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Journal of Neuro-oncology Jun 2016
Topics: Adult; Astrocytoma; Brain; Brain Neoplasms; Demyelinating Diseases; Female; Humans; Isocitrate Dehydrogenase; Tumor Suppressor Protein p53
PubMed: 27033061
DOI: 10.1007/s11060-016-2118-9 -
Journal of Neuro-oncology Sep 2023To report the long-term outcomes in adult patients with grade 2 IDH-mutant astrocytoma treated with temozolomide (TMZ)-based chemoradiation.
PURPOSE
To report the long-term outcomes in adult patients with grade 2 IDH-mutant astrocytoma treated with temozolomide (TMZ)-based chemoradiation.
METHODS
One hundred and three patients with histologically proven grade 2 astrocytoma received radiation therapy (RT), 50.4-54 Gy in 1.8 Gy fractions, and adjuvant TMZ up to 12 cycles. Fifty-two patients received RT at the time of tumor progression and 51 in the early postoperative period for the presence of at least one high-risk feature (age > 40 years, preoperative tumor size > 5 cm, large postoperative residual tumor, tumor crossing the midline, or presence of neurological symptoms). Overall survival (OS) and progression-free survival (PFS) were calculated from the time of diagnosis.
RESULTS
With a median follow-up time of 9.0 years (range, 1.3-15 years), median PFS and OS times were 9 years (95%CI, 6.6-10.3) and 11.8 years (95%CI, 9.3-13.4), respectively. Median PFS was 10.6 years in the early treatment group and 6 years in delayed treatment group (hazard ratio (HR) 0.30; 95%CI 0.16-0.59; p = 0.0005); however, OS was not significantly different between groups (12.8 vs. 10.4 years; HR 0.64; 95%CI 0.33-1.25; p = 0.23). Extent of resection, KPS, and small residual disease were associated with OS, with postoperative tumor ≤ 1 cc that emerged as the strongest independent predictor (HR: 0.27; 95%CI 0.08-0.87; p = 0.01).
CONCLUSIONS
TMZ-based chemoradiation is associated with survival benefit in patients with grade 2 IDH-mutant astrocytoma. For this group of patients, chemoradiation can be deferred until time of progression in younger patients receiving extensive resection, while early treatment should be recommended in high-risk patients.
Topics: Humans; Adult; Temozolomide; Antineoplastic Agents, Alkylating; Dacarbazine; Brain Neoplasms; Astrocytoma; Treatment Outcome
PubMed: 37665475
DOI: 10.1007/s11060-023-04418-z -
Child's Nervous System : ChNS :... Jun 2005Leptomeningeal dissemination of juvenile pilocytic astrocytoma (JPA) is a rare event. We report two children with disseminated JPAs treated with a chemotherapeutic... (Review)
Review
INTRODUCTION
Leptomeningeal dissemination of juvenile pilocytic astrocytoma (JPA) is a rare event. We report two children with disseminated JPAs treated with a chemotherapeutic agent, temozolomide, after progression of the disease despite surgery, traditional chemotherapy, and/or radiation therapy.
CASE REPORTS
Patient 1 presented with hydrocephalus and progressive lower extremity weakness, and was found to have a suprasellar mass as well as extensive spinal disease. Ventriculoperitoneal shunting, decompressive laminectomy with spinal tumor debulking, and chemotherapy with carboplatin and vincristine were initially employed. However, disease progressed and craniospinal irradiation and temozolomide were used. Patient 1 remains in a fair condition today, 2 years later. Patient 2 presented at 8 months of age with failure to thrive. Imaging revealed a cystic lesion in the hypothalamic region with extensive subarachnoid metastatic disease to the spine. Biopsy was performed followed by chemotherapy with vincristine, cyclohexylchloroethylnitrosourea (CCNU), 6-TG, and procarbazine. Due to the continued progression of the disease, cytoreductive surgery was performed and her chemotherapeutic regimen was switched to temozolomide. Two years after initial presentation patient 2 is clinically much improved with stable residual disease.
DISCUSSION
We review the literature and discuss treatment strategies for this challenging disease.
Topics: Antineoplastic Agents, Alkylating; Arachnoid Cysts; Astrocytoma; Brain Neoplasms; Child; Child, Preschool; Dacarbazine; Female; Humans; Magnetic Resonance Imaging; Neurosurgery; Radiotherapy; Temozolomide; Treatment Outcome
PubMed: 15378329
DOI: 10.1007/s00381-004-1002-7 -
Progress in Neurological Surgery 2018The effectiveness of chemotherapy or chemoradiotherapy for diffuse astrocytoma (DA) has been largely unknown until recently. However, a randomized controlled study (RTOG...
