Childhood Cancer Genomics (PDQ®)–Health Professional Version

SECTIONS

Changes to this Summary (05/02/2018)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Central Nervous System Tumors

The Central Nervous System (CNS) Atypical Teratoid/Rhabdoid Tumors (AT/RT) subsection was comprehensively reviewed.

Added text to the Central Nervous System (CNS) Atypical Teratoid/Rhabdoid Tumors (AT/RT) subsection to state that loss of SMARCB1/SMARCA4 staining is a defining marker for AT/RT. Also added text to state that less commonly, SMARCA4-negative (with retained SMARCB1) tumors have been described (cited Hasselblatt et al. as reference 51).

Hepatoblastoma and Hepatocellular Carcinoma

This section was comprehensively reviewed.

Added text to the Hepatoblastoma and Hepatocellular Carcinomasection to state that to date, the genetic mutations discussed in this section have not been used to select therapeutic agents for investigation in clinical trials.

Kidney Tumors

Added text to the Wilms Tumor subsection about the genetic aberrations seen in Wilms tumor (cited Gadd et al. as reference 1).

Revised text in the Wilms Tumor subsection to state that other genes critical for early renal development that are recurrently mutated in Wilms tumor include SIX1, SIX2, EP300, CREBBP, and MYCN. Also added text to state that of the mutations in Wilms tumors, 30% to 50% appear to converge on the process of transcriptional elongation in renal development and include the genes MLLT1, BCOR, MAP3K4, BRD7, and HDAC4.

Revised text in the Wilms Tumor subsection to state that CTNNB1 is the most commonly mutated gene in Wilms tumor, reported to occur in 15% of patients with Wilms tumor.

Revised text in the Wilms Tumor subsection to state that mutations in selected miRNA processing genes are observed in approximately 20% of Wilms tumor cases.

This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® - NCI's Comprehensive Cancer Database pages.

< Previous section
Multiple Endocrine Neoplasia Syndromes

Next section >
About This PDQ Summary