Abnormal cilia in a fourth ventricular ependymoma

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Abstract
Although the luminal surface of the ependymal cells of the human ventricular system is characterized by the presence of cilia with a typical 9+2 arrangement of microtubules, cilia are usually described as sparse or absent in ependymal cells of ependymomas. The incidence of aberrant cilia and structurally abnormal cilia in normal ependymal cells and ependymomal cells has not been documented. This report describes detailed ultrastructural and quantitative studies of cilia of the ependymal cells of a fourth ventricular ependymoma in a 5-year-old boy. Of a total of 267 cilia studied, 50% were located in the extracellular microrosettes, 30% in the intracytoplasmic lumina and 20% in the cytoplasm. Thirty percent of all cilia studied showed structural abnormalities. Those present in the aberrant locations, e.g., intracytoplasmic lumina and in the cytoplasm, had a higher incidence of abnormality. Ciliary abnormalities fell into four majors categories: (1) cilia with abnormal axial microtubules, (2) compound cilia, (3) swollen cilia, and (4) cilia with dynein arm defect. Of these, addition, deletion and disorganization of axonemal microtubules were most common. The present study suggested that ciliary abnormality is common in ependymoma and may represent another example of oncogenetic effect on the ciliogenesis of ciliated epithelium.