Suprasellar monomorphous pilomyxoid neoplasm: an ultastructural analysis.

摘要

The authors report 3 patients, 2 children and 1 adult, each of whom presented with an unusual myxoid lesion reminiscent of pilocytic astrocytoma but with other features of myxopapillary ependymoma. The magnetic resonance imaging findings in all cases showed a diffusely contrast-enhancing suprasellar mass focally extending into the third ventricle. Involvement of adjacent structures was more extensive in both infants. By light microscopy, all were composed of a monotonous population of cells with delicate piloid-like processes, loosely arranged within a prominent myxoid background. Focally, the neoplastic cells converged upon small blood vessels in pseudorosette-like formations. These histomorphologic features are identical to those of the recently described astrocytoma with monomorphous pilomyxoid features. In addition, the individual tumor cells showed strong cytoplasmic immunoreactivity with antibodies to glial fibrillary acidic protein (GFAP) and vimentin, as well as nuclear and cytoplasmic staining with S-100. All stained positive for synaptophysin and negative for chromogranin. By electron microscopy, the tumor cells were bipolar with elongated processes and apical surfaces displaying microvilli, cytoplasmic blebs and rare cilia. Vesicles and coated pits were seen, as were occasional synaptoid complexes. The current study serves to expand our clincopathologic experience with this rare and enigmatic lesion, with particular attention given to the ultrastructural characteristics.