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Child's Nervous System : ChNS :... Sep 2009Astroblastoma is an unusual brain tumor in childhood. Tumor usually arises from cerebral hemispheres. This large tumor is well-demarcated, lobulated, solid or cystic,...
OBJECTIVE
Astroblastoma is an unusual brain tumor in childhood. Tumor usually arises from cerebral hemispheres. This large tumor is well-demarcated, lobulated, solid or cystic, and therefore, may resemble glioblastoma. Histopathologically, there are two types of astroblastoma: well-differentiated (low-grade) or anaplastic (high-grade). In low-grade astroblastoma, treatment of choice is complete excision. This type of tumor usually does not recur. However, anaplastic tumors can recur despite surgery, radiation, and chemotherapy and may be problematic for clinician.
CASE REPORT
A 7-year-old female patient presented with an acute onset of vomiting and seizure. Magnetic resonance imaging study revealed a large mass in the left parieto-occipital region. She underwent total excision of the tumor. Histopathologically, the tumor was an anaplastic astroblastoma. Her adjuvant treatment was planned to consist of radiation therapy and cisplatin-based chemotherapy. However, the tumor recurred early in the course, and she died 18 months after diagnosis.
CONCLUSION
High-grade astroblastomas behave like glioblastoma, as emphasized in this case report. Local control of this type of tumor seems difficult despite surgery, radiation therapy, and cisplatin-based chemotherapy.
Topics: Brain; Brain Neoplasms; Child; Fatal Outcome; Female; Humans; Magnetic Resonance Imaging; Neoplasm Recurrence, Local; Neoplasms, Neuroepithelial
PubMed: 19357852
DOI: 10.1007/s00381-009-0885-8 -
The Oncologist Dec 2019Astroblastoma (ABM) is a rare glial brain tumor. Recurrent meningioma 1 (MN1) alterations have been recently identified in most pediatric cases. Adolescent and adult...
BACKGROUND
Astroblastoma (ABM) is a rare glial brain tumor. Recurrent meningioma 1 (MN1) alterations have been recently identified in most pediatric cases. Adolescent and adult cases, however, remain molecularly poorly defined.
MATERIALS AND METHODS
We performed clinical and molecular characterization of a retrospective cohort of 14 adult and 1 adolescent ABM.
RESULTS
Strikingly, we found that MN1 fusions are a rare event in this age group (1/15). Using methylation profiling and targeted sequencing, most cases were reclassified as either pleomorphic xanthoastrocytomas (PXA)-like or high-grade glioma (HGG)-like. PXA-like ABM show mutation (6/7 with V600E mutation and 1/7 with G466E mutation) and CD34 expression. Conversely, HGG-like ABM harbored specific alterations of diffuse midline glioma (2/5) or glioblastoma (GBM; 3/5). These latter patients showed an unfavorable clinical course with significantly shorter overall survival ( = .021). Mitogen-activated protein kinase pathway alterations (including fusion, and mutations) were present in 10 of 15 patients and overrepresented in the HGG-like group (3/5) compared with previously reported prevalence of these alterations in GBM and diffuse midline glioma.
CONCLUSION
We suggest that gliomas with astroblastic features include a variety of molecularly sharply defined entities. Adult ABM harboring molecular features of PXA and HGG should be reclassified. Central nervous system high-grade neuroepithelial tumors with alterations and histology of ABM appear to be uncommon in adults. Astroblastic morphology in adults should thus prompt thorough molecular investigation aiming at a clear histomolecular diagnosis and identifying actionable drug targets, especially in the mitogen-activated protein kinase pathway.
IMPLICATIONS FOR PRACTICE
Astroblastoma (ABM) remains a poorly defined and controversial entity. Although meningioma 1 alterations seem to define a large subset of pediatric cases, adult cases remain molecularly poorly defined. This comprehensive molecular characterization of 1 adolescent and 14 adult ABM revealed that adult ABM histology comprises several molecularly defined entities, which explains clinical diversity and identifies actionable targets. Namely, pleomorphic xanthoastrocytoma-like ABM cases show a favorable prognosis whereas high-grade glioma (glioblastoma and diffuse midline gliome)-like ABM show significantly worse clinical courses. These results call for in-depth molecular analysis of adult gliomas with astroblastic features for diagnostic and therapeutic purposes.
