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Pediatric Neurosurgery 2010Astroblastoma is a rare primary glial tumor of children and young adults, typically located in the cerebral hemispheres and presenting as a well-circumscribed, nodular,...
Astroblastoma is a rare primary glial tumor of children and young adults, typically located in the cerebral hemispheres and presenting as a well-circumscribed, nodular, often cystic enhancing mass. The existence of astroblastoma as a distinct clinicopathologic entity has long been debated but is recognized in the 2007 WHO classification of CNS tumors. The grading of these tumors remains unsettled. Currently, no grade has been established and there are no clearly defined diagnostic criteria for low- or high-grade tumors. Astroblastomas in general are thought to have a favorable outcome with low rates of recurrence following gross total resection. We report a case of astroblastoma with 'high-grade/anaplastic' histology in a 12-year-old, previously healthy girl. The patient experienced severe, sudden-onset headache, and quickly became comatose. Head CT showed a massive intraparenchymal hemorrhage in the right frontal lobe with surrounding edema. She underwent emergent posterior frontal craniotomy for decompression and hematoma evacuation. Pathologic examination revealed a sharply demarcated hemorrhagic heterogeneous glial tumor with focal papillary architecture, densely hyalinized blood vessels, and intensely GFAP-positive perivascular cells. The tumor showed unequivocal high-grade features including an elevated proliferative index. The diagnosis of anaplastic astroblastoma was rendered. The patient expired on postoperative day 30. This case illustrates the potential poor outcome of high-grade astroblastoma and highlights the morphologic heterogeneity of this rare neoplasm.
Topics: Acute Disease; Biopsy; Brain Neoplasms; Cerebral Hemorrhage; Child; Fatal Outcome; Female; Humans; Neoplasms, Neuroepithelial; Severity of Illness Index; Tomography, X-Ray Computed
PubMed: 21540623
DOI: 10.1159/000325071 -
Child's Nervous System : ChNS :... May 2018Astroblastoma is an uncommon pediatric neuroepithelial tumor. The prognosis and appropriate treatment of astroblastoma were not well understood. Previous reports...
PURPOSE
Astroblastoma is an uncommon pediatric neuroepithelial tumor. The prognosis and appropriate treatment of astroblastoma were not well understood. Previous reports suggested the best treatment for astroblastoma is surgical total resection. The authors report a case of pediatric astroblastoma that underwent gross total resection with the use of fluorescent guidance by 5-aminolevulinic acid (5-ALA).
CASE REPORT
A 13-year-old girl presented with the tumor that was well-circumscribed cystic and solid mass with marked gadolinium enhancement in the right occipital lobe. At surgery, fluorescence of the tumor was clearly distinctive from the normal cerebral tissue. All fluorescent tissue including residual cyst wall was removed. Postoperative MRI showed gross total resection of the tumor. No serious side effect or complications occurred. The histopathologic diagnosis was suggestive of astroblastoma. The patients had no evidence of recurrence of tumor without adjuvant radiotherapy during the last 1 year of follow-up time.
CONCLUSION
5-ALA is useful to achieve gross total resection including cystic lesion of pediatric astroblastoma. A larger prospective study is warranted to establish the use of 5-ALA in pediatric brain tumor.
Topics: Adolescent; Aminolevulinic Acid; Brain Neoplasms; Humans; Magnetic Resonance Imaging; Male; Neoplasms, Neuroepithelial; Neurosurgical Procedures; Photosensitizing Agents; Tomography Scanners, X-Ray Computed
PubMed: 29305683
DOI: 10.1007/s00381-017-3714-5 -
Ultrastructural Pathology 1999An astroblastoma of high-grade type arising in the brain of a 3-year-old child is reported. The first description of the ultrastructural, immunohistochemical, and...
An astroblastoma of high-grade type arising in the brain of a 3-year-old child is reported. The first description of the ultrastructural, immunohistochemical, and cytogenetic findings in this rare tumor variant are presented.
