Use of Multicolor Spectral Karyotyping in Genetic Analysis of Pleuropulmonary Blastoma

2000 ◽  
Vol 3 (5) ◽  
pp. 479-486 ◽  
Author(s):  
Maja Barnard ◽  
Jane Bayani ◽  
Ronald Grant ◽  
Ikuko Teshima ◽  
Paul Thorner ◽  
...  

Pleuropulmonary blastoma (PPB) is a rare, malignant intrathoracic pediatric tumor. It arises from the lung, pleura, or mediastinum and its pathogenesis and relationship to other pediatric solid tumors is not well understood. In this study, a case of PPB in a 3-year-old girl was studied using a combination of molecular genetic methods and cytogenetics. Molecular analysis of the commonly encountered fusion translocation gene products of pediatric solid tumors failed to detect a rearrangement. Cytogenetic analysis, supplemented by multicolor spectral karyotyping (SKY), identified an unbalanced translocation between chromosomes 1 and X, resulting in additional copies of 1q, an extra copy of Xq, and loss of part of Xp. In addition, trisomy 8 was detected. The identification of new chromosomal alterations and confirmation of previously reported ones in this rare neoplasm helps to improve our understanding of its pathogenesis and association with other pediatric tumors.

2019 ◽  
pp. 1-17 ◽  
Author(s):  
Jonathan M. Marron ◽  
Angel M. Cronin ◽  
Steven G. DuBois ◽  
Julia Glade-Bender ◽  
AeRang Kim ◽  
...  

Purpose Increasing use of genomic tumor profiling may blur the line between research and clinical care. We aimed to describe perspectives of research participants about the purpose of genomic tumor profiling research in pediatric oncology. Methods We surveyed 45 participants (response rate, 85%) in a pilot study of genomic profiling in pediatric solid tumors at four academic cancer centers after the return of sequencing results. We defined understanding according to a one-item (basic) definition (recognition that the primary purpose was not to improve the patient’s treatment) and a four-item (comprehensive) definition (primary purpose was not to improve patient’s treatment; primary purpose was to improve treatment of future patients; there may not be direct medical benefit; most likely result of participation was not increased likelihood of cure). Results Sixty-eight percent of respondents (30 of 44 respondents) demonstrated basic understanding of the study purpose; 55% (24 of 44 respondents) demonstrated comprehensive understanding. Understanding was more frequently seen in those with higher education and greater genetic knowledge according to basic (81% v 50% [ P = .05]; and 82% v 46% [ P = .03], respectively) and comprehensive (73% v 28% [ P = .01]; 71% v 23% [ P = .01]) definitions. Ninety-three percent of respondents who believed the primary purpose was to improve the patient’s care simultaneously stated that the research also aimed to benefit future patients. Conclusion Most participants in pediatric tumor profiling research understand that the primary goal of this research is to improve care for future patients, but many express dual goals when they participate in sequencing research. Some populations demonstrate increased rates of misunderstanding. Nuanced participant views suggest that additional work is needed to assess and improve participant understanding, particularly as tumor sequencing moves beyond research and into clinical practice.


1984 ◽  
Vol 2 (8) ◽  
pp. 917-923 ◽  
Author(s):  
S Kramer ◽  
A T Meadows ◽  
G Pastore ◽  
P Jarrett ◽  
D Bruce

This study examines differences between cancer centers (CC) and noncancer centers (NCC) in terms of management procedures and outcomes for three pediatric solid tumors: Wilms' tumor (N = 147), rhabdomyosarcoma (N = 87), and medulloblastoma (N = 76). Data were derived for the period 1970-1979 from the population-based Greater Delaware Valley Pediatric Tumor Registry maintained at the Children's Cancer Research Center, which routinely collects data on all childhood neoplasms that occur in a 31-county region. Management measures reviewed included the degree to which important pretreatment evaluations were performed, types of therapy used, and extent of follow-up examinations conducted. Outcome variables were three-year disease-free survival and frequency of deaths related to complications of therapy. Differences in three-year disease-free survival between CC and NCC were noted for medulloblastoma (52% v 24%) and rhabdomyosarcoma (48% v 10%), but not for Wilms' tumor (79% v 68%). Among medulloblastoma patients, differences were detected in the frequency of pretreatment evaluations and in the therapy used. The principal management contrast found in rhabdomyosarcoma was that multiagent chemotherapy was used less often in NCC. Wilms' tumor patients were evaluated and treated similarly in the community versus the CC, except for some contrasts in the surgical approach and the frequency of follow-up for the detection of late complications.


Author(s):  
Brian H. Kushner ◽  
Michael P. LaQuaglia ◽  
Nai-Kong V. Cheung ◽  
Kim Kramer ◽  
Aimee C. Hamelin ◽  
...  

Author(s):  
Lin Xiao ◽  
Harrison Yeung ◽  
Michelle Haber ◽  
Murray D. Norris ◽  
Klaartje Somers

Cancers ◽  
2021 ◽  
Vol 13 (11) ◽  
pp. 2796
Author(s):  
Aicha E. Quamine ◽  
Mallery R. Olsen ◽  
Monica M. Cho ◽  
Christian M. Capitini

Treatment of metastatic pediatric solid tumors remain a significant challenge, particularly in relapsed and refractory settings. Standard treatment has included surgical resection, radiation, chemotherapy, and, in the case of neuroblastoma, immunotherapy. Despite such intensive therapy, cancer recurrence is common, and most tumors become refractory to prior therapy, leaving patients with few conventional treatment options. Natural killer (NK) cells are non-major histocompatibility complex (MHC)-restricted lymphocytes that boast several complex killing mechanisms but at an added advantage of not causing graft-versus-host disease, making use of allogeneic NK cells a potential therapeutic option. On top of their killing capacity, NK cells also produce several cytokines and growth factors that act as key regulators of the adaptive immune system, positioning themselves as ideal effector cells for stimulating heavily pretreated immune systems. Despite this promise, clinical efficacy of adoptive NK cell therapy to date has been inconsistent, prompting a detailed understanding of the biological pathways within NK cells that can be leveraged to develop “next generation” NK cell therapies. Here, we review advances in current approaches to optimizing the NK cell antitumor response including combination with other immunotherapies, cytokines, checkpoint inhibition, and engineering NK cells with chimeric antigen receptors (CARs) for the treatment of pediatric solid tumors.


Cancer ◽  
2005 ◽  
Vol 104 (3) ◽  
pp. 644-652 ◽  
Author(s):  
Kevin M. Garrett ◽  
Christine E. Fuller ◽  
Victor M. Santana ◽  
Stephen J. Shochat ◽  
Fredric A. Hoffer

2017 ◽  
Vol 59 (2) ◽  
pp. 244-250 ◽  
Author(s):  
Dana C. Baiu ◽  
Ian R. Marsh ◽  
Alexander E. Boruch ◽  
Ankita Shahi ◽  
Saswati Bhattacharya ◽  
...  

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