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Journal of Neurosurgery. Pediatrics May 2022Astroblastoma (AB) is a rare glial tumor. The optimal treatment and prognosis of this tumor remain unclear. The authors retrospectively analyzed the clinical...
OBJECTIVE
Astroblastoma (AB) is a rare glial tumor. The optimal treatment and prognosis of this tumor remain unclear. The authors retrospectively analyzed the clinical characteristics, neuroimaging findings, histopathological results, and treatment outcomes of 7 patients with AB.
METHODS
The study comprised 7 patients with pathologically proven AB who were surgically treated at Samsung Medical Center from November 1994 to January 2019. Clinicoradiological, histopathological, and surgical records were reviewed.
RESULTS
The patients included 5 girls (71.4%) and 2 boys (28.6%), with a median age of 13 years. All patients showed contrast enhancement on preoperative MRI: 5 ABs (71.4%) showed a concomitant solid and cystic appearance, and 2 (28.6%) demonstrated a solid appearance. ABs in 6 patients (85.7%) showed a well-circumscribed, characteristic "bubbly" appearance on T2-weighted MRI. Gross-total resection (GTR) was achieved in all cases (100%). Six patients (85.7%) were diagnosed with high-grade AB and 1 (14.3%) with low-grade AB. Six (85.7%) of the 7 patients received adjuvant treatment after resection, including 5 (83.3%) with AB who received chemotherapy and radiotherapy and 1 (16.7%) who received proton therapy alone. The median clinical follow-up duration was 96 months (range 48-189 months). Two patients experienced recurrence, and all patients in this series were alive at the last follow-up.
CONCLUSIONS
In this study, the clinicoradiological and histopathological features of AB were described. Based on the authors' limited experience with 7 cases, resection with the goal of GTR is currently the mainstream treatment for AB, and adjuvant radiation treatment should be considered after surgery.
Topics: Male; Female; Humans; Child; Adolescent; Brain Neoplasms; Retrospective Studies; Treatment Outcome; Neoplasms, Neuroepithelial; Glioma
PubMed: 35180693
DOI: 10.3171/2022.1.PEDS21389 -
Journal of Clinical Neuroscience :... Mar 2016Astroblastoma is a rare tumor of glial origin with characteristics of both astrocytoma and ependymoma. It is usually seen in children and young adults, and is...
Astroblastoma is a rare tumor of glial origin with characteristics of both astrocytoma and ependymoma. It is usually seen in children and young adults, and is peripherally located, well circumscribed, of solid-cystic composition and with heterogeneous contrast enhancement. Histopathology reveals perivascular pseudorosette formation and thick hyalinised vessels. Hemorrhage in astroblastoma is unusual and rarely described in literature. We report two patients with astroblastoma who presented with hemorrhage and discuss the natural history, radiological findings, pathophysiology of hemorrhage and histopathological characteristics. We emphasize the importance of early suspicion in peripherally located lesions with bleeding.
Topics: Adult; Brain Neoplasms; Cerebral Hemorrhage; Child; Female; Humans; Male; Neoplasms, Neuroepithelial
PubMed: 26547295
DOI: 10.1016/j.jocn.2015.05.058 -
Brain Pathology (Zurich, Switzerland) Jul 2000Astroblastomas are uncommon brain tumors whose classification and histogenesis have been debated. Precise criteria for diagnosis have been described only recently, but...
