A case of cherubism with bilateral central giant cell lesions of the mandible

  • KANDA Taku
    Department of Oral and Maxillofacial Surgery, Hiroshima University Hospital
  • KIMURA Naohiro
    Department of Oral and Maxillofacial Surgery, Hiroshima University Hospital
  • ANDO Toshinori
    Department of Oral and Maxillofacial Pathobiology, Division of Basic Life Sciences, Institute of Biomedical & Health Sciences, Hiroshima University
  • TORATANI Shigeaki
    Department of Molecular Oral Medicine and Maxillofacial Surgery, Division of Applied Life Sciences, Institute of Biomedical & Health Sciences, Hiroshima University
  • HARA Jun-ichi
    Department of Dentistry and Oral Surgery, JA Onomichi General Hospital
  • OKAMOTO Tetsuji
    Department of Oral and Maxillofacial Surgery, Hiroshima University Hospital Department of Molecular Oral Medicine and Maxillofacial Surgery, Division of Applied Life Sciences, Institute of Biomedical & Health Sciences, Hiroshima University

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Other Title
  • ケルビズムと診断された両側下顎骨中心性巨細胞病変の1例
  • ケルビズム ト シンダン サレタ リョウガワ カガクコツ チュウシンセイキョサイボウ ビョウヘン ノ 1レイ

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Abstract

Cherubism is a rare inherited disease characterized by a cherubic facial appearance. Generally, symmetric jaw swellings and indolent clinical course are early symptoms in early childhood. A few sporadic cases have been decribed1,2). We report the case of a central giant cell lesion diagnosed to be cherubism. The patient was 7-year-old girl who consulted our clinic because of bilateral painless swelling of the jaws. Computed tomographic examination showed well-delineated multilocular radiolucent lesions associated with bilateral enlargement of the jaws. Her father had a clinical history of bilateral giant cell reparative granuloma arising in the mandible. Because histopathological examination of a biopsy specimen revealed central giant cell granuloma, enucleation of the lesions was performed with the patient under general anesthesia. Histopathological examination of the enucleated specimen revealed an accumulation of giant cells resembling osteocyte and hemosiderin deposits in granulation tissue with spindle cells. There has been no evidence of recurrence as of 3 years after surgery.

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