Abstract:
BACKGROUND:We wanted to ascertain patterns of recurrence, re-treatment, and outcome among 188 eligible patients treated for localized orbital sarcoma on IRSG Protocols III/IV, 1984-1997. PROCEDURE:Retrospective chart review. RESULTS:Twenty-four of 188 patients (12.8%) developed local (n = 22) or distant relapse (n = 2) at 0.057-7.05 years (median, 1.58) after enrollment. Ages at study entry were 0.14-17 years (median, 5 years). Initial tumor operations included biopsy (n = 20) or gross resection with microscopic residual (n = 4). Initial tumor diameters were 0.5-7 cm (median, 3). Pathologic subtypes were embryonal rhabdomyosarcoma (ERMS, n = 19), sarcoma not otherwise specified (n = 2), and alveolar RMS, botryoid ERMS, or undifferentiated sarcoma (n = 1 each). Initial treatment included vincristine/dactinomycin (n = 24) including an alkylator (n = 4) and radiotherapy (RT, n = 21). Twenty patients responded, 14 completely, 6 partially. After recurrence, patients underwent orbital exenteration (n = 10), enucleation (2), tumor excision (3), or biopsy (1); 7 had no operation, and 1 had no data. Post-relapse chemotherapy included combinations of etoposide (n = 14 patients), doxorubicin (14), ifosfamide (12), cyclophosphamide (7), and dacarbazine (n = 1). Six patients received RT, including four previously treated and two not irradiated initially. Two patients died; one at 1.79 years after contralateral brain metastasis followed by local recurrence, and another at 2.49 years after multiple local recurrences. Twenty-two patients (91.7%) survived sarcoma-free for 0.04-17 years (median, 6.9) after relapse, and 18 of 22 (82%) were alive ≥5 years after relapse. CONCLUSION:Survival following recurrent localized orbital sarcoma appears likely after vigorous re-treatment given with curative intent.
journal_name
Pediatr Blood Cancerjournal_title
Pediatric blood & cancerauthors
Raney B,Huh W,Hawkins D,Hayes-Jordan A,Million L,Rodeberg D,Teot L,Anderson J,Soft Tissue Sarcoma Committee of the Children's Oncology Group, Arcadia, CA.doi
10.1002/pbc.24289subject
Has Abstractpub_date
2013-03-01 00:00:00pages
371-6issue
3eissn
1545-5009issn
1545-5017journal_volume
60pub_type
历史文章,杂志文章abstract:BACKGROUND:Hypogammaglobulinemia (hypo-IgG) is common early post-HSCT in children, occasionally necessitating long-term immunoglobulin (Ig) G replacement therapy. IgG replacement may not reduce mortality, although infectious complications are decreased PROCEDURE:Clinical data and samples from 86 children were analyzed...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25409
更新日期:2015-05-01 00:00:00
abstract::Opportunities for cancer prevention in adolescents range from limitation of sun exposure to the use of human papillomavirus vaccines. Those who develop malignant disease experience longer waiting times for diagnosis and treatment than do children, especially when referred to adult treatment centers, and they are less ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.20634
更新日期:2006-03-01 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:The high cost, coupled with the need for continuous infusion, renders Desferrioxamine (DFO), a non-feasible option for iron-chelation in a large majority of patients with β-thalassemia major in developing countries. Monotherapy with deferiprone (DFP) or deferasirox (DFX) may not always attain optimal control...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25533
更新日期:2015-09-01 00:00:00
abstract::Hemophagocytic lymphohistioytosis (HLH) is a severe, life-threatening hyperinflammatory disorder that requires prompt diagnosis and treatment. Approximately, 25-50% of patients with HLH fail to achieve remission with established regimens that include dexamethasone and etoposide, or methylprednisolone and antithymocyte...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.26308
更新日期:2017-04-01 00:00:00
abstract::Anaphylactic/anaphylactoid reaction to methotrexate (MTX) is uncommon. It may occur with the first dose (non-allergic reactions) or after a previous exposure to the drug (allergic or specific reactions). Desensitization has been shown effective in children with allergic-type reactions permitting the continuation of hi...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22616
更新日期:2010-09-01 00:00:00
