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Introduction <p>Epithelioid osteoblastoma is a rare variant of osteoblastoma, characterized by large osteoblasts with epithelioid morphology. Although benign, it can be locally aggressive, frequently affecting the spine and long bones, with up to 26% of cases in the craniofacial region, especially the mandible.</p> Case Presentation <p>We present a case of a 12-year-old male with a mandibular epithelioid osteoblastoma causing cortical expansion and tooth displacement. CT revealed a 4.7&#xa0;cm mass with calcifications and periosteal reaction. Histology showed epithelioid osteoblast proliferation within a highly vascular stroma, scattered osteoclasts, and hemorrhagic areas, with a low Ki-67 index (&lt; 5%) and no atypical mitoses or infiltrative growth.</p> Differential Diagnosis <p>Osteoid osteoma, cementoblastoma, fibro-osseous lesions, and osteosarcoma were considered. Osteosarcoma was excluded due to the absence of nuclear pleomorphism, atypical mitoses, and infiltrative growth. Clinical, radiographic, and histologic features, along with FOS/FOSB rearrangements and c-fos immunohistochemistry, aided accurate diagnosis.</p> Management and Outcome <p>The patient underwent marginal resection with clear margins. Follow-up for 10&#xa0;months showed no recurrence.</p> Conclusion <p>Epithelioid osteoblastoma reflects a histologic phenotype rather than clinical aggressiveness. Complete excision is recommended to prevent recurrence, while preoperative biopsy and careful differential diagnosis are crucial for distinguishing it from mimics, particularly osteosarcoma.</p>

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Epithelioid Osteoblastoma: Clinicopathologic Features of a Classic Case with Aggressive Behavior

  • 期刊论文

  • 发表日期：

    2025年9月20日

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  • Jamerson Carvalho Silva,
  • Dandara Andrade de Santana,
  • Isabela Teixeira Fernandes,
  • Alessandra Monteiro Santana,
  • Walter Suruagy Motta Padilha,
  • Flávia Caló de Aquino Xavier,
  • Jean Nunes dos Santos

摘要

Introduction

Epithelioid osteoblastoma is a rare variant of osteoblastoma, characterized by large osteoblasts with epithelioid morphology. Although benign, it can be locally aggressive, frequently affecting the spine and long bones, with up to 26% of cases in the craniofacial region, especially the mandible.

Case Presentation

We present a case of a 12-year-old male with a mandibular epithelioid osteoblastoma causing cortical expansion and tooth displacement. CT revealed a 4.7 cm mass with calcifications and periosteal reaction. Histology showed epithelioid osteoblast proliferation within a highly vascular stroma, scattered osteoclasts, and hemorrhagic areas, with a low Ki-67 index (< 5%) and no atypical mitoses or infiltrative growth.

Differential Diagnosis

Osteoid osteoma, cementoblastoma, fibro-osseous lesions, and osteosarcoma were considered. Osteosarcoma was excluded due to the absence of nuclear pleomorphism, atypical mitoses, and infiltrative growth. Clinical, radiographic, and histologic features, along with FOS/FOSB rearrangements and c-fos immunohistochemistry, aided accurate diagnosis.

Management and Outcome

The patient underwent marginal resection with clear margins. Follow-up for 10 months showed no recurrence.

Conclusion

Epithelioid osteoblastoma reflects a histologic phenotype rather than clinical aggressiveness. Complete excision is recommended to prevent recurrence, while preoperative biopsy and careful differential diagnosis are crucial for distinguishing it from mimics, particularly osteosarcoma.