 Cancer

journal_title

Pediatric blood & cancer

authors

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 CA

doi

10.1002/pbc.25118

subject

Has Abstract

pub_date

2014-11-01 00:00:00

pages

1940-8

issue

11

eissn

1545-5009

issn

1545-5017

journal_volume

61

pub_type

杂志文章
  • Multimodality treatment of osteosarcoma: radiation in a high-risk cohort.

    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

    authors: Mahajan A,Woo SY,Kornguth DG,Hughes D,Huh W,Chang EL,Herzog CE,Pelloski CE,Anderson P

    更新日期:2008-05-01 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

    doi:10.1002/pbc.24281

    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

  • 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

    doi:10.1002/pbc.26603

    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

  • Prognostic impact of diagnostic and treatment delays in children with osteosarcoma.

    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

    authors: Vasquez L,Silva J,Chavez S,Zapata A,Diaz R,Tarrillo F,Maza I,Sialer L,García J

    更新日期:2020-04-01 00:00:00

  • Late effects in childhood cancer survivors: a review with a framing effect bias?

    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

    authors: Fryer C

    更新日期:2011-12-15 00:00:00

  • Familial small cell carcinoma of the ovary.

    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

    authors: Martinez-Borges AR,Petty JK,Hurt G,Stribling JT,Press JZ,Castellino SM

    更新日期:2009-12-15 00:00:00

  • NHL in adolescents and young adults: A unique population.

    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

    authors: Hochberg J,Flower A,Brugieres L,Cairo MS

    更新日期:2018-08-01 00:00:00

  • Defibrotide treatment but not prophylaxis is useful in hepatic sinusoidal obstruction syndrome in children undergoing autologous stem cell transplant following high-dose chemotherapy: A single-center experience from the Royal Marsden Hospital, UK.

    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

    authors: Roy Moulik N,Johnson I,Van Bruggen L,Petterson T,Mycroft J,Vaidya SJ

    更新日期:2020-11-01 00:00:00

  • Clinical advances in hemophilia management.

    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

    authors: Sharathkumar AA,Carcao M

    更新日期:2011-12-01 00:00:00

  • Psychosocial aspects of immune thrombocytopenia and secondary human immunodeficiency virus-related pediatric immune thrombocytopenia in the Republic of South Africa.

    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

    authors: Wainwright RD,Poyiadjis S,Naidu G,Mackinnon D

    更新日期:2006-10-15 00:00:00

  • Renal, gastrointestinal, and hepatic late effects in survivors of childhood acute myeloid leukemia treated with chemotherapy only--a NOPHO-AML study.

    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

    authors: Skou AS,Glosli H,Jahnukainen K,Jarfelt M,Jónmundsson GK,Malmros-Svennilson J,Nysom K,Hasle H,Nordic Society of Pediatric Hematology and Oncology.

    更新日期:2014-09-01 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

    doi:10.1002/pbc.20825

    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

  • Review of radiotherapy dose and volume for intracranial ependymoma.

    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

    authors: Taylor RE

    更新日期:2004-05-01 00:00:00

  • A critical assessment of transcranial doppler screening rates in a large pediatric sickle cell center: opportunities to improve healthcare quality.

    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

    authors: Raphael JL,Shetty PB,Liu H,Mahoney DH,Mueller BU

    更新日期:2008-11-01 00:00:00

  • The clinical value of follow-up examinations in childhood T-cell acute lymphoblastic leukemia and T-cell non-Hodgkin's lymphoma.

    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

    authors: Huang L,Lequin M,Pieters R,van den Heuvel-Eibrink MM

    更新日期:2007-04-01 00:00:00

  • Pediatric residents' perceived barriers to opioid use in sickle cell disease pain management.

    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

    authors: Fearon A,Marsh A,Kim J,Treadwell M

    更新日期:2019-02-01 00:00:00

  • Langerhans cell histiocytosis masquerading as tuberculosis: a diagnostic dilemma resulting in inappropriate anti-tubercular therapy.

    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

    authors: Sharma P,Dhingra KK,Sural S,Mandal AK,Singh T

    更新日期:2009-07-01 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

    doi:10.1002/pbc.24526

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

    更新日期:2013-09-01 00:00:00

  • Renal cell carcinoma harboring somatic TSC2 mutations in a child with methylmalonic acidemia.

    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

    authors: Potter SL,Venkatramani R,Wenderfer S,Graham BH,Vasudevan SA,Sher A,Wu H,Wheeler DA,Yang Y,Eng CM,Gibbs RA,Roy A,Plon SE,Parsons DW

    更新日期:2017-05-01 00:00:00

  • 2007 Standards, Options, and Recommendations: use of erythropoiesis-stimulating agents (ESA: epoetin alfa, epoetin beta, and darbepoetin) for the management of anemia in children with cancer.

    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

    authors: Marec-Berard P,Chastagner P,Kassab-Chahmi D,Casadevall N,Marchal C,Misset JL,Ray-Coquard I

    更新日期:2009-07-01 00:00:00

  • Osteoporosis in children with cancer.

    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

    authors: van der Sluis IM,van den Heuvel-Eibrink MM

    更新日期:2008-02-01 00:00:00

  • Management of severe congenital protein C deficiency with a direct oral anticoagulant, edoxaban: A case report.

    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

    authors: Watanabe K,Arakawa Y,Yanagi M,Isobe K,Mori M,Koh K

    更新日期:2019-06-01 00:00:00

  • An overview of mongenic and syndromic obesities in humans.

    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

    authors: Chung WK

    更新日期:2012-01-01 00:00:00

  • Information provision and information needs in adult survivors of childhood cancer.

    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

    authors: Gianinazzi ME,Essig S,Rueegg CS,von der Weid NX,Brazzola P,Kuehni CE,Michel G,Swiss Paediatric Oncology Group (SPOG).

    更新日期:2014-02-01 00:00:00

  • Malignant ectomesenchymoma in children and adolescents: report from the Cooperative Weichteilsarkom Studiengruppe (CWS).

    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

    authors: Dantonello TM,Leuschner I,Vokuhl C,Gfroerer S,Schuck A,Kube S,Nathrath M,Bernbeck B,Kaatsch P,Pal N,Ljungman G,Bielack SS,Klingebiel T,Koscielniak E,CWS.

    更新日期:2013-02-01 00:00:00

  • Fludarabine-based reduced toxicity yet myeloablative conditioning is effective and safe particularly in patients with high-risk thalassemia undergoing allogeneic transplantation.

    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

    authors: Sheth V,Grisariu S,Avni B,Stepensky P,Ashkenazi M,Shapira MY,Or R

    更新日期:2018-11-01 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

    doi:10.1002/pbc.27980

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

    更新日期:2019-12-01 00:00:00

  • PET-guided biopsy with needle navigation facilitates diagnosis of angiosarcoma in neurofibromatosis type 1.

    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

    authors: Koethe Y,Widemann BC,Hajjar F,Wood BJ,Venkatesan AM

    更新日期:2013-12-01 00:00:00

  • Natural course of low risk neuroblastoma.

    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

    authors: Shuangshoti S,Shuangshoti S,Nuchprayoon I,Kanjanapongkul S,Marrano P,Irwin MS,Thorner PS

    更新日期:2012-05-01 00:00:00

  • Long-term outcomes of Group D eyes in bilateral retinoblastoma patients treated with chemoreduction and low-dose IMRT salvage.

    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

    authors: Berry JL,Jubran R,Kim JW,Wong K,Bababeygy SR,Almarzouki H,Lee TC,Murphree AL

    更新日期:2013-04-01 00:00:00