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Academic Radiology Feb 2024The 5th edition of the World Health Organization classification of tumors of the Central Nervous System (WHO CNS) has introduced the term "diffuse" and its counterpart...
RATIONALE AND OBJECTIVES
The 5th edition of the World Health Organization classification of tumors of the Central Nervous System (WHO CNS) has introduced the term "diffuse" and its counterpart "circumscribed" to the category of gliomas. This study aimed to develop and validate models for distinguishing circumscribed astrocytic gliomas (CAGs) from diffuse gliomas (DGs).
MATERIALS AND METHODS
We retrospectively analyzed magnetic resonance imaging (MRI) data from patients with CAGs and DGs across three institutions. After tumor segmentation, three volume of interest (VOI) types were obtained: VOI, VOI, and VOI Clinical and combined models (incorporating radiomics and clinical features) were also established. To address imbalances in training dataset, Synthetic Minority Oversampling Technique was employed.
RESULTS
A total of 475 patients (DGs: n = 338, CAGs: n = 137) were analyzed. The VOI model demonstrated the best performance for differentiating CAGs from DGs, achieving an area under the curve (AUC) of 0.806 and area under the precision-recall curve (PRAUC)of 0.894 in the cross-validation set. Using analysis of variance (ANOVA) feature selector and Support Vector Machine (SVM) classifier, seven features were selected. The model achieved an AUC and AUPRC of 0.912 and 0.972 in the internal validation dataset, and 0.897 and 0.930 in the external validation dataset. The combined model, incorporating interface radiomics and clinical features, showed improved performance in the external validation set, with an AUC of 0.94 and PRAUC of 0.959.
CONCLUSION
Radiomics models incorporating the peritumoral area demonstrate greater potential for distinguishing CAGs from DGs compared to intratumoral models. These findings may hold promise for evaluating tumor nature before surgery and improving clinical management of glioma patients.
Topics: Humans; Nomograms; Retrospective Studies; Radiomics; ROC Curve; Glioma; Magnetic Resonance Imaging; Astrocytoma
PubMed: 37507329
DOI: 10.1016/j.acra.2023.06.033 -
Acta Neuropathologica 1990This autopsy case report describes a clinically aggressive, histologically well-differentiated, diffuse, primary leptomeningeal astrocytoma. This rare tumour produced a...
This autopsy case report describes a clinically aggressive, histologically well-differentiated, diffuse, primary leptomeningeal astrocytoma. This rare tumour produced a meningitic clinical picture associated with hydrocephalus, focal spinal cord necrosis, and signs of spinal and cranial nerve root irritation. Despite the microscopic features, death occurred approximately 7 weeks after the first meningeal symptoms.
Topics: Adult; Arachnoid; Astrocytoma; Humans; Magnetic Resonance Imaging; Male; Microscopy, Electron; Myelography; Pia Mater; Spinal Cord Neoplasms
PubMed: 2399813
DOI: 10.1007/BF00294654 -
Scientific Reports Jan 2023Isocitrate dehydrogenase wild-type (IDHwt) diffuse astrocytomas feature highly infiltrative patterns, such as a gliomatosis cerebri growth pattern with widespread...
Isocitrate dehydrogenase wild-type (IDHwt) diffuse astrocytomas feature highly infiltrative patterns, such as a gliomatosis cerebri growth pattern with widespread involvement. Among these tumors, localized IDHwt histologically diffuse astrocytomas are rarer than the infiltrative type. The aim of this study was to assess and describe the clinical, radiographic, histopathological, and molecular characteristics of this rare type of IDHwt histologically diffuse astrocytomas and thereby provide more information on how its features affect clinical prognoses and outcomes. We retrospectively analyzed the records of five patients with localized IDHwt histologically diffuse astrocytomas between July 2017 and January 2020. All patients were female, and their mean age at the time of the initial treatment was 55.0 years. All patients had focal disease that did not include gliomatosis cerebri or multifocal disease. All patients received a histopathological diagnosis of diffuse astrocytomas at the time of the initial treatment. For recurrent tumors, second surgeries were performed at a mean of 12.4 months after the initial surgery. A histopathological diagnosis of glioblastoma was made in four patients and one of gliosarcoma in one patient. The initial status of IDH1, IDH2, H3F3A, HIST1H3B, and BRAF was "wild-type" in all patients. TERT promoter mutations (C250T or C228T) were detected in four patients. No tumors harbored a 1p/19q codeletion, EGFR amplification, or chromosome 7 gain/10 loss (+ 7/ - 10). We assessed clinical cases of localized IDHwt histologically diffuse astrocytomas that resulted in malignant recurrence and a poor clinical prognosis similar to that of glioblastomas. Our case series suggests that even in patients with histologically diffuse astrocytomas and those who present with radiographic imaging findings suggestive of a localized tumor mass, physicians should consider the possibility of IDHwt histologically diffuse astrocytomas.
