Abstract. The World Health Organization classification of choroid plexus tumors (CPT) includes three distinct grades:Choroid plexus papilloma (CPP), atypical choroid plexus papilloma (ACPP) and choroid plexus carcinoma (CPC). The molecular basis for these pathological distinctions may help to stratify tumors and provide an insight into the clinical behavior of CPTs. In the present study, the progenitor and stem cell markers neuron glia antigen-2 (NG2) and sex-determining region Y-box 2 (SOX2) were investigated as potential biomarkers that may distinguish between distinct CPT grades. Immunohistochemistry was used to determine the expression of NG2 and SOX2 in CPTs (n=34) from Chinese patients (21 males and 13 females) with a mean age of 31.1 years (range, 1-63 years). The proportion of cells stained were scored using a scale between 0 and 3+, where 0 represents no staining and 3+ represents strong staining, and mean scores for each marker were determined on the basis of tumor grade. Pathological diagnosis revealed a distribution of cases as follows: CPP, 25; ACPP, 5; and CPC, 4. NG2 and SOX2 were expressed in CPTs of all grades. The mean labeling indices for CPP, ACPP and CPC were 1.12, 1.80 and 2.75 for NG2, respectively, and 1.20, 2.00 and 3.00 for SOX2, respectively. Statistical analysis of the mean labeling indices revealed a significant association between the expression of NG2 and SOX2 and CPT grade (P= 0.001 and <0.001 for CPP/ACPP and CPP/CPC, respectively). The results of the present study indicated that increased expression of NG2 and SOX2 was associated with higher-grade tumors and that these markers may be useful in determining CPT grade.
IntroductionChoroid plexus tumors (CPTs) are rare intracranial neoplasms that derive from the choroid plexus epithelium of the ventricles (1,2). The lesions are commonly located in the lateral and the fourth ventricles, and rarely in the cerebellopontine angle and the third ventricle (3,4). The majority of CPTs arise with clinical symptoms that are associated with hydrocephalus as a result of direct mechanical obstruction of the flow of cerebrospinal fluid (CSF) and arachnoid granulation blockage from hemorrhage and overproduction of CSF (1,5).Treatment of CPTs is currently based on histological diagnosis. According to the World Health Organization (WHO) classification scheme, CPTs are diagnosed as choroid plexus papilloma (CPP, WHO grade I), atypical choroid plexus papilloma (ACPP, WHO grade II) and choroid plexus carcinoma (CPC, WHO grade III) (3). For all classifications, the general treatment strategy is surgical resection unless this is not possible due to tumor location, for example. Gross total resection (GTR) is the indicated course of treatment for CPP as well as for ACPP. CPP is a benign neoplasm and recurrence following GTR is rare, therefore adjuvant radiotherapy and chemotherapy are not generally recommended (2,3,6,7). ACPP cases exhibit atypical histological features along with increased mitotic activity; however, patients with this type of tumor also e...