Topics: Aneuploidy; Biomarkers, Tumor; Brain Neoplasms; Child, Preschool; Female; Humans; Immunoenzyme Techniques; Karyotyping; Neoplasm Recurrence, Local; Neoplasms, Neuroepithelial; Parietal Lobe
PubMed: 10582270
DOI: 10.1080/019131299281473 -
Journal of Neuro-oncology Jul 2011Astroblastomas are exceedingly rare central nervous system tumors. Surgical resection is the standard initial treatment for astroblastomas. Still, some astroblastomas...
Astroblastomas are exceedingly rare central nervous system tumors. Surgical resection is the standard initial treatment for astroblastomas. Still, some astroblastomas that have been completely resected recur. The optimal treatment for these lesions is unclear. There are no previous reports of the use of Gamma Knife radiosurgery in the treatment of astroblastomas. The patient is a 58 year old woman who had undergone resection of a left parieto-occipital tumor at an outside hospital in 2002, with repeat resection for recurrences in 2005 and 2007. Pathologic analysis at the Mayo Clinic demonstrated the tumor to be a low-grade astroblastoma. Repeat imaging in 2008 again demonstrated recurrence, and the patient was referred to our center for Gamma Knife radiosurgery. Pre and post-contrast T1 stereotactic MR images were obtained and imported into the treatment planning system for the Gamma Knife Perfexion. Two foci of tumor consistent with the patient's known left parietal astroblastoma were identified. A prescription dose of 18.00 Gy to the 50% isodose was delivered, and 16 isocenters were used. Follow-up imaging 17 months post-radiosurgery demonstrated a decrease in tumor size. Gamma Knife radiosurgery represents a useful treatment modality for recurrent astroblastomas. While surgical resection of low grade astroblastomas can be curative, Gamma Knife radiosurgery may be beneficial in cases where gross total resection is not feasible.
Topics: Brain Neoplasms; Female; Humans; Magnetic Resonance Imaging; Middle Aged; Neoplasm Recurrence, Local; Neoplasms, Neuroepithelial; Radiosurgery; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 20938716
DOI: 10.1007/s11060-010-0430-3 -
Clinical Neuropathology 2011Astroblastoma is a rare glial tumor of uncertain origin affecting mostly children, adolescents and young adults. Given the rarity and the definitional problems...
INTRODUCTION
Astroblastoma is a rare glial tumor of uncertain origin affecting mostly children, adolescents and young adults. Given the rarity and the definitional problems concerning this tumor entity, the prognosis and appropriate treatment are at this point unclear.
CASE REPORT
A 50-yearold Caucasian female presented with a seizure. Radiological findings showed a welldefined circumscribed tumor located in the right cerebral frontal lobe. The patient underwent primary surgery followed by postoperative radiotherapy. After 6 months the tumor recurred with multiple small lesions not available for surgery. Chemotherapy was administered with complete radiological response. Seven years after surgery and more than 6 years after completed chemotherapy the patient is free of disease. Histopathology revealed a gliomatous tumor with gemistocyte-like tumor cells arranged in palisades or strings and areas with perivascular pseudorosettes, consistent with astroblastoma. Immunophenotype and ultrastructural findings confirmed the diagnosis and verified the neuroepithelial origin.
CONCLUSION
Astroblastomas are rare brain tumors and pose a challenge in the diagnostic and clinical approach. In general, they have an unpredictable course with a tendency of recurrence. This and other case reports support a survival benefit of chemotherapy, suggesting this as an important treatment option for these patients.
Topics: Brain Neoplasms; Frontal Lobe; Glioma; Humans; Magnetic Resonance Imaging; Neoplasms, Neuroepithelial; Seizures
PubMed: 22011735
DOI: 10.5414/np300411 -
Neuropathology : Official Journal of... Sep 2002We report a case of astroblastoma with unusual signet-ring-like cell components. A 33-year-old-woman presented with occasional partial seizures of the face. Radiological...
We report a case of astroblastoma with unusual signet-ring-like cell components. A 33-year-old-woman presented with occasional partial seizures of the face. Radiological studies revealed an enhanced frontal mass lesion. At surgery, a gray, soft, well-circumscribed mass was seen and shelled out. Histologically, the tumor showed a perivascular arrangement and papillary-like patterns with compact cellularity. The tumor cells radiating from the hyalinized vessels showed broader, shorter, less tapered processes. A part of each tumor cell displayed prominent islands of signet-ring-like cells. Glial fibrillary acidic protein reaction revealed strongly positive staining of tumor cells and signet-ring-like cells. Eight years after the operation the patient remains well with no tumor recurrence. It remains to be determined whether, in this astroblastoma, the unusual signet-ring-like cell components were related to benign biological characteristics or to the tumor's low-grade form with incidental signet-ring-like cell appearance.
