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title: Overview
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## v0.4
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Submitted: September 3, 2025
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Click [here](/files/cc4k/Official_CC4K_v0.4.xlsx){target="_blank"} to download.
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With this release, we are pleased to announce our official name: Cancer Classification for Kids (CC4K).
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This name replaces the provisional title "St. Jude Cloud Disease Ontology (SJC-DO)"" and reflects our mission to provide a unified, community-driven framework for pediatric cancer classification.
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Cancer Classification for Kids (CC4K) version 0.4 introduces a significant restructuring of lymphoblastic leukemia and lymphoma terms to better reflect evolving diagnostic practice and align with current community standards, including [WHO Classification of Hematolymphoid Tumors, 5th edition (WHO5)](https://tumourclassification.iarc.who.int/welcome/), OncoTree <sup>1</sup> , and St. Jude internal classification efforts.
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This update focuses on unifying terms for B- and T-lineage leukemias and lymphomas, improving naming consistency, and structural organization.
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Additional research-relevant classifications were also introduced based on recent findings from Brady et al. <sup>2</sup>
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### Comprehensive Updates
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We combined previously separate leukemia and lymphoma terms for B- and T-lineage neoplasms to align with current diagnostic standards and simplify classification.
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Following WHO5, OncoTree, and current literature, subtypes now use a unified "Leukemia/Lymphoma" label to reflect their shared biology and overlapping presentation.
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For example, cases formerly labeled separately as B-acute Lymphoblastic Leukemia, BCR-ABL1 and B-lymphoblastic Lymphoma, BCR-ABL1 are now merged into a single entity: B-acute Lymphoblastic Leukemia/Lymphoma with BCR::ABL1 Fusion.
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This change streamlines the hierarchy and supports more accurate representation of genomic subtypes across both phenotypes, recognizing that these are biologically the same disease that may present with predominant bone marrow and blood involvement (leukemia) or with primary disease in lymphoid tissues (lymphoma).
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We also introduced a new node, B-acute Lymphoblastic Leukemia/Lymphoma with Near Haploidy (BALLNH), to reflect improved recognition of this genetically and biologically distinct subtype.
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While these cases may resemble B-ALL with hyperdiploidy (BALLHYPER) in gene expression, especially when masked by genome doubling, they are defined by markedly low chromosome counts (typically 24-30 chromosomes), and mutations in RAS signaling pathways are recurrently seen in this category. <sup>3,4</sup>
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These features distinguish them from both hypodiploid and hyperdiploid subtypes.
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Based on criteria from Brady et al. <sup>2</sup>, 45 samples previously labeled as B-ALL with hypodiploidy (BALLHYPO) were reclassified into this more precise BALLNH category.
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### Challenges
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One area of uncertainty was the subtyping of T-acute Lymphoblastic Leukemia/Lymphoma (T-ALL), where molecular subclassification is still an active area of research.
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Although some subtypes have been described in the literature, they are not treated as distinct entities in WHO5 and are instead grouped under T-ALL, NOS.
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For this release, we chose to retain our existing subclassification, as it aligns with published studies and ongoing efforts to refine the T-ALL classification.
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We anticipate further updates as relevant studies are published.
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### Future Updates
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While this release completes the major planned restructuring of hematologic malignancies, additional refinements particularly for T-ALL or in response to new literature or ontology updates, may still occur.
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Our development efforts now shifts to Solid Tumors, where we are conducting a systematic review and restructuring to ensure consistency with WHO5 classifications.
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### References
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1. Kundra, R. et al. OncoTree: A Cancer Classification System for Precision Oncology. JCO Clin Cancer Inform 221–230 (2021). [https://doi.org/10.1200/CCI.20.00108](https://doi.org/10.1200/CCI.20.00108).
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2. Brady, S. W. et al. The genomic landscape of pediatric acute lymphoblastic leukemia. Nat Genet 54, 1376–1389 (2022). [https://doi.org/10.1038/s41588-022-01159-z](https://doi.org/10.1038/s41588-022-01159-z).
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3. Molina, O. et al. Near-Haploidy and Low-Hypodiploidy in B-Cell Acute Lymphoblastic Leukemia: When Less Is Too Much. Cancers 2022, Vol. 14, Page 32 14, 32 (2021). [https://doi.org/10.3390/CANCERS14010032](https://doi.org/10.3390/CANCERS14010032).
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4. Carroll, A. J. et al. Masked hypodiploidy: Hypodiploid acute lymphoblastic leukemia (ALL) mimicking hyperdiploid ALL in children: A report from the Children’s Oncology Group. Cancer Genet 238, 62–68 (2019). [https://doi.org/10.1016/J.CANCERGEN.2019.07.009](https://doi.org/10.1016/J.CANCERGEN.2019.07.009).
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