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Neuroradiology Mar 2007Astroblastoma is a rare glial tumor of uncertain origin. Only a few scattered case reports and one small case series have described the radiologic appearance of this...
INTRODUCTION
Astroblastoma is a rare glial tumor of uncertain origin. Only a few scattered case reports and one small case series have described the radiologic appearance of this uncommon tumor. Many features previously identified are similar to those of other primary malignant brain tumors. We report the largest imaging series to date and further delineate the CT and MRI features of astroblastoma. We identify those features that may be useful in distinguishing astroblastoma from other neoplasms.
METHODS
The radiologic images, pathology reports, and clinical information of 12 patients with pathology-confirmed astroblastoma were retrospectively reviewed. CT and MRI findings including location, morphology, signal intensity, and presence and patterns of enhancement were tabulated.
RESULTS
Patients ranged in age from 0 (newborn) to 50 years with a mean of 20 years at the time of initial diagnosis. A striking female preponderance (11:1) was found. All tumors were supratentorial. There were multiple intratumoral cysts in 7 (58%) of the 12 patients. Nine (75%) showed strong rim enhancement and 3 (25%) showed no rim enhancement.
CONCLUSION
The imaging features of astroblastoma are identified in 12 previously unreported cases. Distinguishing features that can be used to narrow the differential diagnosis with more common primary brain neoplasms reflect a combination of age, anatomic location, and specific imaging findings such as demarcation, heterogeneous tumor enhancement, rim enhancement, and a multicystic "bubbly" appearance. Intraventricular location, intratumoral hemorrhage with a fluid-fluid level, and dural "tails" are less common but important additions to the imaging spectrum.
Topics: Adolescent; Adult; Brain Neoplasms; Child; Child, Preschool; Contrast Media; Diagnosis, Differential; Female; Gadolinium DTPA; Humans; Infant; Infant, Newborn; Magnetic Resonance Imaging; Male; Middle Aged; Neoplasms, Neuroepithelial; Retrospective Studies; Tomography, X-Ray Computed
PubMed: 17216265
DOI: 10.1007/s00234-006-0182-0 -
Neurocirugia (Asturias, Spain) Feb 2006Astroblastoma is a rare glial neoplasm of unknown origin and uncertain prognosis. It usually presents in young adults as a well circumscribed hemispheric mass, often... (Review)
Review
Astroblastoma is a rare glial neoplasm of unknown origin and uncertain prognosis. It usually presents in young adults as a well circumscribed hemispheric mass, often associated with a cystic component. The histological features of astroblastoma are the presence of typical astroblastic perivascular pseudorosettes and perivascular hyalinization. Two different subtypes of astroblastoma have been defined based upon histological characteristics. Prognosis, however, sometimes is in contradiction with the pathological appearance and seems to be more closely related to the grade of surgical resection. We present a new case of a patient with a high-grade astroblastoma with a long survival time, in whom complete surgical resection was confirmed by an early postoperative MRI.
Topics: Adult; Brain Neoplasms; Female; Humans; Neoplasms, Neuroepithelial; Survivors; Time Factors
PubMed: 16565782
DOI: 10.1016/s1130-1473(06)70371-2 -
CNS Oncology Mar 2019Astroblastoma is an uncommon neuroepithelial primary tumor of the brain which is of uncertain origin. We present a case of high-grade astroblastoma in an 18-year-old...
Astroblastoma is an uncommon neuroepithelial primary tumor of the brain which is of uncertain origin. We present a case of high-grade astroblastoma in an 18-year-old female with a severe headache, loss of appetite, vomiting and generalized weakness. The patient had undergone a right frontoparietal craniotomy. Large subfalcine meningioma was excised. The lesion was suspected to be a meningioma. Primary radiological investigation revealed a 6.8 cm × 5.8 cm × 5.4 cm lesion. Although the radiological and intraoperative findings were of an extra-axial tumor, the histology and immunophenotype was of an astroblastoma. The patient was treated with cyclophosphamide, cisplatin and etoposide chemotherapy regimen. The patient was later treated with bi-weekly bevacizumab. The patient had improved symptomatically post-chemotherapy. However, there was no significant difference in lesion size. The patient died after 2 weeks. The prognosis of patients with astroblastoma is extremely poor as observed in our case.
