BCOR (BCL6 Corepressor)

Our findings provide a comprehensive genomic landscape of Korean gastric cancer and underscore the clinical relevance of integrating genomic and established biomarkers to advance precision oncology.

We suggest that HGESS with BCOR alterations may warrant reclassification as 'BCOR-altered uterine sarcomas'.

P2/3, N=437, Active, not recruiting, Children's Oncology Group | Trial completion date: Jul 2032 --> Dec 2027 | Trial primary completion date: Jul 2032 --> Dec 2027

These findings led to the diagnosis of primary renal adenosarcoma. This case highlights the diagnostic challenge of distinguishing this rare tumor from more common renal malignancies and underscores the importance of imaging-pathologic correlation.

The allelic ratio of FLT3-ITD did not further stratify OS and RFS in this subgroup. These findings suggest that the prognostic relevance of MRG mutations in FLT3-ITD AML is modulated by NPM1 co-mutational status and mirror findings in AML lacking FLT3-ITD.

After failure of gemcitabine/docetaxel chemotherapy, next-generation sequencing identified an IGFBP5-ALK fusion (breakpoint: IGFBP5 exon 1 - ALK exon 19), a TERT promoter mutation, and a homozygous CDKN2A/CDKN2B/MTAP deletion. This case highlights the first documented response to an ALK inhibitor in ALK-rearranged HG-ESS. The findings underscore the importance of comprehensive molecular profiling in identifying targetable alterations in rare sarcomas and support the use of iruplinalkib as an effective therapeutic option in this setting.

Treatment included surgery and systemic therapies (nivolumab and ipilimumab, followed later by tovorafenib). The patient's clinical course was marked by aggressive local progression and therapeutic challenges. This case highlights the rarity of such presentations and the need for further research into the clinicopathological and molecular features of infantile melanoma arising in congenital melanocytic nevus (CMN).

Definitive diagnosis relies on molecular testing (such as FISH or NGS), which is crucial for differential diagnosis and prognostic evaluation. This subtype of CCSK is commonly associated with advanced clinical stage and early metastasis/recurrence, highlighting the necessity for improving risk stratification and clinical management.

Despite the relatively favorable outcomes in BCOR sarcomas, the relapse rate is considerable, whereas pediatric patients with CIC sarcoma typically develop metastatic disease and have poor outcomes. The data provide a prospective foundation for genetically based therapeutic strategies and risk stratification.

One series was advanced to a low micromolar confirmed hit via iterative rounds of compound optimization guided by structure activity relationships and co-crystal structures. Overall, we were able to use a rational, structure-based drug design approach to identify and advance a novel series of pyrrolopyrimidinone BCL6 BTB inhibitors.

Key messages Round cell sarcomas share many morphological similarities, so the differential diagnosis can be broad; however, subtle differences between tumours can aid the final diagnosis. Nevertheless, most round cell sarcomas require molecular studies for accurate classification, and cytological specimens are usually high quality material for such ancillary techniques.

Serial assessments were performed before and during treatment with bortezomib and dexamethasone for MM, and later with daunorubicin/cytarabine for AML. It demonstrates the earliest events in pDC-AML evolution. Furthermore, the immature immunophenotype raises the question of appropriate treatment, since a diagnosis of acute undifferentiated leukemia can be established.