Journal
WORLD JOURNAL OF PEDIATRICS
Volume 14, Issue 6, Pages 585-593Publisher
ZHEJIANG UNIV SCH MEDICINE
DOI: 10.1007/s12519-018-0181-3
Keywords
Iraq; Low- and middle-income countries; Next-generation sequencing; Pediatric cancer; Wilms tumor
Categories
Funding
- Carolyn Perot Rathjen Chair in Pediatrics at Vanderbilt
- NIH/NCRR [G20 RR030956]
- NIH/NCI [5R21CA155946-02]
- Vanderbilt Ingram Cancer Center Support Grant [5P30 CA068485]
Ask authors/readers for more resources
BackgroundWilms tumor (WT) is the most common childhood kidney cancer worldwide, yet its incidence and clinical behavior vary according to race and access to adequate healthcare resources. To guide and streamline therapy in the war-torn and resource-constrained city of Baghdad, Iraq, we conducted a first-ever molecular analysis of 20 WT specimens to characterize the biological features of this lethal disease within this challenged population.MethodsNext-generation sequencing of ten target genes associated with WT development and treatment resistance (WT1, CTNNB1, WTX, IGF2, CITED1, SIX2, p53, N-MYC, CRABP2, and TOP2A) was completed. Immunohistochemistry was performed for 6 marker proteins of WT (WT1, CTNNB1, NCAM, CITED1, SIX2, and p53). Patient outcomes were compiled.ResultsMutations were detected in previously described WT hot spots (e.g., WT1 and CTNNB1) as well as novel loci that may be unique to the Iraqi population. Immunohistochemistry showed expression domains most typical of blastemal-predominant WT. Remarkably, despite the challenges facing families and care providers, only one child, with combined WT1 and CTNNB1 mutations, was confirmed dead from disease. Median clinical follow-up was 40.5months (range 6-78months).ConclusionsThese data suggest that WT biology within a population of Iraqi children manifests features both similar to and unique from disease variants in other regions of the world. These observations will help to risk stratify WT patients living in this difficult environment to more or less intensive therapies and to focus treatment on cell-specific targets.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available