Astroblastomas are uncommon brain tumors whose classification and histogenesis have been debated. Precise criteria for diagnosis have been described only recently, but have not found wide acceptance. We report the clinical, radiographic, and histopathologic features of 20 astroblastomas, and the chromosomal alterations in seven cases as detected by comparative genomic hybridization (CGH). The tumors occurred both in children and young adults (average age, 14 years), most often as well circumscribed, peripheral, cerebral hemispheric masses. Radiographically, the lesions were contrast-enhancing and solid, often with a cystic component. All were characterized histologically by astroblastic pseudorosettes, and most displayed prominent perivascular hyalinization, regional hyaline changes, and pushing borders in regard to adjacent brain. Tumor cells were strongly immunoreactive for S-100 protein, GFAP, and vimentin. Staining for EMA was focal. Ten of 20 astroblastomas were classified as "well differentiated" and 10 were classified as "malignant," largely on the basis of hypercellular zones with increased mitotic indices, vascular proliferation, and necrosis with pseudopalisading. All 10 well differentiated lesions and 8 of 10 malignant lesions were completely resected. None of the well differentiated astroblastomas recurred within the limited follow-up period. Three malignant astroblastomas recurred, including two incompletely resected tumors, and one that had been totally resected. One patient died of disease following recurrence. The most frequent chromosomal alterations detected by CGH were gains of chromosome arm 20q (4/7 tumors) and chromosome 19 (3/7). The combination of these gains occurred in three, including two well differentiated and one malignant astroblastoma. Other alterations noted in two tumors each were losses on 9q, 10, and X. These chromosomal alterations are not typical of ependymoma or infiltrating astrocytic neoplasms, and suggest that astroblastomas may have a characteristic cytogenetic profile in addition to their distinctive clinical, radiographic, and histopathologic features.
Topics: Adolescent; Adult; Brain Neoplasms; Child; Chromosome Aberrations; Chromosome Disorders; Combined Modality Therapy; Female; Follow-Up Studies; Humans; Male; Middle Aged; Neoplasms, Neuroepithelial; Nucleic Acid Hybridization; Radiography
PubMed: 10885653
DOI: 10.1111/j.1750-3639.2000.tb00266.x -
Experimental Cell Research Mar 2017Cell lines established from tumors are the most commonly used models in cancer research, and their use in recent years has enabled a greater understanding of the biology...
Cell lines established from tumors are the most commonly used models in cancer research, and their use in recent years has enabled a greater understanding of the biology of cancer and the means to develop effective treatment strategies. Astroblastomas are uncommon neuroepithelial tumors of glial origin, predominantly affecting young people, mainly teenagers and children, predominantly females. To date, only a single study has reported that astroblastomas contain a large number of neural stem-like cells, which had only a partial proliferation capacity and differentiation. Our objective was to establish an astroblastoma cell line to investigate the presence of astroblastic cells and cancer stem-like cells. The migratory and invasion abilities of the cells were quantified with invasion and migration assays and compared to a glioblastoma cell line. The presence of stem cells was detected with surface-marker analysis by using flow cytometry, and measuring the differentiation ability with a differentiation assay and the self-renewal capacity with a sphere-forming assay. These characteristics may determine whether this novel cell line is a model for astroblastomas that may have stem-cell characteristics. With this novel cell line, scientists can investigate the molecular pathways underlying astroblastomas and develop new therapeutic strategies for patients with these tumors.
Topics: Cell Culture Techniques; Cell Line, Tumor; Cell Separation; Humans; Male; Neoplasms, Neuroepithelial; Neoplastic Stem Cells; Spheroids, Cellular; Tumor Cells, Cultured; Young Adult
PubMed: 28232116
DOI: 10.1016/j.yexcr.2017.02.030 -
QJM : Monthly Journal of the... Jul 2011
Topics: Adult; Astrocytoma; Brain Neoplasms; Headache; Humans; Magnetic Resonance Imaging; Male; Neoplasms, Neuroepithelial; Treatment Outcome
PubMed: 20530537
DOI: 10.1093/qjmed/hcq089 -
Neuropathology : Official Journal of... Feb 2006We report the clinicopathological findings of astroblastoma found in an 8-year-old girl who was subsequently treated for 11 years. The primary superficially...
