It has become evident that pediatric brain stem gliomas are a heterogeneous pathology and should be classified according to clinical and radiological criteria. This classification has contributed to better treatment and greatly improved prognosis. Based on a review of the literature, we describe the different types of brain stem astrocytomas reported, which are: cervicomedullary, exophytic, cystic, focal and diffuse. Particular attention is paid to therapeutic modalities. For the first three lesions named the treatment is surgical and oncological therapy should be evaluated only for regrowth of the mass. Focal tumors of the medulla and pons are still treated empirically (surgically and/or with radiotherapy), because a definitive therapeutic protocol has not yet been elaborated; on the other hand it is well established that if the focal mass is in the midbrain this should merely be monitored by means of serial MRI, while radiotherapy should be applied in the event of the tumor's growth. Diffuse gliomas are treated with oncological therapy, and surgery (for biopsy or tumor excision) is not indicated. Five illustrative cases from our department are presented.
Ependymoma is a malignant pediatric brain tumor, often incurable under the current treatment regimen. We aimed to evaluate the expression of microRNAs (miRs) in pediatric ependymoma tumors in an attempt to identify prognostic molecular markers which would lead to potential therapeutic targets. Following miR-array expression analysis, we focused on 9 miRs that correlated with relapse which were further validated by quantitative real-time PCR (qRT-PCR) in a cohort of 67 patients. Western blotting and immunohistochemistry were used to measure target protein expression in 20 and 34 tumor samples, respectively. High expression of miR-124-3p significantly correlated with the lower progression-free survival (PFS) of 16% compared to 67% in those expressing low levels (P = .002). Interestingly, in the group of patients with local disease (n = 56) expression levels of this miR distinguished 2 subgroups with a significantly different outcome (P = .001). miR-124-3p was identified as an independent prognostic factor of relapse in the multivariate analysis performed in the whole cohort and in the group with localized disease. In the localized group, a patient expressing high levels of miR-124-3p had a 4.1-fold increased risk for relapse (P = .005). We demonstrated the direct binding of miR-124-3p to its target TP53INP1. Negative TP53INP1 protein levels correlated with a poor outcome (P = .034). We propose miR-124-3p and TP53INP1 as new biomarkers for prognostic stratification that may be possible therapeutic targets for ependymoma.
Tumor's size has significant impact on the management of CP in children. It affects both short-term factors (initial symptoms, urgency of surgical resection, extent of resection, and perioperative morbidity) as well as long-term parameters (PFS, rate of adjuvant treatments/recurrent surgeries, and metabolic/endocrinal/ophthalmological and functional outcomes). We think that a proper, world-wide accepted definition of what is "large," "giant," or even "monstrous" CP should be established. This will enable carrying multi-institutional studies on a larger group of patients, allowing further determining the importance of tumor's size in the management and outcome of craniopharyngiomas in children.
Histiocytic sarcoma is a rare, lymphohematopoietic malignant neoplasm composed of tumor cells showing morphologic and immunophenotypic features of mature tissue histiocytes. Involvement of the central nervous system (CNS) as either a part of a systemic disease or as a primary lesion has rarely been described so far. We present a case of primary CNS histiocytic sarcoma in an adult patient and review the literature on this rare entity.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.