Topics: Adult; Brain Neoplasms; CD57 Antigens; Diagnosis, Differential; Female; Glial Fibrillary Acidic Protein; Humans; Keratins; Ki-67 Antigen; Mucin-1; Neoplasms, Neuroepithelial; S100 Proteins; Vimentin
PubMed: 12416560
DOI: 10.1046/j.1440-1789.2002.00435.x -
Handbook of Clinical Neurology 2012
Review
Topics: Humans; Neoplasms, Neuroepithelial
PubMed: 22230515
DOI: 10.1016/B978-0-444-53502-3.00005-7 -
Journal of Neuropathology and... Nov 2022
Topics: Humans; Glioma; Brain Neoplasms; Neoplasms, Neuroepithelial; Magnetic Resonance Imaging; Receptor, Fibroblast Growth Factor, Type 2
PubMed: 36164838
DOI: 10.1093/jnen/nlac087 -
Journal of Clinical Neuroscience :... Sep 2017This study aims to review the literature and identify key molecular markers affecting the prognosis of Gliomatosis cerebri (2) to evaluate the level of evidence and... (Review)
Review
This study aims to review the literature and identify key molecular markers affecting the prognosis of Gliomatosis cerebri (2) to evaluate the level of evidence and identify outstanding markers requiring further study. A literature search was conducted across 5 major databases using the key terms: "Molecular markers" AND "Gliomatosis cerebri" OR "diffuse astrocytoma." Critical appraisal and data presentation was performed inline with the PRISMA guidelines. Following search strategy implementation, 11 studies were included in the final review process. Our data demonstrates significant prognostic value associated with IDH1 mutation and variable evidence surrounding the role of INA expression, MGMT promoter methylation and other factors. However, there are significant limitations in the level of evidence obtained. As the genetic basis for the pathogenesis of Gliomatosis cerebri continues to widen, there is little data on markers aside from IDH1 mutation available. IDH1 mutation has been demonstrated to have significant effect on survival, particularly in patients with Gliomatosis cerebri type 2.
Topics: Astrocytoma; Biomarkers, Tumor; Brain Neoplasms; Humans; Neoplasms, Neuroepithelial; Prognosis
PubMed: 28539209
DOI: 10.1016/j.jocn.2017.04.043 -
Epilepsy & Behavior : E&B Feb 2017Neoplastic CNS lesions are a common cause of focal epilepsy refractory to anticonvulsant treatment, i.e. long-term epilepsy-associated tumors (LEATs). Epileptogenic... (Review)
Review
Neoplastic CNS lesions are a common cause of focal epilepsy refractory to anticonvulsant treatment, i.e. long-term epilepsy-associated tumors (LEATs). Epileptogenic tumors encompass a variety of intriguing lesions, e.g. dysembryoplastic neuroepithelial tumors or gangliogliomas, which differ from more common CNS neoplasms in their clinical context as well as on histopathology. Long-term epilepsy-associated tumor classification is a rapidly evolving issue in surgical neuropathology, with new entities still being elucidated. One major issue to be resolved is the inconsistent tissue criteria applied to LEAT accounting for high diagnostic variability between individual centers and studies, a problem recently leading to a proposal for a new histopathological classification by Blümcke et al. in Acta Neuropathol. 2014; 128: 39-54. While a new approach to tissue diagnosis is appreciated and needed, histomorphological criteria alone will not suffice and we here approach the situation of encountering a neoplastic lesion in an epilepsy patient from a clinical perspective. Clinical scenarios to be supported by an advanced LEAT classification will be illustrated and discussed.
Topics: Brain Neoplasms; Epilepsies, Partial; Epilepsy; Ganglioglioma; Humans; Neoplasms, Neuroepithelial
PubMed: 28110204
DOI: 10.1016/j.yebeh.2016.12.020