Topics: Adolescent; Brain; Brain Neoplasms; Diagnosis, Differential; Fatal Outcome; Female; Humans; Neoplasm Grading; Neoplasms, Neuroepithelial
PubMed: 30813777
DOI: 10.2217/cns-2018-0012 -
Unique pathological findings of astroblastoma with MN1 alteration in a patient with late recurrence.Brain Tumor Pathology Jul 2021Astroblastoma is an extremely rare brain tumor that has recently attracted attention owing to its association with MN1 gene alteration. However, its long-term clinical...
Astroblastoma is an extremely rare brain tumor that has recently attracted attention owing to its association with MN1 gene alteration. However, its long-term clinical course remains unclear. We report a late recurrence of MN1-altered astroblastoma with unique pathological findings. A 24-year-old woman presented with seizures due to a left frontal lobe tumor. Gross total resection (GTR) was achieved, and the diagnosis was MN1-altered astroblastoma, which presented cell wrapping, i.e., presence of tumor cells enveloping one another. She received local radiotherapy (50 Gy). However, the tumor recurred after 12 years, and its size increased rapidly. The second surgery achieved GTR and confirmed increasing anaplasia. The patient was tumor-free for 1 year without any neurological deficits. This case implies the importance of long-term follow-up of MN1-altered astroblastoma. The pathological significance of cell wrapping in this case is unclear, but it may be associated with MN1-altered astroblastoma and should be noted in future cases.
Topics: Adult; Biosimilar Pharmaceuticals; Brain Neoplasms; Female; Humans; Mutation; Neoplasm Recurrence, Local; Neoplasms, Neuroepithelial; Time Factors; Tomography, X-Ray Computed; Trans-Activators; Tumor Suppressor Proteins; Young Adult
PubMed: 33913040
DOI: 10.1007/s10014-021-00401-6 -
Journal of Neurosurgery. Spine Jun 2018Astroblastoma is a rare tumor that is thought to occur exclusively in the cerebrum. To the authors' knowledge, no cases of spinal cord astroblastoma have been reported....
Astroblastoma is a rare tumor that is thought to occur exclusively in the cerebrum. To the authors' knowledge, no cases of spinal cord astroblastoma have been reported. A 20-year-old woman presented with numbness in her legs. MRI demonstrated a 2-cm intramedullary enhancing lesion in the spinal cord at the T-1 level. The patient declined to undergo resection of the tumor because she was able to walk unassisted; however, she returned for surgery 1 month later because she had developed paraplegia with bladder and rectal dysfunction, and MRI showed enlargement of the tumor. Intraoperatively, the border between the tumor and normal tissue was poorly defined. Biopsy samples were obtained for histopathological examinations, and a diagnosis of astroblastoma with a Ki-67 index of 5% was made. Considering the rapid tumor growth on MRI and remarkable deterioration in her symptoms, the patient was treated with a combination of radiation therapy, temozolomide (TMZ), and bevacizumab. After completion of the combined treatment, she was able to move her toes, and oral TMZ and bevacizumab injections were continued. Six months later, definite tumor shrinkage was identified on MRI, and the patient was able to stand up from a wheelchair without assistance and walk by herself. No therapeutic regimens for residual astroblastoma are established; however, in this case the authors' therapeutic strategy was successful in treating the spinal cord astroblastoma.
Topics: Combined Modality Therapy; Diagnosis, Differential; Female; Humans; Neoplasms, Neuroepithelial; Recovery of Function; Spinal Cord Neoplasms; Young Adult
PubMed: 29498581
DOI: 10.3171/2017.9.SPINE161302 -
No Shinkei Geka. Neurological Surgery Sep 2023In the fifth edition central nervous system tumours volume of the WHO Classification of Tumours series, gliomas, glioneuronal tumors, and neuronal tumors are divided...
