-
AJNR. American Journal of Neuroradiology Apr 2023Astroblastoma is a rare astrocytic glial neoplasm that affects mainly young girls, peaking between 10 and 30 years of age, with low- and high-grade manifestations....
Astroblastoma is a rare astrocytic glial neoplasm that affects mainly young girls, peaking between 10 and 30 years of age, with low- and high-grade manifestations. Imaging characteristics are well-described, but histopathologic and, more recently, molecular analysis is fundamental to establish the diagnosis, now based on alterations. We describe a case with typical imaging and histologic features of an -altered astroblastoma.
Topics: Female; Humans; Brain Neoplasms; Neoplasms, Neuroepithelial; Glioma; Astrocytoma; Radiology
PubMed: 36958802
DOI: 10.3174/ajnr.A7824 -
Ryoikibetsu Shokogun Shirizu 2000
Review
Topics: Adult; Brain Neoplasms; Child; Child, Preschool; Humans; Neoplasms, Neuroepithelial
PubMed: 11043181
DOI: No ID Found -
Brain and Nerve = Shinkei Kenkyu No... Apr 2022Astroblastoma is an extremely rare primary brain tumor accounting for 0.45 to 2.8% of all neuroglial tumors and usually occurs in pediatrics and young adults. The...
Astroblastoma is an extremely rare primary brain tumor accounting for 0.45 to 2.8% of all neuroglial tumors and usually occurs in pediatrics and young adults. The natural history of astroblastoma still remains unknown. In the World Health Organization (WHO) classification of tumors of the central nervous system, astroblastoma is classified as other neuroepithelial tumors and standard treatment other than surgery has not been established. As molecular and genetic diagnosis becomes more important in the latest WHO classification of brain tumors, the development of therapeutic options based on the information of molecular genetics are expected. Here we report a case of astroblastoma in a 49-year-old male. Small tumor was discovered by coincidence during his check-up following traffic accident, but three months later, tumor bleeding with cystic enlargement resulted in disturbance of consciousness. Initial diagnosis of low grade astroblastoma with BRAF mutation was made. After 1 year, local tumor recurrence was observed. The histological diagnosis at recurrence was high grade astroblastoma. We here, discuss about diagnosis, treatment and the possibility of usefulness of molecular genetic analysis for astroblastoma with some literature review. (Received 10 August, 2021; Accepted 15 December, 2021; Published 1 April, 2022).
Topics: Adult; Brain Neoplasms; Child; Cysts; Glioma; Hemorrhage; Humans; Male; Middle Aged; Neoplasms, Neuroepithelial; Young Adult
PubMed: 35437291
DOI: 10.11477/mf.1416202049 -
Neuropathology : Official Journal of... Oct 2018A 41-year-old man presented to us with left arm and leg weakness and mild word finding difficulties. His preoperative magnetic resonance imaging (MRI) demonstrated... (Review)
Review
A 41-year-old man presented to us with left arm and leg weakness and mild word finding difficulties. His preoperative magnetic resonance imaging (MRI) demonstrated abnormal T1 and T2 signal changes in the right temporal lobe and basal ganglia, indicative of possible glioma. An awake craniotomy for right temporal lobectomy was performed and the tumor was resected. Full pathologic workup later revealed the patient had two distinct tumors occurring simultaneously, anaplastic astrocytoma and astroblastoma. We review the literature regarding the treatment of anaplastic astrocytoma and astroblastoma and discuss their co-occurrence.
