Osteofibrous dysplasia-like adamantinoma versus osteofibrous dysplasia in children: A case report of challenging diagnosis

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Abstract

Introduction: Osteofibrous dysplasia (OFD) and Osteofibrous dysplasia-like Adamantinoma have a similar appearance both in clinical and radiography, but different in its histopathology. Despite this similarity, the treatment and prognosis are different, therefore the diagnosis should be established precisely. Case illustration: A three-year-old boy was admitted to hospital after falling on his lower leg. A bead size lump appeared on his tibia with pain and swelling, which later became enlarged. Diagnosis of osteofibrous dysplasia and adamantinoma was considered. We performed limb-salvage procedure by curretage, bone grafting, and internal fixation application. The histology section showed woven bone rimmed by polygonal osteoblast cell with intervening fibrous stroma and small nests of tumour cells raised the possibility of epithelial differentiation. The positivity for cytokeratin immunostaining confirmed the diagnosis as osteofibrous dysplasia-like adamantinoma. In this case it is a very rare spectrum of malignancy in children. Discussion: These two tumor entities have identical radiographic characteristics, histopathology features the distinction between classic adamantinoma and OFD-like adamantinoma based on the predominant epithelial component. The relationship of osteofibrous dysplasia with adamantinoma is unclear. Several authors considered possible calling relationship osteofibrous dysplasia as “juvenile adamantinoma”. However, does not rule out the possible existence of de novo osteofibrous dysplasia not related to adamantinoma. Conclusions: OFD-like adamantinoma and Osteofibrous Dysplasia had similar histopathology pattern, a pathologist must be aware of this feature and perform immunohistochemical staining for keratin particularly when the histopathological feature of osteofibrous dysplasia showed small nests of tumor cells within the fibrous stroma. diagnostic challenging and require multidisciplinary approach.

Original languageEnglish
Article number105599
JournalInternational Journal of Surgery Case Reports
Volume80
DOIs
Publication statusPublished - Mar 2021

Keywords

  • Immunohistochemical staining
  • Osteofibrous dysplasia
  • Osteofibrous dysplasia-like adamantinoma

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