Topics: Humans; Female; Middle Aged; Male; Brain Neoplasms; Retrospective Studies; Mutation; Neoplasm Recurrence, Local; Astrocytoma; Glioblastoma; Neoplasms, Neuroepithelial; Isocitrate Dehydrogenase
PubMed: 36646712
DOI: 10.1038/s41598-022-25928-2 -
Frontiers in Bioscience : a Journal and... Jan 2000Our understanding of diffuse glioma development and progression has expanded remarkably over the past decade. As the genetic alterations responsible for these tumors are... (Review)
Review
Our understanding of diffuse glioma development and progression has expanded remarkably over the past decade. As the genetic alterations responsible for these tumors are identified, molecular models of glioma pathogenesis are emerging and hold great promise to explain the biologic mechanisms of these neoplasms. Although these models continue to evolve and remain highly simplified, some of the genetic alterations that they encompass appear to be prognostically useful. Among the astrocytic gliomas, age and tumor grade are the most powerful indicators of patient survival, however, a wide range of variability remains, particularly among the low- grade and anaplastic astrocytomas. Recent reports indicate that alterations of the PTEN tumor suppressor gene are independent predictors of overall survival for anaplastic astrocytoma patients, helping to distinguish the cases with behavior resembling their more malignant counterparts, the glioblastomas. Among the oligodendroglial tumors, alterations of the 1p and 19q chromosome arms have emerged as potentially powerful predictors of overall patient survival and in vivo chemotherapeutic response, while alterations of the p16/CDKN2A tumor suppressor gene suggest shorter overall survival. As our molecular models continue to improve, through functional analyses and the identification of additional genetic contributors, we will expand our capacity to more effectively prognose these patients and to design rational therapeutic strategies.
Topics: Astrocytoma; Central Nervous System Neoplasms; Glioma; Humans; Oligodendroglioma; Prognosis
PubMed: 10702383
DOI: 10.2741/smith -
Acta Neuropathologica Jun 2015Diffusely infiltrating astrocytomas include diffuse astrocytomas WHO grade II and anaplastic astrocytomas WHO grade III and are classified under astrocytic tumours... (Review)
Review
Diffusely infiltrating astrocytomas include diffuse astrocytomas WHO grade II and anaplastic astrocytomas WHO grade III and are classified under astrocytic tumours according to the current WHO Classification. Although the patients generally have longer survival as compared to those with glioblastoma, the timing of inevitable malignant progression ultimately determines the prognosis. Recent advances in molecular genetics have uncovered that histopathologically diagnosed astrocytomas may consist of two genetically different groups of tumours. The majority of diffusely infiltrating astrocytomas regardless of WHO grade have concurrent mutations of IDH1 or IDH2, TP53 and ATRX. Among these astrocytomas, no other genetic markers that may distinguish grade II and grade III tumours have been identified. Those astrocytomas without IDH mutation tend to have a distinct genotype and a poor prognosis comparable to that of glioblastomas. On the other hand, diffuse astrocytomas that arise in children do not harbour IDH/TP53 mutations, but instead display mutations of BRAF or structural alterations involving MYB/MYBL1 or FGFR1. A molecular classification may thus help delineate diffusely infiltrating astrocytomas into distinct pathogenic and prognostic groups, which could aid in determining individualised therapeutic strategies.
Topics: Astrocytoma; Biomarkers, Tumor; Brain Neoplasms; Humans; Neuroimaging
PubMed: 25975377
DOI: 10.1007/s00401-015-1439-7 -
AJNR. American Journal of Neuroradiology 1993This case demonstrated the classic gross, pathologic, CT, and MR findings of pontine astrocytoma. The role of functional brain imaging in identifying regrowth of tumor...
This case demonstrated the classic gross, pathologic, CT, and MR findings of pontine astrocytoma. The role of functional brain imaging in identifying regrowth of tumor was illustrated and the differential diagnosis of a brain stem lesion summarized.