Topics: Adult; Astrocytoma; Brain Neoplasms; Humans; Male; Neoplasms, Multiple Primary; Neoplasms, Neuroepithelial
PubMed: 29939429
DOI: 10.1111/neup.12483 -
Journal of Neuro-oncology Jul 2009Astroblastoma is one of the rarest tumors of the central nervous system (CNS), and its classification, histogenesis, diagnosis and therapeutic management are still being... (Review)
Review
Astroblastoma is one of the rarest tumors of the central nervous system (CNS), and its classification, histogenesis, diagnosis and therapeutic management are still being debated. The typical histopathological appearance is the perivascular, astroblastic pseudorosette, which is however present in other CNS tumors. To clarify the clinical, radiological, histopathological, prognostic and therapeutic characteristics, which have been treated only recently and are not well established yet due to the rarity of this tumor, six cases of histologically proven astroblastoma were retrospectively analyzed in light of more pertinent literature and paying special attention to therapeutic remarks. Between 1996 and 2005, six patients with cerebral astroblastoma were surgically treated at the Department of Neurosciences-Neurosurgery of Sapienza University in Rome. In three cases the lesion was termed low-grade astroblastoma, and high grade in the other three, according to current standard parameters. Median age of the six patients was 36 years. The time to diagnosis ranged from 1 week to 18 months. The radiological and anatomopathological features of this lesion are described. Surgical removal was total in four cases and subtotal in two. All patients received radiotherapy: two also had chemotherapy with temozolomide (TMZ). The three patients with low-grade astroblastoma are still alive today after a follow-up of 2, 5 and 19 years, respectively. Of the three patients with high-grade lesions, one is still alive after a 7-year follow-up, while the other two survived for 17 months (progression time 15 months) and 35 months (progression-reoperation time 23 months), respectively. Conclusions radical surgical resection is the treatment of choice for astroblastomas. Radiotherapy may play an adjuvant role in the treatment of high-grade lesions. The role of chemotherapy is still very debatable. We propose an aggressive standardized treatment for those lesions that meet anaplastic criteria, owing to their postulated glial origin and the propensity to have aggressive courses, and we advocate the use of a safe adjuvant chemotherapeutic regimen with TMZ, used concomitantly and subsequently to radiotherapy, especially for the high-grade astroblastoma cases. Multicenter studies, taking into account molecular biological findings, are necessary to define a common therapeutic strategy for astroblastomas.
Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Brain Neoplasms; Combined Modality Therapy; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Neoplasms, Neuroepithelial; Neurosurgical Procedures; Radiotherapy
PubMed: 19214707
DOI: 10.1007/s11060-008-9789-9 -
Journal of Neuro-oncology Feb 2023Review of the clinicopathologic and genetic features of early ependymal tumor with MN1-BEND2 fusion (EET MN1-BEND2), classical astroblastomas, and recently described... (Review)
Review
PURPOSE
Review of the clinicopathologic and genetic features of early ependymal tumor with MN1-BEND2 fusion (EET MN1-BEND2), classical astroblastomas, and recently described related pediatric CNS tumors. I also briefly review general mechanisms of gene expression silencing by DNA methylation and chromatin remodeling, and genomic DNA methylation profiling as a powerful new tool for CNS tumor classification.
METHODS
Literature review and illustration of tumor histopathologic features and prenatal gene expression timelines.
RESULTS
Astroblastoma, originally descried by Bailey and Cushing in 1926, has been an enigmatic tumor. Whether they are of ependymal or astrocytic derivation was argued for decades. Recent genetic evidence supports existence of both ependymal and astrocytic astroblastoma-like tumors. Studies have shown that tumors exhibiting astroblastoma-like histology can be classified into discrete entities based on their genomic DNA methylation profiles, gene expression, and in some cases, the presence of unique gene fusions. One such tumor, EET MN1-BEND2 occurs mostly in female children, and has an overall very good prognosis with surgical management. It contains a gene fusion comprised of portions of the MN1 gene at chromosomal location 22q12.1 and the BEND2 gene at Xp22.13. Other emerging pediatric CNS tumor entities demonstrating ependymal or astroblastoma-like histological features also harbor gene fusions involving chromosome X, 11q22 and 22q12 breakpoint regions.
CONCLUSIONS
Genomic DNA profiling has facilitated discovery of several new CNS tumor entities, however, traditional methods, such as immunohistochemistry, DNA or RNA sequencing, and cytogenetic studies, including fluorescence in situ hybridization, remain necessary for their accurate biological classification and diagnosis.
