1. BCOR-CCNB3 sarcoma arising in the pharynx.
- Author
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Eguchi K, Omura G, Shimoi T, Kageyama D, Igaki H, Abe Y, Watanabe T, Aihara Y, Sakai A, Matsumoto Y, Sakai T, Yonemori K, Mori T, Yoshida A, and Yoshimoto S
- Subjects
- Male, Humans, Adolescent, In Situ Hybridization, Fluorescence, Proto-Oncogene Proteins genetics, Repressor Proteins genetics, Biomarkers, Tumor, Cyclin B, Pharynx pathology, Sarcoma genetics, Sarcoma surgery
- Abstract
Reports on BCOR-CCNB3 sarcoma in the head and neck region are scarce, given their unknown etiology. An 18-year-old male patient presented a rapidly enlarging tumor extending from the right nasopharynx to the oropharynx. Histological examination showed a spindle cell sarcoma with BCOR-CCNB3 fusion detected by fluorescence in situ hybridization, and BCOR-CCNB3 was diagnosed. After three courses of alternating VDC-IE therapy, the patient underwent tumor resection based on the original tumor range with a minimal margin, using the mandibular swing technique. Radiation therapy (50.4 Gy) was administered postoperatively, followed by three additional courses of alternating VDC-IE therapy. The patient survived and showed no evidence of disease at 12 months postoperatively. BCOR-CCNB3 sarcoma is a chemotherapy-sensitive sarcoma, and conservative resection with a minimal margin that does not interfere with the treatment flow is preferable., Competing Interests: Conflicts of Interest The authors have no conflicts of interest to disclose., (Copyright © 2022. Published by Elsevier B.V.)
- Published
- 2023
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