title: Juvenile intracortical adamantinoma of the tibia with predominant osteofibrous dysplasia-like features.
abstract: In view of the still disputed relationship between adult adamantinoma and osteofibrous dysplasia in children, a unique case of adamantinoma, indicating a direct relationship between the two lesions, is presented with a review of the literature. The patient was a six-year-old boy who complained of pain and swelling in the left lower leg. Roentgenographs showed a loculate osteolysis surrounded by sclerosis within the cortex of the tibial shaft that would be typical of osteofibrous dysplasia. Although an osteofibrous-dysplastic component predominated histologically, some small islands of epithelial cells were scattered throughout the lesion. Immunohistochemically, the tumor cells of these epithelial islands gave a constant positive reaction for cytokeratin as well as vimentin, while the stromal cells in the osteofibrous dysplasia-like lesion were positive for vimentin only. This type of lesion is recorded in the Bone Tumor Registry of Westphalia at a rate of 8.3% for osteofibrous dysplasia, and of 25% for adamantinoma. A review of the literature, yielding reports with remarkable uniformity on 14 cases beyond the present one, suggests the existence of a separate clinicopathologic entity to be called juvenile intracortical adamantinoma with predominant osteofibrous dysplasia-like features, and which might be a regressing form of adamantinoma specific in childhood.
authors.affiliation: Gerhard-Domagk-Institute for Pathology, University of Münster, FRG.
authors.forename: Y
authors.initials: Y
authors.lastname: Ueda
authors.forename: A
authors.initials: A
authors.lastname: Roessner
authors.forename: A
authors.initials: A
authors.lastname: Bosse
authors.forename: G
authors.initials: G
authors.lastname: Edel
authors.forename: W
authors.initials: W
authors.lastname: Böcker
authors.forename: P
authors.initials: P
authors.lastname: Wuisman
mesh_terms: D000564:Ameloblastoma / Q000737:chemistry / Q000150:complications / Q000473:pathology*; D001859:Bone Neoplasms / Q000737:chemistry / Q000150:complications / Q000473:pathology*; D002648:Child; D005357:Fibrous Dysplasia of Bone / Q000150:complications / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D007633:Keratins / Q000032:analysis; D008297:Male; D013977:Tibia / Q000737:chemistry / Q000473:pathology*; D014746:Vimentin / Q000032:analysis
chemical_list: D014746:Vimentin; D007633:Keratins