EP329 / #1049 Intratumoral Hemorrhage In Pediatric Low-Grade-Gliomas: An International Case Series
Details
Serval ID
serval:BIB_D20A41BF5A97
Type
Inproceedings: an article in a conference proceedings.
Publication sub-type
Abstract (Abstract): shot summary in a article that contain essentials elements presented during a scientific conference, lecture or from a poster.
Collection
Publications
Institution
Title
EP329 / #1049 Intratumoral Hemorrhage In Pediatric Low-Grade-Gliomas: An International Case Series
Title of the conference
Pediatric Blood & Cancer
Publisher
The American Society of Pediatric Hematology/Oncology, The International Society of Paediatric Oncology
Organization
54th Congress of the International Society of Paediatric Oncology (SIOP)
Address
Barcelona, Spain, September 28 – October 1, 2022
ISSN
1545-5009
1545-5017
1545-5017
Publication state
Published
Issued date
11/2022
Editor
Vázquez-Gómez Felisa, Baroni Lorena
Volume
69
Number
S5
Language
english
Abstract
Background and Aims: Pediatric low-grade gliomas (PLGG) are grade I and II WHO tumors. Intratumoral hemorrhage is a common complication (up to 8-20%), not well reported in the literature. It can happen at diagnosis, treatment, or follow-up. We have collected an International cohort of PLGG which developed an intratumoral hemorrhage.
Methods: International, multicentric, observational, descriptive and retrospective analysis of 28 patients with a PLGG who suffered an intratumoral hemorrhage. We gathered data on demographic characteristics, histology, molecular findings, treatment received, and information related to the hemorrhagic event.
Results: Twenty-eight patients were enrolled onto the study. Median age at tumor diagnosis was 10.1 years (range, 0.4 to 17.5 years). Three patients (10.7%) had a cancer predisposition syndrome (2 Neurofibromatosis type 1 and 1 Noonan syndrome). Most frequent histological subtypes: pilocytic astrocytoma (67.8%), pleomorphic xantoastrocytoma (10.7%) and pilomyxoid astrocytoma (7%). The most common locations: hypothalamic-chiasmatic (32.1%) and posterior fossa (25%). BRAF status was available in 16 patients (4 had a KIAA1549BRAF fusion and 2 a BRAFV600E mutation). NGS was performed in 10 patients, four of them had an FGFR alteration. Regarding therapy, all patients had initial surgery, with 42.8% needing further surgeries. Seventeen patients (60.7%) received at least one chemotherapy regimen, and six patients (21.4%) received radiotherapy. Twenty-four patients (85.7%) had a spontaneous intratumoral hemorrhage; other etiologies were post-traumatic (10.7%) and post-surgical (3.6%). In thirteen patients (46.4%), the hemorrhages occurred at the time of diagnosis. The main treatment strategies were surgery and observation (39.2% each group). Two patients (7.1%) died because of the bleeding event. To date, 75% of the patients are alive, although 4 of them (14.2%) had a recurrence of their hemorrhage.
Conclusions: Pediatric low grade gliomas are at risk of intratumoral hemorrhage, which occurs usually at diagnosis and spontaneously. Despite being a life-threatening situation, mortality and recurrence rates are low in these patients.
Methods: International, multicentric, observational, descriptive and retrospective analysis of 28 patients with a PLGG who suffered an intratumoral hemorrhage. We gathered data on demographic characteristics, histology, molecular findings, treatment received, and information related to the hemorrhagic event.
Results: Twenty-eight patients were enrolled onto the study. Median age at tumor diagnosis was 10.1 years (range, 0.4 to 17.5 years). Three patients (10.7%) had a cancer predisposition syndrome (2 Neurofibromatosis type 1 and 1 Noonan syndrome). Most frequent histological subtypes: pilocytic astrocytoma (67.8%), pleomorphic xantoastrocytoma (10.7%) and pilomyxoid astrocytoma (7%). The most common locations: hypothalamic-chiasmatic (32.1%) and posterior fossa (25%). BRAF status was available in 16 patients (4 had a KIAA1549BRAF fusion and 2 a BRAFV600E mutation). NGS was performed in 10 patients, four of them had an FGFR alteration. Regarding therapy, all patients had initial surgery, with 42.8% needing further surgeries. Seventeen patients (60.7%) received at least one chemotherapy regimen, and six patients (21.4%) received radiotherapy. Twenty-four patients (85.7%) had a spontaneous intratumoral hemorrhage; other etiologies were post-traumatic (10.7%) and post-surgical (3.6%). In thirteen patients (46.4%), the hemorrhages occurred at the time of diagnosis. The main treatment strategies were surgery and observation (39.2% each group). Two patients (7.1%) died because of the bleeding event. To date, 75% of the patients are alive, although 4 of them (14.2%) had a recurrence of their hemorrhage.
Conclusions: Pediatric low grade gliomas are at risk of intratumoral hemorrhage, which occurs usually at diagnosis and spontaneously. Despite being a life-threatening situation, mortality and recurrence rates are low in these patients.
Keywords
Oncology, Hematology, Pediatrics, Perinatology and Child Health
Pubmed
Create date
26/01/2024 11:29
Last modification date
27/01/2024 8:38
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