The effectiveness of chemotherapy or chemoradiotherapy for diffuse astrocytoma (DA) has been largely unknown until recently. However, a randomized controlled study (RTOG 9802) showed that adding of procarbazine, CCNU, and vincristine (PCV) chemotherapy to fractionated radiotherapy (FRT) in patients with "high-risk" WHO grade II gliomas, including DA, has significant positive impact on both progression-free survival and overall survival. Effectiveness of temozolomide (TMZ) in cases of low-grade gliomas was also reported, and a randomized phase III trial comparing FRT alone or in combination with TMZ in cases of unresectable DA (JCOG 1303) is currently ongoing.
Topics: Adult; Astrocytoma; Brain Neoplasms; Chemotherapy, Adjuvant; Dacarbazine; Glioma; Humans; Procarbazine; Temozolomide
PubMed: 29393182
DOI: 10.1159/000467375 -
Brain Pathology (Zurich, Switzerland) Mar 2019Granular cell astrocytoma (GCA) is a rare adult infiltrating glioma subtype. We studied a series of 39 GCAs. Median age of presentation was 57.8 years and most cases...
Granular cell astrocytoma (GCA) is a rare adult infiltrating glioma subtype. We studied a series of 39 GCAs. Median age of presentation was 57.8 years and most cases developed in the frontal or temporal lobes. Tumors included grade II (n = 14), grade III (n = 11), and grade IV (n = 14) by WHO criteria. Granular cell morphology was diffuse in 31 (79%) cases and partial in eight (21%). Immunohistochemistry showed frequent positivity for GFAP (28 of 31), OLIG2 (16 of 16), and CD68 (27 of 30), but HAM56, CD163, and IBA-1 histiocytic markers were all negative (22 of 22). IDH1(R132H) was negative in all the cases tested (16 of 16), while ATRX expression was retained (12 of 12). Cytogenetics demonstrated monosomy 10 (6 of 6) cases, +7 in 4 (of 6), -13q in 4 of 6, and -14 in 4 of 6. Next-generation sequencing demonstrated mutations in PTEN/PIK3 genes in 6/13 (46%), NF1 in 3 of 10 (30%), TP53 in 3 of 13 (23%), PALB2 in 3 of 10 (30%), STAG2 in 3 of 10 (30%), EGFR mutation/amplification in 3 of 13 (23%), and AR in 2 of 10 (20%). CDKN2A/B deletion was identified in 5 of 13 (30%) cases (homozygous deletion in 4). The TERT C228T mutation was identified in 9 of 13 (69%). No mutations were encountered in IDH1, IDH2, CIC, FUBP1, H3F3A, BRAF or ATRX genes. The mean overall survival was 11.3 months. Patients >60 years old at diagnosis had a worse survival than patients <60 years (P = 0.001). There were no statistically significant differences in survival by WHO grade, extent of granular cell change, sex or MIB-1 (P > 0.05). GCA is a variant of IDH-wildtype diffuse glioma with aggressive behavior irrespective of grade and extent of granular cell morphology, and with molecular genetic features corresponding to primary glioblastoma.
Topics: Adult; Aged; Aged, 80 and over; Astrocytoma; Biomarkers, Tumor; Brain Neoplasms; Female; Glioblastoma; Glioma; Granular Cell Tumor; High-Throughput Nucleotide Sequencing; Humans; Immunohistochemistry; Isocitrate Dehydrogenase; Kaplan-Meier Estimate; Male; Middle Aged; Mutation; Promoter Regions, Genetic
PubMed: 30222900
DOI: 10.1111/bpa.12657 -
Journal of Neuro-oncology Sep 2007We present a 68-year-old woman who presented with symptoms of frontotemporal dementia. Brain MRI revealed tumor mass in both thalami and according to WHO classification,...
We present a 68-year-old woman who presented with symptoms of frontotemporal dementia. Brain MRI revealed tumor mass in both thalami and according to WHO classification, the tumor corresponded to diffuse fibrillary astrocytoma grade II. This case points to the role of neuroimaging in patients presenting with classical symptoms of dementia.
Topics: Aged; Alzheimer Disease; Astrocytoma; Brain Neoplasms; Dementia; Diagnosis, Differential; Female; Humans; Magnetic Resonance Imaging; Thalamus
PubMed: 17522784
DOI: 10.1007/s11060-007-9404-5 -
Neurologia Medico-chirurgica 2015We report the case of a 58-year-old woman with low-grade astrocytoma, who developed massive intracranial hemorrhage as the first presentation of this disease, and become...