In the fifth edition central nervous system tumours volume of the WHO Classification of Tumours series, gliomas, glioneuronal tumors, and neuronal tumors are divided into six groups. The term "circumscribed" is used to refer to a relatively contained growth pattern, as compared to other inherently "diffuse" tumors. Circumscribed astrocytic gliomas include six types: pilocytic astrocytoma, high-grade astrocytoma with piloid features, pleomorphic xanthoastrocytoma, subependymal giant cell astrocytoma, chordoid glioma, and astroblastoma, -altered. The vast majority of circumscribed astrocytic gliomas harbor genetic alterations in the mitogen-activated protein kinase pathway. Here, we review the circumscribed astrocytic gliomas, including etiology, clinical and imaging features, pathology and molecular genetics, treatment, and prognosis. This study will lead to better understanding of these newly classified tumors.
Topics: Humans; Astrocytoma; Brain Neoplasms; Central Nervous System Neoplasms; Glioma; Neoplasms, Neuroepithelial
PubMed: 37743340
DOI: 10.11477/mf.1436204830 -
Brain Tumor Pathology Oct 2021The most recurrent fusion of central nervous system high-grade neuroepithelial tumor with MN1 alteration (HGNET-MN1) is MN1 rearrangement. Here, we report the case of a...
The most recurrent fusion of central nervous system high-grade neuroepithelial tumor with MN1 alteration (HGNET-MN1) is MN1 rearrangement. Here, we report the case of a 36-year-old man with spinal cord astroblastoma showing Ewing Sarcoma breakpoint region 1/EWS RNA-binding protein 1 (EWSR1)-BEN domain-containing 2 (BEND2) fusion. The patient presented with back pain, gait disturbance and dysesthesia in the lower extremities and trunk. Magnetic resonance imaging showed an intramedullary tumor at the T3-5 level, displaying homogeneous gadolinium enhancement. Partial tumor removal was performed with laminectomy. Histological examinations demonstrated solid growth of epithelioid tumor cells showing high cellularity, a pseudopapillary structure, intervening hyalinized fibrous stroma, and some mitoses. Astroblastoma was diagnosed, classified as HGNET-MN1 by the German Cancer Research Center methylation classifier. MN1 alteration was not detected by fluorescence in situ hybridization (FISH), but EWSR1-BEND2 fusion was detected by FISH and RNA sequencing. Previously, a child with EWSR1-BEND2 fusion-positive spinal astroblastoma classified as HGNET-MN1 was reported. In conjunction with that, the present case provides evidence that EWSR1-BEND2 fusion is identified in the entity of HGNET-MN1. Taken together, the BEND2 alteration rather than MN1 may determine the biology of a subset of the central nervous system HGNET-MN1 subclass.
Topics: Adult; Contrast Media; Gadolinium; Humans; In Situ Hybridization, Fluorescence; Male; Methylation; Neoplasms, Neuroepithelial; RNA-Binding Protein EWS; Spinal Cord; Trans-Activators; Tumor Suppressor Proteins
PubMed: 34313881
DOI: 10.1007/s10014-021-00412-3 -
AJNR. American Journal of Neuroradiology Feb 2002Astroblastoma is a rare primary glial tumor with a characteristic appearance on neuroradiologic images. Typically, astroblastomas are large, lobulated, peripheral,...
Astroblastoma is a rare primary glial tumor with a characteristic appearance on neuroradiologic images. Typically, astroblastomas are large, lobulated, peripheral, supratentorial, solid, and cystic masses with relatively little associated vasogenic edema and tumor infiltration for their large size. The solid component of the mass has a bubbly appearance and a T2 signal that is isointense to gray matter. Punctate calcifications are often present. Neuroradiologists should be familiar with the characteristic appearance of this tumor.
