The role of clinical factors and immunocheckpoint molecules in the prognosis of patients with supratentorial extraventricular ependymoma: a single-center retrospective study

Abstract

Purpose Supratentorial extraventricular ependymoma (SEE) is a rare subset of ependymomas located in the supratentorial parenchyma, and little is known regarding its management and prognosis. Our study aimed to reveal the prognostic factors in patients with SEE and the roles of programmed death ligand-1 (PD-L1), programmed cell death protein 1 (PD-1), Ki-67, and neural cell adhesion molecule L1 (L1CAM) in predicting these patients’ outcomes. Methods We retrospectively studied the clinical features and prognostic factors in 48 patients with SEE admitted to our center from April 2008 to October 2018. Tissue slides were constructed from patient samples, and PD-L1, PD-1, Ki-67, and L1CAM expression levels were evaluated by immunohistochemistry. Results Patients with gross total resection (GTR) had better progression-free survival than patients with subtotal resection (STR). Moreover, the recurrence hazard ratios in patients with STR at 3, 5, and 10 years were 8.746, 6.866 and 3.962 times those of patients with GTR, respectively. PD-L1 positivity predicted worse progression-free survival, while the recurrence hazard ratios for patients with PD-L1 positivity at 3, 5, and 10 years were 10.445, 5.539, and 3.949 times those of patients with PD-L1 negativity, respectively. Multivariate analysis revealed that PD-L1 expression and GTR could independently predict outcomes in patients with SEE. Conclusion PD-L1 expression was an independent and more readily obtained predictor of outcomes, representing a simple and reliable biological prognostic factor for patients with SEE. Further studies are needed to explore PD-L1 inhibitor treatment for patients with ependymoma. Clinical trial registration No clinical trials were performed in the study.

Molecular Classification and Therapeutic Targets in Ependymoma

“…Lastly, immune checkpoint molecules are another possible target currently being investigated. Wang et al uncovered that expression of PD-L1 independently predicts outcomes in patients with ST, extraventricular ependymomas [ 193 ]. T-cell exhaustion induced by overexpression of PD-L1 is a mechanism by which tumors evade immune-mediated clearance [ 194 ].…”

Supratentorial extra ventricular intraparenchymal ependymoma in a child

“…According to our revision, patients who underwent macroscopically total resection had better progression-free survival than those who had subtotal resection [8]. Grade II ependymal tumors had a better progression-free survival (PFS) and overall survival (OS) compared to grade III tumors with statistically significant differences (P < 0.01 and 0.02, respectively) [2].…”

“…Studies note an average of 1 to 3 cases per year. Thus, there were 15 cases in 15 years [2], 11 cases in 11 years [5], 6 cases in 12 years [7], 56 cases in 17 years [9], 66 cases in 20 years [8]. Other authors have published single cases in male adults (23, 42, 61 years) [3,4,6] or in a 16-year-old patient [1].…”

Immunobiology and Cytokine Modulation of the Pediatric Brain Tumor Microenvironment: A Scoping Review

“…Although the expression of this immune-suppressive axis is common in many solid cancers, the expression in pediatric tumors is less common [96,97] and can be markedly heterogeneous [98]. In supratentorial extra-ventricular ependymomas, PD-L1 expression was a negative prognosticator for progression-free survival [99]. Ependymoma that harbored a RELA fusion (ST-RELA) had high PD-L1 expression on both the tumor cells and myeloid cells.…”