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Establishment of a Primary Culture of Patient-derived Soft Tissue Sarcoma
Published on: April 11, 2018
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Soft Tissue and Visceral Organ Sarcomas With BCOR Alterations
Sarah B Whittle1,2, Stephanie Fetzko1,2, Angshumoy Roy3
1Departments of Pediatrics.
Journal of Pediatric Hematology/Oncology
|May 10, 2022
Summary
BCOR-altered sarcomas, including BCOR-ITD, BCOR::CCNB3, and ZC3H7B::BCOR, present in diverse age groups and locations. BCOR::CCNB3 fusions show a better prognosis than BCOR-ITD and ZC3H7B::BCOR.
Area of Science:
- Oncology
- Genetics
- Pathology
Background:
- BCOR alterations define a heterogeneous group of sarcomas.
- These alterations include internal tandem duplications (ITDs) and fusions with CCNB3, ZC3H7B, and other genes.
- Genomic testing increasingly identifies BCOR alterations in Ewing-like sarcoma, endometrial stromal sarcoma, and clear cell sarcoma of kidney.
Purpose of the Study:
- To review and analyze the characteristics and outcomes of BCOR-altered soft tissue and organ sarcomas.
- To identify demographic, clinical, and prognostic differences between specific BCOR alteration subtypes.
Main Methods:
- A systematic literature search was conducted on PubMed for "Sarcoma (AND) BCOR" from 2005 to October 2021.
- Data from 41 publications describing 190 patients were analyzed.
- Summary statistics and outcome data were calculated using STATA v12.1.
Main Results:
- BCOR-ITD was the most frequent alteration, followed by BCOR::CCNB3 and ZC3H7B::BCOR.
- Age distribution varied: BCOR-ITD in infants, BCOR::CCNB3 in adolescent young adults, and ZC3H7B::BCOR in adults.
- Tumor sites differed: extremity for BCOR::CCNB3, kidney for BCOR-ITD, and uterus for ZC3H7B::BCOR.
- Metastasis was rare in BCOR::CCNB3.
- Five-year overall survival was 68% for BCOR::CCNB3, 35% for BCOR-ITD, and 41% for ZC3H7B::BCOR.
Conclusions:
- BCOR-altered sarcomas exhibit distinct demographic and clinical features based on the specific alteration.
- Prognoses vary significantly, with BCOR::CCNB3 fusions associated with better survival compared to BCOR-ITD and ZC3H7B::BCOR.
- Collaborative efforts are needed to develop optimal treatment strategies for these rare tumors.
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