We report the clinicopathological findings of astroblastoma found in an 8-year-old girl who was subsequently treated for 11 years. The primary superficially circumscribed tumor was located in the frontoparietal lobe, while the recurrent and the second recurrent tumor were restricted to the same region 11 years later. The tumors obtained on these three occasions showed fundamentally the same histological, immunohistochemical and fine structural features. They exhibited astrocytic as well as ependymal tanycytic features with apparent epithelial cell lineage. The tumor cells showed typical features of astroblastoma comprising prominent perivascular pseudorosettes with remarkable vascular sclerosis. The immunohistochemical study revealed intensive positivity of GFAP, vimentin, epithelial membrane antigen (EMA), cytokeratin, connexin 26 and 32, desmocollin 1 and neuronal cadherin. The fine structure revealed divergent types of junctional complexes, some of which were connected with tonofilament bundles. Numerous microvilli protruded and basal lamina abutted on the tumor cell surface. We report these unique histological features, and stress that astroblastoma should be categorized as a specific type of neuroepithelial tumor.
Topics: Biomarkers, Tumor; Brain Neoplasms; Cell Lineage; Child; Diagnosis, Differential; Epithelial Cells; Female; Glioma; Humans; Immunohistochemistry; Magnetic Resonance Imaging; Microscopy, Electron, Transmission; Neoplasms, Neuroepithelial
PubMed: 16521483
DOI: 10.1111/j.1440-1789.2006.00636.x -
Medicina (Kaunas, Lithuania) 2010Gliomatosis cerebri is a rare diffusely infiltrating glial tumor involving two or more lobes and is frequently is bilateral. Infiltrative extent of tumor is out of...
Gliomatosis cerebri is a rare diffusely infiltrating glial tumor involving two or more lobes and is frequently is bilateral. Infiltrative extent of tumor is out of proportion to histological and clinical features. We present a case in which finally the diagnosis of gliomatosis cerebri was made. In this case, computed tomography showed that midline structures were insignificantly shifted to the left, there was a mild dilatation of lateral ventricles more expressed on the right, and no pathologic changes of brain tissue density were found. On magnetic resonance tomography, T2W/SE and T2W/FLAIR images revealed zones of hyperintense signal, spreading with time, through several lobes of the brain with no enhancement on T1W images. Diagnosis of gliomatosis cerebri was suspected, stereotaxic biopsy was performed, and pathological examination revealed changes typical of diffuse glial tumor. In this article, changes typical of gliomatosis cerebri seen in other radiological methods such as computed tomography, magnetic resonance spectroscopy, dynamic contrast-enhanced T2*-weighted magnetic resonance, and positron emission tomography also are discussed.
Topics: Adrenal Cortex Hormones; Biopsy; Brain; Brain Neoplasms; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Neoplasms, Neuroepithelial; Tomography, X-Ray Computed
PubMed: 20679750
DOI: No ID Found -
BMJ Case Reports Sep 2011Astroblastoma is a rarely diagnosed primary brain neoplasm whose histogenesis has been clarified recently. It occurs in children and young adults and presents as a...
Astroblastoma is a rarely diagnosed primary brain neoplasm whose histogenesis has been clarified recently. It occurs in children and young adults and presents as a well-circumscribed, contrast enhancing lesion in the cerebral hemisphere. Here the authors present a case of 25-year-old woman with an astroblastoma in the left frontal convexity that was excised. The characteristic radiological and histopathological features of this case are described. An astroblastoma should be included in the differential of a localised brain tumour, especially in a young patient.
Topics: Adult; Brain Neoplasms; Contrast Media; Diagnosis, Differential; Female; Humans; Magnetic Resonance Imaging; Neoplasms, Neuroepithelial
PubMed: 22679316
DOI: 10.1136/bcr.06.2011.4323 -
Brain Pathology (Zurich, Switzerland) Mar 2018Astroblastoma is a rare and controversial glioma with variable clinical behavior. The diagnosis currently rests on histologic findings of a circumscribed glioma with...
