Brain Tumor Pathology最新文献

Here, we report on a patient presenting with two histopathologically distinct gliomas. At the age of 42, the patient underwent initial resection of a right temporal oligodendroglioma IDH mutated 1p/19q co-deleted WHO Grade II followed by adjuvant radiochemotherapy with temozolomide. 15 months after initial diagnosis, the patient showed right hemispheric tumor progression and an additional new left frontal contrast enhancement in the subsequent imaging. A re-resection of the right-sided tumor and resection of the left frontal tumor were conducted. Neuropathological work-up showed recurrence of the right-sided oligodendroglioma with features of an anaplastic oligodendroglioma WHO Grade III, but a glioblastoma WHO grade IV for the left frontal lesion. In depth molecular profiling revealed two independent brain tumors with distinct molecular profiles of anaplastic oligodendroglioma IDH mutated 1p/19q co-deleted WHO Grade III and glioblastoma IDH wildtype WHO grade IV. This unique and rare case of a patient with two independent brain tumors revealed by in-depth molecular work-up and epigenomic profiling emphasizes the importance of integrated work-up of brain tumors including methylome profiling for advanced patient care.

To determine the prognostic significance of histopathological features included in the diagnostic criteria of atypical meningioma for progression-free survival (PFS). We performed a retrospective cohort study and meta-analysis. Brain invasion, mitotic index, spontaneous necrosis, sheeting, prominent nucleoli, high cellularity, and small cells were the histopathological features of interest. The data from 25 studies involving 3590 patients including our cohort (n = 262) were included. The pooled HR of mitotic index at a cutoff value of 4 showed no statical significance in the gross analysis (pooled HR, 1.09; 95% CI 0.61-1.96; p = 0.7699). Furthermore, it failed to prognosticate PFS in other pooled analyses. For brain invasion, no consistent association with the progression was found in each pooled analysis according to the included studies. Among the remaining five atypical features, spontaneous necrosis, sheeting, and prominent nucleoli showed a significant correlation with PFS in the gross analysis. In the analysis that pooled the HRs from the multivariate analyses, only spontaneous necrosis had significant association with PFS. The available evidence supports that the current cutoff value of mitotic index for diagnosing atypical meningioma might be improper to have prognostic value. The prognostic significance of brain invasion also needs further evaluation.

Glioneuronal and neuronal tumors (GNTs) are rare heterogeneous central nervous system tumors characterized by slow growth and favorable outcomes, but are often associated with diagnostic difficulties. A thorough analysis of three rare and recently recognized GNTs was performed in the context of clinicopathological features and molecular genetic characterization. The current spinal diffuse leptomeningeal glioneuronal tumor (DLGNT) was characterized with oligodendroglioma-like tumor with chromosome 1p/19q codeletion without IDH mutations and KIAA1549:BRAF fusion. The current occipital multinodular and vacuolating neuronal tumor (MVNT) was characteristic of the variable-sized vague nodules consisted of gangliocytic tumor cells with intracytoplasmic and pericellular vacuolation and the next-generation sequencing (NGS) revealed MAP2K1 p.Q56_V60del. A diffuse glioneuronal tumor with oligodendroglioma-like features and nuclear clusters (DGONC) of the amygdala was characterized by oligodendroglia-like cells and nuclear clusters, and monosomy 14. From the current cases and literature review, we found that DLGNT commonly occurs in the spinal cord and can make mass and more commonly have KIAA1549:BRAF fusion; MVNT is a neoplasm rather than malformation and MAP2K1 deletion is one of the hallmarks of this tumor; although DGONC may require a methylation profile, we can reach a diagnosis through its unique histology, monosomy 14, and exclusion diagnosis without a methylation profile.