Topics: Child; Female; Humans; Brain Neoplasms; Central Nervous System Neoplasms; Glioma; In Situ Hybridization, Fluorescence; Neoplasms, Neuroepithelial; Prognosis; Supratentorial Neoplasms; Trans-Activators; Tumor Suppressor Proteins
PubMed: 36604386
DOI: 10.1007/s11060-022-04222-1 -
Pediatric Neurosurgery 2015Astroblastomas are rare intracranial tumours of uncertain origin and are commonly present in the supratentorial compartment. Astroblastomas often affect children and... (Review)
Review
Astroblastomas are rare intracranial tumours of uncertain origin and are commonly present in the supratentorial compartment. Astroblastomas often affect children and young adults. Their occurrence in an intraventricular location, in an infant, is extremely uncommon. We describe a 1-year-old boy who presented with features of raised intracranial pressure. An MRI scan revealed an intralateral ventricular tumour. Complete excision was performed. Its histopathological examination revealed features consistent with astroblastoma. A follow-up MRI scan after 9 months did not show any residual tumour, and the patient was followed up without any adjuvant therapy. The radiological and histopathological characteristics of this tumour are described in our report.
Topics: Cerebral Ventricle Neoplasms; Humans; Infant; Male; Neoplasms, Neuroepithelial
PubMed: 26406441
DOI: 10.1159/000369032 -
Clinical Neuropathology 2015Astroblastoma is a rare brain tumor occurring in children and adults, rarely in the elderly. It constitutes up to 3% of all brain tumors. We report a case of a... (Review)
Review
Astroblastoma is a rare brain tumor occurring in children and adults, rarely in the elderly. It constitutes up to 3% of all brain tumors. We report a case of a 14-year-old girl who presented with recurrent seizures and minimal right hemiparesis. Magnetic resonance imaging (MRI) revealed a left fronto-parietal brain tumor. It was managed with subtotal resection in a local hospital. Subsequently, she was referred to Princess Nora Oncology Center for further characterization and management. Pathology slide revision revealed well-differentiated astroblastoma. Upon follow up, the patient had multiple recurrences of the same tumor and emergence of a new lesion at the area of Sylvian fissure. Excision of the emerging tumor revealed anaplastic astroblastoma. Astroblastoma is a glial tumor that predominantly affects females. Its clinical progression is unpredictable, with high recurrence rate. Surgical intervention is considered the mainstay of treatment, while radiotherapy and chemotherapy effectiveness is debatable. To our knowledge, this is the first reported case of well-differentiated and anaplastic astroblastoma as two separate neoplastic lesions in the same patient with its clinical, radiological, and pathological features.
Topics: Adolescent; Biomarkers, Tumor; Brain Neoplasms; Female; Glioma; Humans; Magnetic Resonance Imaging; Neoplasms, Neuroepithelial; Treatment Outcome
PubMed: 26227256
DOI: 10.5414/NP300864 -
Pediatric Neurosurgery 2012Astroblastoma is a rare glial tumor with uncertain histopathological origin and unpredictable clinical behavior. We present a case of an 11-year-old girl who presented... (Review)
Review
Astroblastoma is a rare glial tumor with uncertain histopathological origin and unpredictable clinical behavior. We present a case of an 11-year-old girl who presented with headache and blurring of vision for 2 months. A well-demarcated mass was found in the right frontoparietal lobe on a brain MRI. The patient was treated with total tumor resection followed by postoperative radiotherapy. Histologically, the features were suggestive of high-grade astroblastoma. The patient is alive and disease free 23 months after surgery. The characteristic radiological and histopathological features and treatment of this case are described with a literature review.
Topics: Biopsy; Brain Neoplasms; Child; Female; Humans; Magnetic Resonance Imaging; Neoplasms, Neuroepithelial; Radiotherapy
PubMed: 23038221
DOI: 10.1159/000342538 -
Singapore Medical Journal Jan 2016
Topics: Adult; Biopsy; Cerebral Ventricle Neoplasms; Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Male; Neoplasms, Neuroepithelial; Third Ventricle
PubMed: 26831319
DOI: 10.11622/smedj.2016013