Astroblastoma is a rare and controversial glioma with variable clinical behavior. The diagnosis currently rests on histologic findings of a circumscribed glioma with astroblastomatous pseudorosettes and vascular hyalinization. Immunohistochemical studies have suggested different oncogenic drivers, such as BRAF p.V600E, but very few cases have been studied using genome-wide methodologies. Recent genomic profiling identified a subset of CNS embryonal tumors with astroblastoma-like morphology that harbored MN1 gene fusions, termed "CNS high-grade neuroepithelial tumors with MN1 alteration" (CNS-HGNET-MN1). To further characterize the genetic alterations that drive astroblastomas, we performed targeted next-generation sequencing (NGS) of 500 cancer-associated genes in a series of eight cases. We correlated these findings with break-apart fluorescence in situ hybridization (FISH) analysis of the MN1 locus and genome-wide DNA methylation profiling. Four cases showed MN1 alteration by FISH, including two pediatric cases that lacked other pathogenic alterations, and two adult cases that harbored other cancer-associated gene mutations or copy number alterations (eg, CDKN2A/B homozygous deletion, TP53, ATM and TERT promoter mutations). Three of these cases grouped with the CNS-HGNET-MN1 entity by methylation profiling. Two of four MN1 intact cases by FISH showed genetic features of either anaplastic pleomorphic xanthoastrocytoma (BRAF p.V600E mutation, CDKN2A/B homozygous deletion and TERT promoter mutation) or IDH-wildtype glioblastoma (trisomy 7, monosomy 10, CDK4 amplification and TP53, NRAS and TERT promoter mutations) and these cases had an aggressive clinical course. Two clinically indolent cases remained unclassifiable despite multimodal molecular analysis. We conclude that astroblastoma histology is not specific for any entity including CNS-HGNET-MN1, and that additional genetic characterization should be considered for astroblastomas, as a number of these tumors likely contain a methylation profile or genetic alterations that suggest classification as other tumor entities. Our heterogeneous molecular findings help to explain the clinical unpredictability of astroblastoma.
Topics: Adult; Aged; Brain; Brain Neoplasms; Child; Diagnosis, Differential; Female; Genotyping Techniques; Humans; Immunohistochemistry; Male; Neoplasm Grading; Neoplasms, Neuroepithelial; Phenotype; Retrospective Studies; Trans-Activators; Tumor Suppressor Proteins
PubMed: 28960623
DOI: 10.1111/bpa.12561 -
Neurologia Medico-chirurgica Sep 2006A 17-year-old male presented with morning headache and double vision. Neuroimaging revealed a lobulated enhanced mass lesion with a blurred margin and remarkable...
A 17-year-old male presented with morning headache and double vision. Neuroimaging revealed a lobulated enhanced mass lesion with a blurred margin and remarkable peritumoral edema, and high uptake of methionine. The gray, soft, well-circumscribed mass was grossly totally resected. Histological examination showed the tumor cells were well differentiated with the perivascular pseudorosette pattern with broad, non-tapering processes radiating towards a central vessel without anaplastic features such as necrosis and endothelial proliferation. The histological diagnosis was low-grade astroblastoma. Follow-up magnetic resonance imaging demonstrated local recurrence 5 months later. Second surgery was followed by adjuvant radiotherapy and combination chemotherapy. Histological examination disclosed wide invasion by tumor cells into the subpial and perivascular space of the surrounding brain tissue. Follow-up magnetic resonance imaging demonstrated further recurrence around the tumor cavity. Surgical removal followed by six courses of combination chemotherapy (ifosfamide, cisplatin, and etoposide) resulted in complete remission of the tumor. Although gross total resection of astroblastoma usually results in long-term survival, some of these yet unfamiliar tumors may develop a more malignant character.
Topics: Adolescent; Brain Neoplasms; Chemotherapy, Adjuvant; Humans; Male; Neoplasm Invasiveness; Neoplasm Recurrence, Local; Neoplasms, Neuroepithelial; Radiotherapy, Adjuvant
PubMed: 16998280
DOI: 10.2176/nmc.46.450