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BCOR-CCNB3 sarcoma arising in the pharynx.

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|May 11, 2022
PubMed
Summary

BCOR-CCNB3 sarcoma in the head and neck is rare. This case shows successful treatment with chemotherapy, conservative surgery, and radiation, leading to disease-free survival.

Keywords:
BCOR-CCNB3 sarcomaChemotherapy-sensitive tumorEwing sarcomaFluorescence in-situ hybridizationVDC-IE therapy

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Area of Science:

  • Oncology
  • Pathology
  • Genetics

Background:

  • BCOR-CCNB3 sarcoma is a rare malignancy with limited reported cases in the head and neck region.
  • The etiology of BCOR-CCNB3 sarcoma remains largely unknown, presenting diagnostic and therapeutic challenges.

Observation:

  • An 18-year-old male presented with a rapidly growing tumor spanning the nasopharynx to the oropharynx.
  • Histological examination revealed a spindle cell sarcoma, with BCOR-CCNB3 fusion confirmed via fluorescence in situ hybridization.

Findings:

  • The patient received three cycles of VDC-IE chemotherapy followed by tumor resection using the mandibular swing technique with a minimal margin.
  • Postoperative radiation therapy (50.4 Gy) and three additional VDC-IE cycles were administered.
  • The patient achieved disease-free survival at 12 months postoperatively.

Implications:

  • BCOR-CCNB3 sarcoma demonstrates sensitivity to chemotherapy, suggesting its crucial role in treatment protocols.
  • Conservative surgical resection with minimal margins, when feasible without compromising treatment, is a preferred approach.
  • This case highlights a successful multimodal treatment strategy for a rare head and neck sarcoma.