We report the case of a 58-year-old woman with low-grade astrocytoma, who developed massive intracranial hemorrhage as the first presentation of this disease, and become comatose and subsequently underwent an emergency craniotomy. A small amount of tumor-like tissue was observed on the wall of the hematoma cavity. Histological analysis of the resected specimen indicated diffuse astrocytoma [World Health Organization (WHO) grade II]. The patient was discharged without neurological deficits 2 weeks after the operation. A non-enhanced tumor-like nodule was observed on magnetic resonance imaging 3 months after the operation, which was monitored carefully but was not treated by adjuvant therapy. The tumor grew gradually, and a second operation was performed 3 years after the first, in which the tumor was completely resected. Histological analysis of the resected specimen again indicated diffuse astrocytoma (WHO grade II). Although rare, brain tumors, including low-grade astrocytoma, should be considered a possible cause of subcortical hemorrhage in patients without risk factors for intracranial hemorrhage.
Topics: Astrocytoma; Brain Neoplasms; Cerebral Hemorrhage; Female; Humans; Middle Aged; Neoplasm Grading; Risk Factors
PubMed: 24418786
DOI: 10.2176/nmc.cr.2013-0177 -
Cells Jun 2022Glioblastoma (GBM, grade IV astrocytoma), the most frequently occurring primary brain tumor, presents unique challenges to therapy due to its location, aggressive...
Glioblastoma (GBM, grade IV astrocytoma), the most frequently occurring primary brain tumor, presents unique challenges to therapy due to its location, aggressive biological behavior, and diffuse infiltrative growth, thus contributing to having disproportionately high morbidity and mortality [...].
Topics: Astrocytoma; Brain Neoplasms; Glioblastoma; Humans; Molecular Biology
PubMed: 35681545
DOI: 10.3390/cells11111850 -
Journal of Neuro-oncology Mar 2021In 2018, cIMPACT-NOW update 3 concluded that WHO grade II/III IDH-wildtype diffuse astrocytomas that contain TERT promoter mutations, chromosome 7 gain/10 loss, and/or... (Review)
Review
OBJECTIVE
In 2018, cIMPACT-NOW update 3 concluded that WHO grade II/III IDH-wildtype diffuse astrocytomas that contain TERT promoter mutations, chromosome 7 gain/10 loss, and/or EGFR amplification, correspond to a WHO grade IV diagnosis and should be classified as Diffuse astrocytic glioma, IDH-wildtype, with molecular features of glioblastoma, WHO grade IV (DAG-G). We present a single-institution series of patients with DAG-G and IDH-mutant astrocytomas and compare their clinical, molecular, and radiographic characteristics.
METHODS
Patient data was retrospectively extracted from the EMR for all patients undergoing surgical biopsy/resection of a diffuse astrocytoma at our institution from 2018 to 2020. Clinical presentation, molecular alterations, radiographic appearance, surgery, and survival were reviewed for each patient.
RESULTS
Six DAG-G patients were identified in our cohort. All patients had diffuse disease, and presented with expansile, T2 hyperintense lesions with minimal enhancement. Compared to patients with classic IDH-mutant astrocytomas, mean age for DAG-G patients was older (68 vs 33 years, p < 0.0001), tumors were more diffuse (p = 0.02), with patients more likely to present with focal deficits and receive a biopsy only (p = 0.005). Overall survival was significantly shorter for DAG-G patients (p = 0.03).
CONCLUSION
Patients with DAG-G are more likely to be older than typical IDH-mutant diffuse astrocytoma patients. They are more likely to present with tumors in a diffuse pattern with focal deficits. When such patients are encountered, prompt biopsy/resection to confirm the diagnosis and immediate initiation of adjuvant therapy is recommended, as the disease progression and overall prognosis is similar to glioblastoma.
Topics: Aged; Aged, 80 and over; Astrocytoma; Biomarkers, Tumor; Brain Neoplasms; Female; Humans; Isocitrate Dehydrogenase; Male; Middle Aged; Retrospective Studies
PubMed: 33389563
DOI: 10.1007/s11060-020-03677-4 -
Acta Neuropathologica Aug 2022
Topics: Astrocytoma; Encephalitis; Humans; Proto-Oncogene Proteins; Proto-Oncogene Proteins c-myb; Receptors, N-Methyl-D-Aspartate; Trans-Activators
PubMed: 35727368
DOI: 10.1007/s00401-022-02447-y