Topics: Adult; Aged; Brain Neoplasms; Female; Humans; Male; Middle Aged; Mitogen-Activated Protein Kinases; Neoplasms, Neuroepithelial; Young Adult
PubMed: 31346129
DOI: 10.1634/theoncologist.2019-0223 -
Computerized Medical Imaging and... 2002A patient with grade II astroblastoma is reported. The tumor revealed an inhomogenous enhancement pattern on T1-weighted images after contrast medium. It contained...
A patient with grade II astroblastoma is reported. The tumor revealed an inhomogenous enhancement pattern on T1-weighted images after contrast medium. It contained cyst-like structures with high signal in FLAIR images, consistent with presence of viscous material. In spectroscopy, NAA was decreased, and Cho was increased. Peaks belonging to lipids and a combined broad peak belonging to myoinositol and glycine were noticed. In diffusion MRI, the mean apparent diffusion coefficient (ADC) value of intratumoral cyst-like structures was 2.12 x 10(-3)mm(2)/s less than that of CSF. The matrix of the tumor had lower ADC values ranging from 1.19 to 1.25 x 10(-3)mm(2)/s, higher than that of normal cerebral parenchyma.
Topics: Brain Neoplasms; Child, Preschool; Echo-Planar Imaging; Female; Humans; Magnetic Resonance Spectroscopy; Neoplasms, Neuroepithelial
PubMed: 11918982
DOI: 10.1016/s0895-6111(01)00042-8 -
Surgical Neurology Mar 2007Astroblastoma is one of the most unusual types of tumors whose histogenesis has been recently clarified. It occurs mainly among children and young adults. It is grossly...
BACKGROUND
Astroblastoma is one of the most unusual types of tumors whose histogenesis has been recently clarified. It occurs mainly among children and young adults. It is grossly well demarcated and shows histologically characteristic perivascular pseudorosettes with frequent vascular hyalinization. Immunohistochemically, the tumor cells show diffuse strong positivity for GFAP, S-100 protein, vimentin, as well as neuron-specific enolase and focal positivity for EMA.
CASE DESCRIPTION
Our patient is a 33-year-old gentleman who presented with intraparenchymal hemorrhage in the left temporal lobe. A 4-vessel cerebral angiogram revealed a dural AVF in the left posterior temporal area. The lesion was resected, and pathology showed a GFAP-positive lesion with prominent astroblastoma rosettes. There was abundant hemosiderin consistent with a prior hemorrhage. Because of its high degree of proliferation, the presence of astroblastic pseudorosettes, prominent perivascular hyalinization, regional hyaline changes, and pushing borders with regard to the adjacent brain, the tumor was considered anaplastic.
CONCLUSIONS
Astroblastoma is a rare pure pathologic entity--a distinct form of astrocytic gliomas. The diagnosis of astroblastoma is often difficult because of the astroblastic aspects that can be found in astrocytic tumors, in ependymomas, and in nonneuroepithelial tumors. The natural history seems to be between astrocytomas and glioblastomas. Total resection and radiation therapy seem to be the advisable treatments.
Topics: Adult; Central Nervous System Vascular Malformations; Cerebral Angiography; Cerebral Hemorrhage; Diagnosis, Differential; Diagnostic Errors; Humans; Magnetic Resonance Imaging; Male; Neoplasm Staging; Neoplasms, Neuroepithelial
PubMed: 17320647
DOI: 10.1016/j.surneu.2006.05.050 -
Neurocirugia (Asturias, Spain) Oct 2002We report a new case of low grade astroblastoma of pure type and consider the definition and the clinical, neuroimaging, intraoperative and pathological characteristics... (Review)
Review
We report a new case of low grade astroblastoma of pure type and consider the definition and the clinical, neuroimaging, intraoperative and pathological characteristics of this type of uncommon intracranial tumour. We analyse the differences found in the literature concerning the immunochemistry, genetics and electron microscopy. We point out the lack of protocols for treatment of this type of intracranial tumour, and comment the various hypothesis of its origin.
Topics: Adolescent; Brain Neoplasms; Female; Humans; Magnetic Resonance Imaging; Neoplasms, Neuroepithelial; Tomography, X-Ray Computed
PubMed: 12444409
DOI: 10.1016/s1130-1473(02)70591-5 -
International Journal of Clinical and... 2015We present a rare case of low-grade astroblastoma coexisting with an arteriovenous malformation (AVM) underwent surgery two times in a 38-year-old man. After the first...
