Polyostotic form of metaphyseal fibrous bone defect in children

Abstract

Fourteen patients, 8—16 years, with polyossal form of metaphyseal fibrous bone defect (MFBD) were treated. The most rear variant of polyossal form of MFBD, i.e. Jaffe-Campanacci syndrome, was described for the first time in native literature. Two variants of pathologic focus localization, i.e. subperiosteal and intracortical, were detected by radiologic examination. Histologic pictures of mono- and polyossal forms of MFBD were identical. However, in Jaffe- Campanacci syndrome marked polymorphism of one-nucleiform cellular elements was observed. Differentiation-diagnostic signs of polyossal form of MFBD were defined. In most cases the conservative treatment was used, 3 patients were treated surgically.

Keywords

metaphyseal fibrous bone defect / tumor-like diseases of the skeleton / skin pigmentation

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