Background
Pseudoprogression in gliomas has been extensively described after radiotherapy with or without chemotherapy, but not after chemotherapy alone. Here we describe the occurrence of ...pseudoprogression in patients with anaplastic oligodendrogliomas treated with postoperative procarbazine, lomustine and vincristine (PCV) chemotherapy alone.
Methods
We retrospectively reviewed the medical and radiological files of patients with 1p/19q codeleted, IDH‐mutant anaplastic oligodendrogliomas treated with PCV chemotherapy alone who presented magnetic resonance imaging (MRI) modifications suggestive of tumour progression and in whom the final diagnosis was a pseudoprogression.
Results
We identified six patients. All patients underwent a surgical resection and were treated with PCV chemotherapy without radiotherapy. After a median of 11 months following the initiation of chemotherapy (range: 3–49 months), the patients developed asymptomatic white matter MRI modifications around the surgical cavity leading to the suspicion of a tumour progression. These modifications appeared as hyperintense on T2‐fluid‐attenuated inversion recovery (FLAIR) sequence, hypointense on T1 sequence, and lacked mass effect (0/6), contrast enhancement (0/6), restriction on diffusion‐weighted imaging (0/4), relative cerebral blood volume (rCBV) increase on perfusion MRI (0/4), and hypermetabolism on 18F‐fluoro‐L‐dopa positron emission tomography (18F‐DOPA PET) scan (0/3). One patient underwent a surgical resection demonstrating no tumour recurrence; the five other patients were considered as having post‐therapeutic modifications based on imaging characteristics. After a median follow‐up of 4 years all patients were progression‐free.
Conclusions
Anaplastic oligodendroglioma patients treated with postoperative PCV chemotherapy alone occasionally develop T2/FLAIR hyperintensities around the surgical cavity that can wrongly suggest tumour progression. Multimodal imaging and close follow‐up should be considered in this situation.
The Central Nervous System (CNS) tumor with BCOR internal tandem duplication (ITD) has recently been added as a novel embryonal histomolecular tumor type to the 2021 World Health Organization (WHO) ...Classification of CNS Tumors. In addition, other CNS tumors harboring a BCOR/BCORL1 fusion, which are defined by a distinct DNA-methylation profile, have been recently identified in the literature but clinical, radiological and histopathological data remain scarce. Herein, we present two adult cases of CNS tumors with EP300::BCOR fusion. These two cases presented radiological, histopathological, and immunohistochemical homologies with CNS tumors having BCOR ITD in children. To compare these tumors with different BCOR alterations, we performed a literature review with a meta-analysis. CNS tumors with EP300::BCOR fusion seem to be distinct from their BCOR ITD counterparts in terms of age, location, progression-free survival, tumor growth pattern, and immunopositivity for the BCOR protein. CNS tumors from the EP300::BCOR fusion methylation class in adults may be added to the future WHO classification.
Here we report two tumors with a HGNET-BCOR methylation class (MC) but harboring a BCOR fusion with the EP300 gene (encoding the protein p300 which is an acetyltransferase histone implicated in ...controlling cell growth and differentiation). SEE PDF Interestingly, this same fusion was previously reported in gliomas 7 but these cases were distinct of our cases from radiology (infiltrative pattern), histopathology and immunohistochemistry (infiltrative proliferation with calcifications, composed of GFAP positive cells without expression of neuronal markers) 7. ...gliomas described by Torre et al. were in close vicinity to LGG with an MYB/MYBL1 alteration by t-Distributed Stochastic Neighbor Embedding plot (t-SNE) analysis whereas our cases were classified into the MC HGNET-BCOR and clearly clustered with HGNET-BCOR by t-SNE analysis (Fig. 4) 7. ...the EP300:BCOR fusion expands the spectrum of the alterations encountered in the MC HGNET-BCOR, and therefore, the terminology “CNS tumors with BCOR ITD” seems to be too restrictive. Because the BCOR immunohistochemistry does not allow detections of HGNET-BCOR with fusion, we recommand searching for alternative alterations of the BCOR gene in the event of radiological and histopathological suspicion of this diagnosis when ITD is absent.
•Common long-term memory defects after pediatric brain tumor affect school achievement•The hippocampi, the cerebellum and cerebellar-cortical networks play a role in several memory systems.•This ...study will provide long-term neuropsychological data about four different memory systems.•We will investigate the correlations between neuropsychological, neuroimaging and radiotherapy dose data.•Imaging will be structural (3DT1), microstructural (DTI), functional (rs-fMRI), vascular (ASL) and metabolic (spectroscopy).