abstract:BACKGROUND:Stroke is a severe complication of sickle cell anemia (SCA). The role of glucose-6-phosphate dehydrogenase (G6PD) deficiency in the development of stroke in children with SCA is controversial. PROCEDURE:The aim of this study was to investigate the association of clinical ischemic stroke, high-risk transcran...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25924
更新日期:2016-06-01 00:00:00
abstract:BACKGROUND:Ispinesib is a highly specific inhibitor of kinesin spindle protein (KSP, HsEg5), a mitotic kinesin required for separation of the spindle poles. Here we report the activity of ispinesib against the in vitro and in vivo panels of the Pediatric Preclinical Testing Program (PPTP). PROCEDURES:Ispinesib was tes...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22056
更新日期:2009-12-15 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
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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
doi:10.1002/pbc.24389
更新日期:2013-06-01 00:00:00
abstract:BACKGROUND:Primary mediastinal germ cell tumors (GCTs) are rare in children and still represent a challenge for both adult and pediatric oncologists because of their worse outcome compared to their gonadal counterpart. PROCEDURE:Prospectively collected data concerning patients enrolled in the Italian Association of Pe...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/pbc.25895
更新日期:2016-05-01 00:00:00
abstract:BACKGROUND:While pediatric low-grade glioma/glioneuronal tumors (LGG/LGGNTs) are considered slow-growing, indolent tumors with excellent long-term prognosis, mortality due to the disease is not unknown. Few studies have addressed the cause of death in this population. METHODS:Retrospective review of clinicopathologic ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26717
更新日期:2018-01-01 00:00:00
abstract:PURPOSE:To evaluate and compare psychological distress in long-term survivors of solid tumors diagnosed in childhood and their siblings, and to identify significant correlates of psychological distress. PROCEDURE:Adult survivors (2,778) of solid tumors diagnosed in childhood and 2,925 siblings completed a long-term fo...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,多中心研究
doi:10.1002/pbc.20914
更新日期:2007-07-01 00:00:00
abstract::Natural killer (NK) cell lymphoma of lung is very rare. We report a case of NK cell lymphoma presented in a 17-year-old male with bilateral pleural effusions and hemophagocytic lymphohistocytosis. Morphologic and immunohistochemical tests revealed an association of NK cell lymphoma with Epstein-Barr virus. A literatur...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21943
更新日期:2009-05-01 00:00:00
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
doi:10.1002/pbc.28702
更新日期:2020-12-01 00:00:00
abstract:BACKGROUND:Infection in neutropenic children is a major cause of morbidity and mortality in children treated for cancer. In developing countries, children with cancer are often malnourished at diagnosis. In Blantyre, Malawi, children with Burkitt lymphoma are treated with a local protocol with limited toxicity. The aim...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22032
更新日期:2009-07-01 00:00:00
abstract::Deferasirox (Exjade®, Novartis) is a widely used oral iron chelator for the treatment of patients with iron overload due to chronic transfusion therapy for diseases such as β-thalassemia and sickle cell disease. Renal side effects of deferasirox are common and include non-progressive increases in serum creatinine, how...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22711
更新日期:2011-04-01 00:00:00
abstract:BACKGROUND:The prognostic significance of having extraskeletal (EES) versus skeletal Ewing sarcoma (ES) in the setting of modern chemotherapy protocols is unknown. The purpose of this study was to compare the clinical characteristics, biologic features, and outcomes for patients with EES and skeletal ES. METHODS:Patie...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26096
更新日期:2016-10-01 00:00:00
abstract:BACKGROUND:Pulmonary hypertension (PHT) may be the leading cause of death in β-thalassemia patients; however, its pathophysiologic mechanisms are still unclear. Recent studies indicate that asymmetric dimethylarginine (ADMA) plays a role in the initiation and progression of a variety of diseases, especially the cardiov...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25076
更新日期:2014-09-01 00:00:00
