Dataset Information

Clinical Features and Prognostic Risk Factors of Choroid Plexus Tumors in Children.

ABSTRACT:

Background

Decision-making concerning the treatment of choroid plexus tumor (CPT) in pediatric patients remains a topic of considerable debate. The aim of this work was to describe clinical features and prognostic risk factors of CPT in the pediatric population and to provide theoretical opinions regarding clinical decisions for CPT.

Methods

The data of 96 patients with CPT and younger than 14 years were retrospectively analyzed. Clinical characteristics such as pathological type of CPTs, rate and severity of hydrocephalus, treatment and outcome, and recurrence were investigated. For categorical variables, the Pearson's Chi-square test was performed. The Mann-Whitney U-test was used for comparisons between nonnormally distributed parameters. Log-rank test was used for progression-free survival (PFS).

Results

The study included 70 choroid plexus papilloma (CPP) cases, 17 atypical choroid plexus papilloma (aCPP) cases, and 9 choroid plexus carcinoma (CPC) cases. Compared with patients with CPP or aCPP, patients with CPC had a shorter disease course (median: CPP, 4 months; aCPP, 2 months; CPC, 1 month; H: 23.5, P < 0.001), higher rate of acute hydrocephalus (CPP, 27.1%; aCPP, 52.9%; CPC, 77.8%; ?² = 10.9, P < 0.05), and lower incidence of cure rate (CPP, 85.7%; aCPP, 70.5%; CPC, 33.3%; ?² = 13.5, P < 0.05). The severity of hydrocephalus with tumor in the lateral or third ventricle was significantly higher than that with tumors in the fourth ventricle (severe hydrocephalus: lateral ventricle, 51.7%; third ventricle, 47.0%; fourth ventricle, 11.1%; ?² = 26.0, P < 0.001). Patients with gross total surgical resection had no better PFS than those with partial resection because of the use of adjuvant therapy in the latter (?² = 4.0, P > 0.05). Patients with CPC experienced shorter time for recurrence than those with CPP or aCPP (?² = 40.1, P < 0.0001).

Conclusions

Our results indicated that CPP in the fourth ventricle could trigger serious clinical symptoms at an early stage, requiring early intervention. Adjuvant treatment might be necessary for patients with partially resected CPP, aCPP, and CPC to achieve a favorable outcome.

SUBMITTER: Zhou WJ

PROVIDER: S-EPMC6302644 | biostudies-literature | 2018 Dec

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

Clinical Features and Prognostic Risk Factors of Choroid Plexus Tumors in Children.

Zhou Wen-Jian-Long WJ Wang Xi X Peng Jia-Yi JY Ma Shun-Chang SC Zhang Dai-Nan DN Guan Xiu-Dong XD Diao Jin-Fu JF Niu Jian-Xing JX Li Chun-De CD Jia Wang W

Chinese medical journal 20181201 24

<h4>Background</h4>Decision-making concerning the treatment of choroid plexus tumor (CPT) in pediatric patients remains a topic of considerable debate. The aim of this work was to describe clinical features and prognostic risk factors of CPT in the pediatric population and to provide theoretical opinions regarding clinical decisions for CPT.<h4>Methods</h4>The data of 96 patients with CPT and younger than 14 years were retrospectively analyzed. Clinical characteristics such as pathological type ...[more]

PMID: 30539906

Similar Datasets

Project description:The World Health Organization characterizes human choroid plexus tumor (CPT) as papilloma (CPP), atypical CPP (ACPP), and carcinoma (CPC). CPCs can disseminate via cerebrospinal fluid and be mistaken for metastatic carcinoma, creating a diagnostic challenge. Kir7.1 immunohistochemistry (IHC) is a highly reliable tool for diagnostic confirmation of CPTs and their differentiation from metastatic carcinomas in human beings and dogs. This study describes the neuropathology, Kir7.1 staining profile, and the immune cell population within the tumor microenvironment in 11 CPTs in dogs. Archived tissue sections with a diagnosis of CPT were examined and immunolabelled with Kir7.1 for diagnostic confirmation. The number of Ki67-positive neoplastic cells was calculated in 2.4 mm2 (equivalent to 10 FN22/40X fields), and a mean value was generated for each neoplasm. IHC for CD3, CD20, MAC387, and Iba1 was performed for immune cell characterization, and the number of stained cells for each antibody was counted in 2.4 mm2, generating individual cumulative values for each antibody. T-tests with Bonferroni correction evaluated IHC differences between tumor types, and Spearman's rank correlations evaluated relationships among IHC markers. Kir7.1 immunoreactivity was intense at the apical cell membrane in CPPs and ACPPs, and at the apical cell membrane and cytoplasm in CPCs. Ki67 immunoreactivity was detected in all cases. CD3+ and CD20+ lymphocytes trended together (p = 0.005) and were present within and around all CPTs. Five cases had intravascular MAC387+ monocytes. Iba1 immunoreactivity was robust within and around all tumors. Statistical differences in immune cell markers were not found among tumor types. As previously reported, Kir7.1 is a reliable antibody for the diagnosis of canine CPTs. Although immune cells were present in all cases, no significant associations were found between the type of cells and tumor diagnosis. The characterization of the immune cells within CPTs could be useful in future studies involving immunotherapy.

Project description:BackgroundHistological grading of choroid plexus tumors (CPTs) remains the best prognostic tool to distinguish between aggressive choroid plexus carcinoma (CPC) and the more benign choroid plexus papilloma (CPP) or atypical choroid plexus papilloma (aCPP); however, these distinctions can be challenging. Standard treatment of CPC is very aggressive and often leads to severe damage to the young child's brain. Therefore, it is crucial to distinguish between CPC and less aggressive entities (CPP or aCPP) to avoid unnecessary exposure of the young patient to neurotoxic therapy. To better stratify CPTs, we utilized DNA methylation (DNAm) to identify prognostic epigenetic biomarkers for CPCs.MethodsWe obtained DNA methylation profiles of 34 CPTs using the HumanMethylation450 BeadChip from Illumina, and the data was analyzed using the Illumina Genome Studio analysis software. Validation of differentially methylated CpG sites chosen as biomarkers was performed using pyrosequencing analysis on additional 22 CPTs. Sensitivity testing of the CPC DNAm signature was performed on a replication cohort of 61 CPT tumors obtained from Neuropathology, University Hospital Münster, Germany.ResultsGenerated genome-wide DNAm profiles of CPTs showed significant differences in DNAm between CPCs and the CPPs or aCPPs. The prediction of clinical outcome could be improved by combining the DNAm profile with the mutational status of TP53. CPCs with homozygous TP53 mutations clustered as a group separate from those carrying a heterozygous TP53 mutation or CPCs with wild type TP53 (TP53-wt) and showed the worst survival outcome. Specific DNAm signatures for CPCs revealed AK1, PER2, and PLSCR4 as potential biomarkers for CPC that can be used to improve molecular stratification for diagnosis and treatment.ConclusionsWe demonstrate that combining specific DNAm signature for CPCs with histological approaches better differentiate aggressive tumors from those that are not life threatening. These findings have important implications for future prognostic risk prediction in clinical disease management.

Dataset Information

Clinical Features and Prognostic Risk Factors of Choroid Plexus Tumors in Children.

Background

Methods

Results

Conclusions

Publications

Clinical Features and Prognostic Risk Factors of Choroid Plexus Tumors in Children.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets