Molecular Diagnostic and Prognostic Subtyping of Gliomas in Tunisian Population.

It has become increasingly evident that morphologically similar gliomas may have distinct clinical phenotypes arising from diverse genetic signatures. To date, glial tumours from the Tunisian population have not been investigated. To address this, we correlated the clinico-pathology with molecular data of 110 gliomas by a combination of HM450K array, MLPA and TMA-IHC. PTEN loss and EGFR amplification were distributed in different glioma histological groups. However, 1p19q co-deletion and KIAA1549:BRAF fusion were, respectively, restricted to Oligodendroglioma and Pilocytic Astrocytoma. CDKN2A loss and EGFR overexpression were more common within high-grade gliomas. Furthermore, survival statistical correlations led us to identify Glioblastoma (GB) prognosis subtypes. In fact, significant lower overall survival (OS) was detected within GB that overexpressed EGFR and Cox2. In addition, IDH1R132H mutation seemed to provide a markedly survival advantage. Interestingly, the association of IDHR132H mutation and EGFR normal status, as well as the association of differentiation markers, defined GB subtypes with good prognosis. By contrast, poor survival GB subtypes were defined by the combination of PTEN loss with PDGFRa expression and/or EGFR amplification. Additionally, GB presenting p53-negative staining associated with CDKN2A loss or p21 positivity represented a subtype with short survival. Thus, distinct molecular subtypes with individualised prognosis were identified. Interestingly, we found a unique histone mutation in a poor survival young adult GB case. This tumour exceptionally associated the H3F3A G34R mutation and MYCN amplification as well as 1p36 loss and 10q loss. Furthermore, by exhibiting a remarkable methylation profile, it emphasised the oncogenic power of G34R mutation connecting gliomagenesis and chromatin regulation.

Transcortical approach to a huge pineal mature teratoma.

Intracranial teratomas are rare germ cell neoplasms occurring more often during childhood. We report the case of a huge mature teratoma of the pineal region in a 10-year-old patient that was not correctly diagnosed preoperatively by stereotactic biopsy. The tumor was revealed by intracranial hypertension and a Parinaud syndrome. The tumor markers were within normal levels in the serum. A left transcortical parietal approach was used to completely resect the tumor. No adjuvant treatment was given. A complete neurological recovery was observed after the surgical procedure. Follow-up at 2 years did not show any recurrence. Pineal mature teratomas have a good prognosis, in contrast to their immature or mixed counterparts. A rigorous histological examination of the tumor samples is mandatory, in order to not omit a mixed contingent within the tumor. The treatment is exclusively surgical.

[Intramedullary epidermoid cyst: report of five cases]. / Kystes ipidermoïdes intramédullaires: a propos de cinq cas.

BACKGROUND: Spinal epidermoid cysts are rare tumors and they are exceptionally intramedullary. AIM: Report of news cases CASE REPORT: The diagnosis is established by magnetic resonance imaging and the treatment is surgical. The prognosis is generally good, with possible recurrence after many years. The author report five new cases of intramedullary epidermoide cysts operated between 1996 and 2001.