abstract:BACKGROUND:Testicular germ cell tumors (T-GCTs) occur from infancy to adulthood, and are the most common solid tumor in adolescent and young adult males. Traditionally, pediatric T-GCTs were perceived as more indolent than adult T-GCTs. However, there are few studies comparing these groups and none that specifically ev...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24773
更新日期:2014-03-01 00:00:00
abstract:BACKGROUND:ABT-751, an orally bioavailable sulfonamide, binds beta-tubulin to inhibit microtubule polymerization. We described response and event-free survival (EFS) in children with neuroblastoma and other solid tumors receiving ABT-751, assessed in vitro cytotoxicity of ABT-751 and evaluated the effect of ABT-751 on ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22267
更新日期:2010-01-01 00:00:00
abstract::A case of invasive, keratinizing squamous cell carcinoma of the larynx in an 8-year-old female treated with laryngectomy is presented. Perinatal exposure to human papilloma virus and constitutional heterozygosity for a FANCC mutation were identified, though FANCC heterozygosity is not known to be cancer predisposing. ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
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更新日期:2017-08-01 00:00:00
abstract:BACKGROUND:The prognostic impact of central nervous system (CNS) involvement in children with acute myeloid leukemia (AML) has varied in past trials, and controversy exists over the degree of involvement requiring intensified CNS therapy. Two recent Children's Oncology Group protocols, AAML03P1 and AAML0531, directed a...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26612
更新日期:2017-12-01 00:00:00
abstract::The commonly accepted definition of Fanconi anemia (FA) relying on DNA repair deficiency is submitted to a critical review starting from the early reports pointing to mitomycin C bioactivation and to the toxicity mechanisms of diepoxybutane and a group of nitrogen mustards causing DNA crosslinks in FA cells. A critica...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.25452
更新日期:2015-07-01 00:00:00
abstract:BACKGROUND:Many pediatric oncology patients undergoing hematopoietic stem cell transplantation (HSCT) require nutritional support (NS) because of their inability to consume adequate caloric intake enough calories orally. Although NS can be provided either enteraly (EN) or parenteraly (PN), EN is the preferred method of...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25473
更新日期:2015-08-01 00:00:00
abstract::Immunotherapy with the anti-GD2 monoclonal antibody ch14.18, or dinutuximab, represents an important therapeutic advance in the treatment of pediatric high-risk neuroblastoma and is now considered part of standard of care in this patient population. To date, transverse myelitis as a result of dinutuximab therapy has n...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26732
更新日期:2018-01-01 00:00:00
abstract:BACKGROUND:The potential role of VEGF in osteosarcoma has been evaluated in several studies. The majority of them included heterogeneous and limited series of patients, giving conflicting results. The aim of presented study is to evaluate the prognostic role of VEGF-A in biopsy samples of clinically homogeneous group o...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.23021
更新日期:2011-07-15 00:00:00
abstract:BACKGROUND:Malnutrition is a pro-inflammatory state, yet data on nutritional risk factors and development of acute graft-versus-host disease (aGVHD) are extremely limited. PROCEDURE:We conducted a retrospective cohort analysis of pediatric patients up to age 21 years who underwent allogeneic hematopoietic stem cell tr...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章
doi:10.1002/pbc.26853
更新日期:2018-02-01 00:00:00
abstract:BACKGROUND:Mutations in the DNMT3A, TET2, IDH1, and IDH2 genes carry prognostic significance and occur frequently in adult acute myeloid leukemia (AML). Leukemic mutations in all four genes have recently been implicated in aberrant DNA methylation, a hallmark of neoplasia. We previously reported that IDH1 mutations wer...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.23179
更新日期:2011-08-01 00:00:00
abstract:BACKGROUND:Insomnia is common among adolescent and young adult (AYA) cancer survivors. Cognitive-behavioral therapy for insomnia (CBT-I) is considered the gold standard treatment. Standard CBT-I was designed for adults and not adapted to the unique medical, psychosocial, and developmental needs of AYA cancer survivors,...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.28506
更新日期:2020-09-01 00:00:00