scholarly journals MPTH-06BRAFV600E MUTATION IN HIGH GRADE GLIOMAS OF CHILDREN AND YOUNG ADULTS

2015 ◽  
Vol 17 (suppl 5) ◽  
pp. v139.1-v139
Author(s):  
Sridhar Epari ◽  
Hetakshi Kurani ◽  
Tejpal Gupta ◽  
Girish Chinnaswamy ◽  
Jayant Sastri Goda ◽  
...  
Author(s):  
Winai Sirichativapee ◽  
Taweechok Wisanuyotin ◽  
Porjai Pattanittum ◽  
Permsak Paholpak ◽  
Pat Laupattarakasem ◽  
...  

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii345-iii346
Author(s):  
Alexandre Roux ◽  
Johan Pallud ◽  
Raphaël Saffroy ◽  
Myriam Edjlali-Goujon ◽  
Marie-Anne Debily ◽  
...  

Abstract BACKGROUND Considering that paediatric high-grade gliomas (HGGs) are biologically distinct from their adult counterparts, the objective of this study was to define the landscape of HGGs in adolescents and young adults (AYAs). METHODS We performed a multicentric retrospective study of 112 AYAs from adult and paediatric Ile-de-France neurosurgical units, treated between 1998 and 2013 to analyse their clinicoradiological and histomolecular profiles. The inclusion criteria were age between 15 and 25-years, histopathological HGG diagnosis, available clinical data, pre-operative and follow-up MRI. MRI and tumoral samples were centrally reviewed. Immunohistochemistry and complementary molecular techniques such as targeted/next generation sequencing, whole exome sequencing and DNA-methylation analyses were performed to achieve an integrated diagnosis according to the 2016 WHO classification. RESULTS Based on 80 documented AYA patients, HGGs constitute heterogeneous clinicopathological and molecular groups, with a predominant representation of paediatric-subtypes (Histone H3-mutants, 40%) but also adult-subtypes (IDH-mutants, 28%) characterized by the rarity of oligodendrogliomas, IDH-mutant and 1p/19q co-deleted and the relative high frequency of “rare adult IDH mutations” (20%). H3G34-mutants (14%) represent the most specific subgroup in AYAs. In the H3K27-mutant subgroup, the non-brainstem diffuse midline gliomas are more frequent (66.7%) than diffuse intrinsic pontine gliomas (23.8%), contrary to children. We found that WHO grade has no prognostic value, but molecular subgrouping has major prognostic importance. CONCLUSIONS HGGs in AYAs could benefit from a more personalized neuro-oncological management, driven by molecular subtyping rather than age group. Collaborative efforts are needed from paediatric and adult neuro-oncology teams to improve the management of HGGs in AYAs.


Author(s):  
Winai Sirichativapee ◽  
Taweechok Wisanuyotin ◽  
Porjai Pattanittum ◽  
Permsak Paholpak ◽  
Pat Laupattarakasem ◽  
...  

1996 ◽  
Vol 14 (9) ◽  
pp. 2495-2503 ◽  
Author(s):  
J L Finlay ◽  
S Goldman ◽  
M C Wong ◽  
M Cairo ◽  
J Garvin ◽  
...  

PURPOSE This study was designed to determine the toxicity, radiographic response rate, and outcome following high-dose thiotepa, etoposide, and autologous bone marrow rescue (ABMR) for young patients with recurrent malignant brain tumors. METHODS Eligibility criteria required adequate renal, hepatic, and pulmonary function, and no bone marrow infiltration. Thiotepa 300 mg/m2 and etoposide 500 mg/ m2 were infused on 3 consecutive days, and autologous bone marrow was infused 72 hours following chemotherapy. RESULTS Forty-five patients with recurrent high-grade brain tumors, aged 8 months to 36 years (median, 8 years), were treated. Seven patients (16%) died of treatment-related toxicities within 56 days of marrow reinfusion. Delayed platelet engraftment occurred in 44% of patients who survived beyond day 56. Of 35 patients with radiographically measurable disease who survived at least 28 days following ABMR, there were two complete responses (CRs) and six partial responses (PRs), for an overall response (CRs plus PRs) rate of 23% (SE = 7%). Objective responses were observed in four of 14 assessable patients with high-grade glioma and in two of six with primitive neuroectodermal tumors (PNETs)/ medulloblastoma. Survival was significantly improved in patients treated with minimal residual disease (P < .0005). Five of 18 patients (28%) with high-grade gliomas remain free of disease at 39+, 44+, 49+, 52+, and 59+ months post-ABMR. CONCLUSION The combination of high-dose thiotepa and etoposide has activity against a variety of recurrent childhood brain tumors. These results merit further evaluation in children and young adults with both recurrent and newly diagnosed high-grade brain tumors.


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