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Comparative Lesions Analysis Through a Targeted Sequencing Approach
Published on: November 5, 2019
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[Clinicopathologic analysis of Ewing-like BCOR-CCNB3 undifferentiated sarcoma]
1Department of Pathology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China; #Department of Pathology, Xijing Hospital, Fourth Military Medical University, Xi'an 710005, China.
Zhonghua Bing Li Xue Za Zhi = Chinese Journal of Pathology
|February 9, 2017
Summary
This study identifies Ewing-like BCOR-CCNB3 gene fusion undifferentiated sarcoma, a rare tumor distinct from classic Ewing sarcoma. Early identification is crucial for appropriate patient management and prognosis.
Area of Science:
- Oncology
- Molecular Pathology
- Genetics
Background:
- Ewing-like sarcomas encompass a heterogeneous group of tumors.
- Distinguishing BCOR-CCNB3 fusion sarcoma from classical Ewing sarcoma is critical for accurate diagnosis and treatment.
Observation:
- This study identified three cases of BCOR-CCNB3 fusion sarcoma among 260 Ewing-like sarcomas.
- Patients were young, aged 8-11 years.
- Tumor cells exhibited diffuse nuclear CCNB3, TLE1, and cyclin D1 positivity, contrasting with infrequent positivity in classical Ewing's sarcoma.
Findings:
- BCOR-CCNB3 fusion transcripts were confirmed via RT-PCR and sequencing.
- Histologically, tumors showed oval or plump spindle cells with fine chromatin and inconspicuous nucleoli.
- Prognosis varied, with one patient responding to chemotherapy and two succumbing to the disease within 12 months post-surgery.
Implications:
- The findings support the distinction of BCOR-CCNB3 sarcoma from Ewing sarcoma based on histological, immunohistochemical, and genetic profiles.
- Accurate diagnosis can guide targeted therapeutic strategies.
- Further research into the specific molecular mechanisms and optimal treatment protocols for BCOR-CCNB3 sarcoma is warranted.