We present a rare case of low-grade astroblastoma coexisting with an arteriovenous malformation (AVM) underwent surgery two times in a 38-year-old man. After the first surgery, this case was reported as a mixed low-grade astroblastoma and AVM. The lesion was completely resected surgically along with AVM. The patient underwent postoperative radiotherapy. Twenty months later, MRI showed enhanced lesions in suprasellar, pineal region and multiple small lesions in the spinal cord, whereas completely no recurrent lesion at the primary tumor site. So, the patient rationally underwent surgical removal in suprasellar and pineal region. After the second surgery, this case was diagnosed as a high-grade astroblastoma. Cells from the second surgical specimens showed high MIB-1 index and an increased olig-2 index. In addition, it is not common for low-grade astroblastoma metastasis to suprasellar, pineal region and spine with completely no recurrence at the original primary tumor site. Therefore it is difficult to predict tumor behavior and patient's clinical outcome merely based on histologic features. The important issue is whether the AVM was thought to be the cause of poor progress of this tumor. More cases are needed to confirm this. Classification and histogenesis of this tumor is still debated. Lack of clinicopathological correlation makes the prognosis of this tumor unpredictable. Anyway, we should be very discreet to treat the astroblastoma, even for low-grade astroblastoma.
Topics: Adult; Arteriovenous Fistula; Brain Neoplasms; Humans; Intracranial Arteriovenous Malformations; Male; Neoplasms, Neuroepithelial; Pinealoma; Spinal Cord Neoplasms
PubMed: 26261652
DOI: No ID Found -
Pathology, Research and Practice 2008Astroblastoma is a historically traded microscopic diagnosis to denote a rare neuroepithelial tumor of uncertain nosology, involving a distinctive pattern of...
Astroblastoma is a historically traded microscopic diagnosis to denote a rare neuroepithelial tumor of uncertain nosology, involving a distinctive pattern of pseudorosette arrangement of neoplastic cells. While displaying some glial properties, the latter shall not - by definition - be either reducible to or part of any conventional glioma type. We report on clinicopathologic correlations in a case of astroblastoma involving an extensive rhabdoid phenotype of tumor cells. The male patient was operated on at the age of 53 and 59 years for a left parietal tumor measuring 5.8 cm in diameter at the first presentation. On magnetic resonance imaging and angiography, both the primary and its recurrence were discrete, highly vascularized, and contrast-enhancing. The second surgery was complemented with radiotherapy of 66 Gy, followed by chemotherapy with Temozolomide. Twelve years into clinical history, the patient has stable minimal residual disease at the age of 65. A review of pathology samples from both surgeries showed well-differentiated astroblastoma according to current standards, with an MIB-1 labeling index of 1% and 4%, respectively. Neither of the specimens involved cellular anaplasia, overt mitotic activity, microvascular proliferation, or palisading necrosis. Most tumor cells harbored paranuclear filamentous rhabdoid inclusions that were immunostained for vimentin and, in part, also for GFAP. No polyantigenic reactivity was observed. This example contributes another facet to the spectrum of the so-called composite rhabdoid tumors. Involving a low-grade parent neoplasm, it also further substantiates the incipient perception that the rhabdoid phenotype neither is a peculiar but nonspecific convergence point of anaplastic evolution, nor are such lesions indiscriminately bound for a relentless course.
Topics: Antibodies, Antinuclear; Antibodies, Monoclonal; Antineoplastic Agents, Alkylating; Brain Neoplasms; Cell Differentiation; Cerebral Angiography; Chemotherapy, Adjuvant; Craniotomy; Dacarbazine; Glial Fibrillary Acidic Protein; Humans; Ki-67 Antigen; Magnetic Resonance Imaging; Male; Middle Aged; Neoplasm Recurrence, Local; Neoplasms, Neuroepithelial; Parietal Lobe; Radiotherapy, Adjuvant; Reoperation; Rhabdoid Tumor; Temozolomide; Time Factors; Treatment Outcome; Vimentin
PubMed: 18280055
DOI: 10.1016/j.prp.2007.12.003 -
Neuropathology : Official Journal of... Jun 2013Astroblastoma is a rare glial tumor of unknown origin, usually affecting the cerebral hemispheres of children and young adults. Here we report an unusual cerebral tumor...