Posterior fossa tumors represent two thirds of brain tumors in children. Although progress in treatment has improved survival rates over the past few years, long-term memory impairments in survivors are frequent and have an impact on academic achievement. The hippocampi, cerebellum and cerebellar-cortical networks play a role in several memory systems. They are affected not only by the location of the tumor itself and its surgical removal, but also by the supratentorial effects of complementary treatments, particularly radiotherapy. The IMPALA study will investigate the impact of irradiation doses on brain structures involved in memory, especially the hippocampi and cerebellum.
In this single-center prospective behavioral and neuro-imaging study, 90 participants will be enrolled in three groups. The first two groups will include patients who underwent surgery for a posterior fossa brain tumor in childhood, who are considered to be cured, and who completed treatment at least 5 years earlier, either with radiotherapy (aggressive brain tumor; Group 1) or without (low-grade brain tumor; Group 2). Group 3 will include control participants matched with Group 1 for age, sex, and handedness. All participants will perform an extensive battery of neuropsychological tests, including an assessment of the main memory systems, and undergo multimodal 3 T MRI. The irradiation dose to the different brain structures involved in memory will be collected from the initial radiotherapy dosimetry.
This study will provide long-term neuropsychological data about four different memory systems (working memory, episodic memory, semantic memory, and procedural memory) and the cognitive functions (attention, language, executive functions) that can interfere with them, in order to better characterize memory deficits among the survivors of brain tumors. We will investigate the correlations between neuropsychological and neuroimaging data on the structural (3DT1), microstructural (DTI), functional (rs-fMRI), vascular (ASL) and metabolic (spectroscopy) impact of the tumor and irradiation dose. This study will thus inform the setting of dose constraints to spare regions linked to the development of cognitive and memory functions.
ClinicalTrials.gov: NCT04324450, registered March 27, 2020, updated January 25th, 2021. Retrospectively registered, https://www.clinicaltrials.gov/ct2/show/NCT04324450.
To collect outcome data in a large cohort of patients with aggressive pituitary tumours (APT)/carcinomas (PC) and specifically report effects of temozolomide (TMZ) treatment.
Electronic survey to ESE ...members Dec 2015-Nov 2016.
Reports on 166 patients (40 PC, 125 APT, 1 unclassified) were obtained. Median age at diagnosis was 43 (range 4-79) years. 69% of the tumours were clinically functioning, and the most frequent immunohistochemical subtype were corticotroph tumours (45%). Ki-67 index did not distinguish APT from PC, median 7% and 10% respectively. TMZ was first-line chemotherapy in 157 patients. At the end of the treatment (median 9 cycles), radiological evaluation showed complete response (CR) in 6%, partial response (PR) in 31%, stable disease (SD) in 33% and progressive disease in 30%. Response was more frequent in patients receiving concomitant radiotherapy and TMZ. CR was seen only in patients with low MGMT expression. Clinically functioning tumours were more likely to respond than non-functioning tumours, independent of MGMT status. Of patients with CR, PR and SD, 25, 40 and 48% respectively progressed after a median of 12-month follow-up. Other oncological drugs given as primary treatment and to TMZ failures resulted in PR in 20%.
This survey confirms that TMZ is established as first-line chemotherapeutic treatment of APT/PC. Clinically functioning tumours, low MGMT and concurrent radiotherapy were associated with a better response. The limited long-term effect of TMZ and the poor efficacy of other drugs highlight the need to identify additional effective therapies.
Les gliomes de haut grade IDH-mutés prennent souvent le contraste. Cependant la fréquence et la valeur pronostique de la prise de contraste (PC) au sein de ces tumeurs restent à déterminer.
Décrire ...la fréquence et la valeur pronostique de la PC au sein des gliomes de haut grade IDH-mutés diagnostiqués (classification OMS 2021).
Étude rétrospective au sein des gliomes de haut grade IDH-mutés inclus dans le réseau POLA dans une série de référence (IRM relues, n=108) et au sein d’une série de validation (n=684, annotation disponible dans l’e-crf).
Dans la série de référence et la série de validation, une PC était plus fréquente dans les oligodendrogliomes de grade 3 (O3) et les astrocytomes de grade 4 (A4) que dans les astrocytomes de grade 3 (A3), 75 %, 63 % vs 46 % (p<0,001) et 70 %, 78 % vs 53 % (p<0,001). Dans les 2 séries, la présence d’une PC était associée à un moins bon pronostic au sein des O3 (p=0,08 et p=0,006), indépendamment de l’âge, de l’index de Karnofsky et du type de traitement initial (p=0,001).
La fréquence et la valeur pronostique de la PC varient au sein des gliomes de haut grade IDH-mutés.
Face à une suspicion clinicoradiologique de gliome IDH-muté, l’absence de PC ne permet pas d’écarter le diagnostic de gliome de haut grade. Au sein des oligodendrogliomes, l’existence d’une PC est associée à un moins bon pronostic.
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