engleski

A case of psammous desmo-osteoblastoma of the skull

Introduction. Fibro-cemental lesions of the jaw and skull represent a very heterogeneous group of diseases. The variety of the clinical pictures and histological features sometimes leads to confusion. The separation of these lesions into distinct pathological entities is justifiable on their differences in biological behaviour. Psammous desmo-osteoblastoma (PDOB) is one of four newly described entities by Makek. PDOB presents as progressively enlarging, usually painless mass of the bone. Clinical details. A 16-year old male patient presented with an incidentally detected painless and swelling lesion of the skull situated in the right parieto-occipital region. CT scan revealed a hypodense osteolytic lesion of 2,5-cm diameter with thinning of the outer and inner tables. The radiolucent area had a uniform appearance with focal osteosclerosis. There was no change in the subjacent dura. According to radiographic features suspected osteoid osteoma was diagnosed. Surgical therapy consisted of resection of the lytic area. Moreover, cranioplasty was performed. Pathologic features. The bone sample was formalin-fixed and decalcified. Paraffin-embedded material showed a tumor with fasciculi and whorl of varying cell density composed of a poorly vascularized fibrous tissue. In these fasciculi basophilic calcified structures were found, frequently spheroid-like, the so-called psammoma bodies. Most of them were concentically surrounded by spindle cells. Furthermore, foci of osteoid and woven bone trabeculae were also found. Examination of the pheripheral areas of the tumor showed no clear evidence of encapsulation, but rather, the new bone formation appeared to be simply reactive. Conclusion. PDOB is a lesion described on the basis of light microscopical and ultrastructural features with evidence of an osteogenic histogenesis. It is extremely rare tumor and represents a distinct entity occurying in desmal preformed cranio-facial bones, which should be incorporated in a revised WHO-classification.

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