H3K27M Mutation Doesn’t Mean Worse Prognosis in Old Patients

Hu, Xiao Mu; Nie, Xiao yu; Xu, Kai; Wang, Yin; Tang, Feng; Du, Zun guo; Xiong, Ji

doi:10.3389/fonc.2022.912166

Cited by 4 publications

(5 citation statements)

References 27 publications

(27 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Other studies also demonstrated that tumor location was not statistically relevant to the survival of adult DMG. 48,49 Single lesion was another favorable prognosticator in the entire cohort and the adult subgroup when compared with multiple lesions or LMD. The prognostic value of dissemination characteristics was rarely reported.…”

Section: Discussionmentioning

confidence: 89%

See 1 more Smart Citation

Differences in survival prognosticators between children and adults with H3K27M‐mutant diffuse midline glioma

et al. 2023

View full text Add to dashboard Cite

AimsH3K27M‐mutant diffuse midline glioma (DMG) is a rare and aggressive central nervous system tumor. The biological behavior, clinicopathological characteristics, and prognostic factors of DMG have not yet been completely uncovered, especially in adult patients. This study aims to investigate the clinicopathological characteristics and identify prognostic factors of H3K27M‐mutant DMG in pediatric and adult patients, respectively.MethodsA total of 171 patients with H3K27M‐mutant DMG were included in the study. The clinicopathological characteristics of the patients were analyzed and stratified based on age. The Cox proportional hazard model was used to determine the independent prognostic factors in pediatric and adult subgroups.ResultsThe median overall survival (OS) for the entire cohort was 9.0 months. Significant differences were found in some clinicopathological characteristics between children and adults. The median OS was also significantly different between the pediatric and adult subgroups, with 7.1 months for children and 12.3 months for adults (p < 0.001). In the overall population, the multivariate analysis identified adult patients, single lesion, concurrent chemoradiotherapy/radiotherapy, and intact ATRX expression as independent favorable prognostic factors. In the age‐stratified subgroups, the prognostic factors varied between children and adults, with intact ATRX expression and single lesion being independent favorable prognostic factors in adults, while infratentorial localization was significantly associated with worse prognosis in children.ConclusionsThe differences in clinicopathological features and prognostic factors between pediatric and adult patients with H3K27M‐mutant DMG suggest the need for further clinical and molecular stratification based on age.

show abstract

Section: Discussionmentioning

confidence: 89%

“…In this study, the patients with supratentorial/spinal cord tumors exhibited a longer survival time, and tumor location was an independent prognostic factor in the pediatric cohort, but not in adults. Other studies also demonstrated that tumor location was not statistically relevant to the survival of adult DMG 48,49 …”

Section: Discussionmentioning

confidence: 99%

Differences in survival prognosticators between children and adults with H3K27M‐mutant diffuse midline glioma

et al. 2023

View full text Add to dashboard Cite

show abstract

“…The prevalence of the H3 K27 mutation in adult midline glioma ranges from 13% to 53%. 9 , 11 , 30 , 31 According to the literature review, P53 and ATRX alterations were found to be the most common genetic mutations associated with the H3 K27 alteration in adult DMGs. Conversely, recent studies have found that IDH1/IDH2 mutations and MGMT promoter methylation were frequently absent in this tumor, which contributes to its poor response to temozolomide and its dismal prognosis.…”

Section: Discussionmentioning

confidence: 99%

“… 3 Although the H3 K27 mutation has been shown to be a poor prognostic factor in children and younger adults, its presence in older adults does not seem to be associated with a worse prognosis than H3 K27 wild-type high-grade midline gliomas. 10 , 11 , 33 …”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Fulminant hemorrhagic course of a thalamic H3 K27-altered diffuse midline glioma in an adult patient: illustrative case

Poletti,

Marques,

Deigendesch

et al. 2024

Journal of Neurosurgery: Case Lessons

View full text Add to dashboard Cite

BACKGROUND H3 K27-altered diffuse midline gliomas (DMGs) are rare tumors, which are, regardless of their histological appearance, classified as World Health Organization grade 4 tumors. They are characterized by a diffuse growth pattern, midline anatomical location, and poor prognosis. Although DMGs occur predominantly in childhood, these tumors can also be found in young adults. OBSERVATIONS The authors present a case of a 29-year-old patient who was found unconscious with a Glasgow Coma Scale score of 4, along with abnormal extensor movements and bilateral middilated nonreactive pupils. Computed tomography revealed obstructive hydrocephalus due to an acute hemorrhage in a right thalamic lesion. To drain the hydrocephalus and relieve the ongoing central herniation, emergent placement of a right-sided, and later a left-sided, extraventricular drain was performed. Despite the postoperative resolution of hydrocephalus, the patient died shortly after because of the central brain herniation that had occurred. Brain autopsy revealed a H3 K27-altered DMG in the right thalamus. LESSONS Although typically described in the pediatric population and located in the pons, H3 K27-altered DMG should also be considered in young adult patients with midline lesions, particularly if they are located in the thalamus or brainstem. In rare cases, H3 K27-altered DMG may present with an acute tumor-related hemorrhage, leading to a fulminant clinical course.

show abstract

Signaling pathways in brain tumors and therapeutic interventions

Wang

Chen

et al. 2023

Sig Transduct Target Ther

View full text Add to dashboard Cite

Brain tumors, although rare, contribute to distinct mortality and morbidity at all ages. Although there are few therapeutic options for brain tumors, enhanced biological understanding and unexampled innovations in targeted therapies and immunotherapies have considerably improved patients’ prognoses. Nonetheless, the reduced response rates and unavoidable drug resistance of currently available treatment approaches have become a barrier to further improvement in brain tumor (glioma, meningioma, CNS germ cell tumors, and CNS lymphoma) treatment. Previous literature data revealed that several different signaling pathways are dysregulated in brain tumor. Importantly, a better understanding of targeting signaling pathways that influences malignant behavior of brain tumor cells might open the way for the development of novel targeted therapies. Thus, there is an urgent need for a more comprehensive understanding of the pathogenesis of these brain tumors, which might result in greater progress in therapeutic approaches. This paper began with a brief description of the epidemiology, incidence, risk factors, as well as survival of brain tumors. Next, the major signaling pathways underlying these brain tumors’ pathogenesis and current progress in therapies, including clinical trials, targeted therapies, immunotherapies, and system therapies, have been systemically reviewed and discussed. Finally, future perspective and challenges of development of novel therapeutic strategies in brain tumor were emphasized.

show abstract

H3K27M Mutation Doesn’t Mean Worse Prognosis in Old Patients

Cited by 4 publications

References 27 publications

Differences in survival prognosticators between children and adults with H3K27M‐mutant diffuse midline glioma

Differences in survival prognosticators between children and adults with H3K27M‐mutant diffuse midline glioma

Fulminant hemorrhagic course of a thalamic H3 K27-altered diffuse midline glioma in an adult patient: illustrative case

Signaling pathways in brain tumors and therapeutic interventions

Contact Info

Product

Resources

About