Astroblastoma is a rare glial tumor of unknown origin, usually affecting the cerebral hemispheres of children and young adults. Here we report an unusual cerebral tumor in a 60-year-old woman. On MRI, the tumor appeared as a well circumscribed lesion in the left frontal lobe. Histopathologically, it was composed of rounded eosinophilic cells, and was divisible into two areas. One area was characterized by a collection of GFAP-positive cells around sclerotic blood vessels (astroblastic pseudorosettes and perivascular hyalinization), and had a Ki-67 labeling index of 2.8%. However, the other area was highly cellular, showing many GFAP-negative cells often with a rhabdoid appearance, mitoses and a Ki-67 index of 15.7%. Thus, a final diagnosis of malignant astroblastoma was made. In both areas of the tumor, nearly all the cells were positive for epithelial membrane antigen, and many were positive for oligodendrocyte transcription factor 2 (Olig2). Focal expression of cytokeratin was also evident. With regard to genetic markers, the tumor cells were positive for INI1 and negative for mutant IDH1. The p53 labeling index was <1%. Ultrastructurally, the presence of intra- and intercellular lumina with microvilli was a feature. DNA examination of IDH1/2 and TP53 showed no mutations. In conclusion, although ependymal features were evident ultrastructurally in the present tumor, the immunohistochemical expression pattern of Olig2 was that of diffuse astrocytoma. On the other hand, the absence of mutations in both IDH1/2 and TP53 suggested that the present tumor was not a purely astrocytic neoplasm. Further studies, including molecular and genetic analyses, will provide insight into the histogenesis of astroblastoma.
Topics: Brain Neoplasms; DNA; Female; Humans; Immunohistochemistry; Isocitrate Dehydrogenase; Magnetic Resonance Imaging; Middle Aged; Neoplasms, Neuroepithelial; Neurosurgical Procedures; Tomography, X-Ray Computed; Tumor Suppressor Protein p53
PubMed: 22994361
DOI: 10.1111/j.1440-1789.2012.01351.x -
CNS Oncology Apr 2018To evaluate the use of chemotherapy and radiation, and their outcomes for patients with astroblastoma.
AIM
To evaluate the use of chemotherapy and radiation, and their outcomes for patients with astroblastoma.
PATIENTS & METHODS
This is a retrospective review of patients extracted from the National Cancer Database. We investigated overall survival (OS) using Kaplan-Meier curves. Cox proportional hazards models were used to correlate OS with risk variables and treatments.
RESULTS
OS at 5 years was 79.5%. Patients with high-grade tumors were more likely to receive chemotherapy and radiation. Patients with high-grade astroblastoma who did not receive adjuvant radiation had poor survival.
CONCLUSION
Patients with astroblastoma should be treated with curative intent. Radiation is likely beneficial in high-grade astroblastoma. The exact role of radiation and chemotherapy following surgical resection warrant further investigation.
Topics: Adolescent; Adult; Brain Neoplasms; Child; Child, Preschool; Follow-Up Studies; Humans; Infant; Infant, Newborn; Neoplasm Grading; Neoplasms, Neuroepithelial; Retrospective Studies; Survival Analysis; Treatment Outcome; Young Adult
PubMed: 29708401
DOI: 10.2217/cns-2017-0038 -
Neurosurgery Mar 2007Astroblastomas are rare glial neoplasms that usually occur in young adults and have a predilection for the cerebral hemispheres. Patients typically present with signs of...
OBJECTIVE
Astroblastomas are rare glial neoplasms that usually occur in young adults and have a predilection for the cerebral hemispheres. Patients typically present with signs of increased intracranial pressure and seizures. Imaging studies reveal circumscribed, contrast-enhancing tumors that contain both cystic and solid components with variable peritumoral edema. Hemorrhage, which suggested the presence of a vascular lesion in this patient, has not been previously described as a feature of this neoplasm.
CLINICAL PRESENTATION
The authors report the case of a 33-year-old woman who presented with spontaneous intraparenchymal hemorrhage. The collective radiographic data suggested the presence of a cavernous malformation.
INTERVENTION
A right frontotemporal craniotomy was performed under frameless stereotactic image guidance. An astroblastoma was diagnosed after resection and neuropathological examination.
CONCLUSION
A rare radiological to pathological correlation of astroblastoma is presented in which the evolving hematoma, as observed on magnetic resonance imaging scans, complicated the radiographic diagnosis of this lesion. The clinical, radiographic, and pathological features of astroblastomas, as well as the natural history of these rare glial neoplasms, are reviewed. This case illustrates the capacity of astroblastomas to hemorrhage, disguising the classic radiographic findings typical of this glial neoplasm.
Topics: Adult; Brain Neoplasms; Diagnosis, Differential; Female; Hemangioma, Cavernous, Central Nervous System; Hematoma, Epidural, Cranial; Humans; Neoplasms, Neuroepithelial; Radiography
PubMed: 17327764
DOI: 10.1227/01.NEU.0000255336.80285.70