Topics: Adolescent; Adult; Brain Neoplasms; Child, Preschool; Diagnosis, Differential; Ependymoma; Female; Humans; Magnetic Resonance Imaging; Male; Neoplasms, Neuroepithelial; Retrospective Studies; Tomography, X-Ray Computed
PubMed: 11847049
DOI: No ID Found -
Child's Nervous System : ChNS :... May 2023Astroblastomas (AB) are high-grade neoplasms which typically occur within the cerebral hemisphere. However, given the rarity of this neoplasm and the number of variants,...
PURPOSE
Astroblastomas (AB) are high-grade neoplasms which typically occur within the cerebral hemisphere. However, given the rarity of this neoplasm and the number of variants, the relevance of this molecular makeup is unknown. We sought to describe the clinical presentation, treatment, and pathological analysis of a novel MN1 (meningioma 1) cervical spinal cord astroblastoma variant presenting in a pediatric patient.
METHODS
A retrospective review of electronic medical records was performed with an emphasis on neuroimaging, perioperative course, and pathological analysis.
RESULTS
An 11-month-old male with no significant history presented with two weeks of neck stiffness and cervicalgia. Neurologically, the patient was intact without signs of infection or trauma. Cervical CT was unremarkable. A subsequent MRI demonstrated a heterogeneously enhancing intramedullary lesion extending from the craniocervical junction to T4. The patient was treated with perioperative steroids and underwent C1-C3 laminectomies and C4-T4 laminotomies for tumor resection. Upon completion of the durotomy, an exophytic gray-red tumor was appreciated within the epidural space and gross total resection was achieved (no change on intraoperative neurophysiological monitoring) and confirmed on post-operative imaging. Immunohistochemical analysis was consistent with an astroblastoma with atypical diffuse positivity of CD56, CD99, and nuclear OLIG2. Molecular analysis revealed not only MN1 alterations but also changes in genes encoding APC and LRP1B. Both alterations were not previously documented to be associated with an astroblastoma.
CONCLUSION
Our case represents the first report of an infant with an MN1 astroblastoma with APC and LRP1B gene alterations in the cervical spine. Gross total resection paired with a detailed histopathologic analysis is vital for optimizing adjuvant treatment.
Topics: Child; Humans; Infant; Male; Brain Neoplasms; Cervical Vertebrae; Magnetic Resonance Imaging; Mutation; Neoplasms, Neuroepithelial; Neuroimaging; Receptors, LDL; Trans-Activators; Tumor Suppressor Proteins
PubMed: 36648513
DOI: 10.1007/s00381-022-05795-y -
Cancer Genetics Feb 2019Astroblastoma is a rare glial neoplasm composed of cells that have broad processes oriented perpendicular to central vessels and often demonstrate vascular sclerosis....
Astroblastoma is a rare glial neoplasm composed of cells that have broad processes oriented perpendicular to central vessels and often demonstrate vascular sclerosis. The WHO 2016 classification does not specify a grading system for astroblastoma, and categorizes them as well-differentiated or malignant. These broad classification rubrics, however, do not accurately predict clinical outcome. Genetic profiling of astroblastoma has therefore been of particular interest in the recent years. These efforts, although in small number, have revealed heterogeneous molecular findings that may explain astroblastoma's unpredictable clinical outcome. Here, we report a case of recurrent astroblastoma in a 23-year-old female with a unique molecular characteristic. Our patient's tumor harbored an RNA-binding motif 10 (RBM10) truncation. RBM10 codes for a widely expressed RNA binding protein, and its mutation has been described in a variety of solid cancers. RBM10 is thought to be involved in stabilization of pro-apoptotic proteins in breast cancer, and its reduced protein expression is associated with advanced stages of lung adenocarcinoma. To our knowledge, this is the first report of astroblastoma harboring RBM10 truncation. Interestingly, our patient also has a history of mandibular ameloblastoma, but the link between these two rare tumors is unclear.
Topics: Ameloblastoma; Female; Humans; Magnetic Resonance Imaging; Mandibular Neoplasms; Mutation; Neoplasms, Neuroepithelial; RNA-Binding Proteins; Young Adult
PubMed: 30803556
DOI: 10.1016/j.cancergen.2019.01.001