Topics: Astrocytoma; Brain Neoplasms; Child, Preschool; Diagnosis, Differential; Female; Humans; Magnetic Resonance Imaging; Pons; Tomography, X-Ray Computed
PubMed: 8352167
DOI: No ID Found -
Acta Neuropathologica Communications Jun 2021Primary spinal cord astrocytomas are rare, hence few data exist about the prognostic significance of molecular markers. Here we analyze a panel of molecular alterations...
Primary spinal cord astrocytomas are rare, hence few data exist about the prognostic significance of molecular markers. Here we analyze a panel of molecular alterations in association with the clinical course. Histology and genome sequencing was performed in 26 spinal astrocytomas operated upon between 2000 and 2020. Next-generation DNA/RNA sequencing (NGS) and methylome analysis were performed to determine molecular alterations. Histology and NGS allowed the distinction of 5 tumor subgroups: glioblastoma IDH wildtype (GBM); diffuse midline glioma H3 K27M mutated (DMG-H3); high-grade astrocytoma with piloid features (HAP); diffuse astrocytoma IDH mutated (DA), diffuse leptomeningeal glioneural tumors (DGLN) and pilocytic astrocytoma (PA). Within all tumor entities GBM (median OS: 5.5 months), DMG-H3 (median OS: 13 months) and HAP (median OS: 8 months) showed a fatal prognosis. DMG-H3 tend to emerge in adolescence whereas GBM and HAP develop in the elderly. HAP are characterized by CDKN2A/B deletion and ATRX mutation. 50% of PA tumors carried a mutation in the PIK3CA gene which is seemingly associated with better outcome (median OS: PIK3CA mutated 107.5 vs 45.5 months in wildtype PA). This exploratory molecular profiling of spinal cord astrocytomas allows to identify distinct subgroups by combining molecular markers and histomorphology. DMG-H3 tend to develop in adolescence with a similar dismal prognosis like GBM and HAP in the elderly. We here describe spinal HAP with a distinct molecular profile for the first time.
Topics: Adolescent; Adult; Aged; Astrocytoma; Child; Female; High-Throughput Nucleotide Sequencing; Humans; Male; Middle Aged; Molecular Diagnostic Techniques; Retrospective Studies; Spinal Cord Neoplasms; Young Adult
PubMed: 34193285
DOI: 10.1186/s40478-021-01222-6 -
The Spine Journal : Official Journal of... Jul 2023Diffuse gliomas of the spine (DGS)-consisting of intradural intramedullary glioblastoma, astrocytoma, and oligodendroglioma-are exceedingly rare tumors that account for...
BACKGROUND CONTENT
Diffuse gliomas of the spine (DGS)-consisting of intradural intramedullary glioblastoma, astrocytoma, and oligodendroglioma-are exceedingly rare tumors that account for about 2% of primary spinal cord tumors. Much is unknown about their optimal treatment regimen due to a relative lack of clinical outcome data.
PURPOSE
To provide an updated analysis on treatment and outcomes in DGS.
STUDY DESIGN/SETTING
Observational cohort study using The National Cancer Database (NCDB), a multicenter prospectively collected oncology outcomes database. A systematic literature review was also performed to compare the resulting data to previous series.
PATIENT SAMPLE
Patients with histologically confirmed DGS from 2004 to 2018.
OUTCOME MEASURES
Long-term overall survival and short-term 30/90-day postsurgical mortality, 30-day readmission, and prolonged hospital length of stay.
METHODS
Impact of extent of resection and adjuvant therapy on overall survival was evaluated using Kaplan-Meier estimates and multivariable Cox proportional hazards regression. Univariate and multivariate logistic regression was used to analyze covariables and their prognostic impact on short-term surgical outcomes.
RESULTS
Of the 747 cases that met inclusion criteria, there were 439 astrocytomas, 14 oligodendrogliomas, and 208 glioblastomas. Sixty percent (n=442) of patients received radiation, and 45% (n=324) received chemotherapy. Tumor histology significantly impacted survival; glioblastoma had the poorest survival (median survival time [MS]: 12.3 months), followed by astrocytoma (MS: 70.8 months) and oligodendroglioma (MS: 71.6 months) (p<.001). Gross total resection (GTR) independently conferred a survival benefit in patients with glioblastoma (hazard ratio [HR]: 0.194, p<0.001) and other WHO grade four tumors (HR: 0.223, p=.003). Adjuvant chemotherapy also improved survival in patients with glioblastoma (HR: 0.244, p=.007) and WHO grade four tumors (HR: 0.252, p<.001). Systematic literature review identified 14 prior studies with a combined DGS mortality rate of 1.3%, which is lower than the 4% real-world outcomes calculated from the NCDB. This difference may be explained by selection biases in previously published literature in which only centers with favorable outcomes publish their results.
