Evaluation of RB Gene and Cyclin-Dependent Kinase Inhibitors P21 and P27 in Pleomorphic Xantoastrocytoma

Pleomorphic xantoastrocytoma (PXA) is a rare, circumscribed astrocytic tumor that usually occurs in the superficial cerebral hemispheres in children and young adults. Most patients have a favorable prognosis, but recurrence and malignant transformation have been reported. In diffuse gliomas, approximately one third demonstrate mutations of the RB gene. Low expression level and high activity of p27 are known to constitute an independent prognostic factor in patients with malignant gliomas, while p21 expressions have variable labeling ranges. The molecular and genetic basis for tumorigenesis and progression of PXA are still largely unknown. In this study, 13 PXAs were examined immunohistochemically for pRb, p21, and p27 expression. Nine PXAs expressed homogeneous pRb positivity in the most nuclei of the tumor cells. Four cases showed an abnormal pRb staining pattern. All PXAs were positive for nuclear expression of p21. Diffuse nuclear positivity of p27 was seen in 10 cases, focal in 2, and in 1 case was not present. The cases with focal and negative p27 nuclear expression had few pRb-positive nuclei. The majority of PXAs appear to have preserved pRb, p21, and p27 functions. Additional studies are necessary to investigate whether cases with altered pRb and p27 expressions are associated with increased risk of recurrence or malignant transformation.