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BCOR-CCNB3 sarcoma arising in the proximal tibia: A case report
Kayo Suzuki1, Taketoshi Yasuda1, Yuki Haruhara1
1Department of Orthopedic Surgery, Faculty of Medicine, University of Toyama, Toyama 930-0194, Japan.
Molecular and Clinical Oncology
|March 7, 2022
Summary
BCOR-CCNB3 fusion sarcoma is a rare bone tumor. This case study details a 12-year-old boy with proximal tibia sarcoma, treated successfully with surgery and chemotherapy.
Area of Science:
- Orthopedic Oncology
- Pediatric Oncology
- Molecular Pathology
Background:
- BCOR-CCNB3 fusion sarcoma, identified in 2020, is a rare subgroup of undifferentiated small round cell sarcoma.
- This entity constitutes 1.5-14% of undifferentiated unclassified sarcomas and is a rare primary malignant bone tumor.
Observation:
- A 12-year-old boy presented with a 6-month history of knee pain and a growing mass in the proximal tibia.
- Radiography and CT revealed a lytic lesion with cortical destruction; MRI showed soft tissue expansion.
- Histopathology indicated a proliferation of small mesenchymal cells, leading to a diagnosis of BCOR-CCNB3 sarcoma via immunohistochemistry and molecular analysis.
Findings:
- The study presents a case of BCOR-CCNB3 fusion sarcoma in the proximal tibia of a pediatric patient.
- The patient underwent surgical resection and chemotherapy following a Ewing sarcoma protocol.
- No local recurrence or distant metastasis was observed at 12 months post-treatment.
Implications:
- This case adds to the literature on BCOR-CCNB3 sarcoma of bone, particularly in the proximal tibia.
- Successful treatment using a Ewing sarcoma protocol suggests potential therapeutic strategies for this rare sarcoma.
- Further research into BCOR-CCNB3 sarcoma is warranted to improve understanding and treatment outcomes.