CONCLUSIONS
There remains a paucity of data regarding treatment paradigms and outcomes for DGS. Our analysis, the largest to date, demonstrates that GTR and adjuvant therapy independently improve survival for certain high-grade subgroups of DGS. This best-available data informs optimal management for such patients.
Topics: Humans; Glioblastoma; Oligodendroglioma; Neurosurgical Procedures; Astrocytoma; Prognosis; Retrospective Studies; Observational Studies as Topic; Multicenter Studies as Topic
PubMed: 36804437
DOI: 10.1016/j.spinee.2023.02.010 -
Surgical Neurology Mar 2006Primary diffuse leptomeningeal gliomatosis is an exceptional neoplasm, and only 30 cases have been reported in the literature. We report a recent case and compare data... (Review)
Review
BACKGROUND
Primary diffuse leptomeningeal gliomatosis is an exceptional neoplasm, and only 30 cases have been reported in the literature. We report a recent case and compare data with previously published observations.
METHODS
A 50-year-old man was admitted to the neurosurgery department for a previous 4-month history of headache, associated with nonspecific neurological signs. Biologic data and cerebrospinal fluid examination suggested an inflammatory process. The patient was given an antituberculous therapy. Magnetic resonance imaging revealed a multinodular enhancement of spinal nerve roots. A biopsy of sacral rootlets was performed. Histological examination revealed an anaplastic astrocytoma. Patient's status worsened, and death occurred 7 months later.
RESULTS
Complete neuraxis postmortem examination revealed no intraparenchymatous glioma and was conclusive for the diagnosis of primary leptomeningeal gliomatosis (astrocytic, World Health Organization grade III), with a multinodular pattern in the spinal cord, the brainstem, and the brain base with diffuse extension into the cerebellar subarachnoid spaces.
CONCLUSIONS
Our case illustrates the diagnostic difficulties in making the premortem diagnosis. The review of the literature indicates that there are no specific clinical or biologic signs. Magnetic resonance imaging using T1-weighted images with gadolinium enhancement and biopsy material may be useful diagnostic tools. In most cases, autopsy evaluation alone permits definitive primary diffuse leptomeningeal gliomatosis diagnosis. Whatever the histological characteristics of proliferating cells are, the prognosis remains poor. No prognostic factors have been shown to be correlated with survival time. Unfortunately, no routine treatment has been yet proposed.
Topics: Astrocytoma; Brain; Cerebellum; Diagnosis, Differential; Fatal Outcome; Humans; Intracranial Pressure; Male; Meningeal Neoplasms; Meninges; Middle Aged; Neoplasm Invasiveness; Neoplasms, Neuroepithelial; Neurologic Examination; Peripheral Nervous System Neoplasms; Prognosis; Spinal Nerve Roots
PubMed: 16488248
DOI: 10.1016/j.surneu.2005.06.038 -
Clinical Neuropathology 2014Rosette-forming glioneuronal tumor (WHO grade I) is a rare neoplasm primarily arising in young adults that is characterized by distinctive neurocytic rosette formation,...
Rosette-forming glioneuronal tumor (WHO grade I) is a rare neoplasm primarily arising in young adults that is characterized by distinctive neurocytic rosette formation, a spindled glial component resembling pilocytic astrocytoma, and a high incidence of PIK3CA mutation. Low-grade diffuse astrocytoma (WHO grade II), on the other hand, is far more common and is characterized by a high incidence of IDH mutation. Here we report a patient with simultaneous presentation of a midbrain-cerebellar rosetteforming glioneuronal tumor and a cerebral diffuse astrocytoma. Molecular characterization of both tumors confirmed characteristic, mutually exclusive, distinct signatures, with the rosette-forming glioneuronal tumor exhibiting a previously unreported novel PIK3CA gene mutation.
Topics: Adult; Astrocytoma; Brain; Brain Neoplasms; Cerebral Ventricle Neoplasms; Female; Fourth Ventricle; Ganglioglioma; Humans; Neoplasms, Neuroepithelial; Rosette Formation
PubMed: 24986181
DOI: 10.5414/NP300767