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Purpose: Supratentorial (ST) ependymoma subgroups are defined by two different fusions with different prognoses. Astroblastomas, MN1-altered, have ependymal-like histopathologic features and represent a differential diagnosis in children. We hypothesized that ZFTA-fused ependymoma and YAP1-fused ependymoma on the one hand, and astroblastoma, MN1-altered, on the other hand, show different MRI characteristics. Methods: We retrospectively analyzed the preoperative imaging of 45 patients with ST ependymoma or astroblastoma between January 2000 and September 2020, blinded to histomolecular grouping. Several characteristics, such as location, tumor volume, calcifications, solid/cystic component, and signal enhancement or diffusion were evaluated. We compared imaging characteristics according to their molecular subtype (ZFTA-fused, YAP1-fused, and astroblastoma, MN1-altered). Results: Thirty-nine patients were classified as having an ependymoma, 35 with a ZFTA fusion and four with a YAP1 fusion, and six as having an astroblastoma, MN1-altered. YAP1-fused ependymomas were more likely to involve at least 3 lobes than ZFTA-fused ependymomas. Astroblastomas were located in the frontal lobe in 100% of the tumors versus 49% of the ependymomas. Cerebral blood flow by arterial spin labeling was higher in astroblastomas than in ependymomas. There were no differences in the other characteristics between the molecular groups. All the tumors showed common features: intra-axial extra-ventricular tumors, very frequent contrast enhancement (39/43, 91%), a cystic/necrotic component (41/45, 91%), restricted diffusion (32/36, 89%), calcifications (15/18, 83%), and peri-tumoral edema (38/44, 86%). Conclusion: The distinction between ST ependymoma subtypes and astroblastomas can be guided by several imaging features. These tumors share common imaging features that may help to differentiate ST ependymomas and astroblastomas from other pediatric ST tumors. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Purpose: Central nervous system high-grade neuroepithelial tumor with MN1 alteration (CNS-HGNET-MN1) is a rare entity defined by its DNA methylation pattern and pathologically considered to be high-grade with mixed patterns, stromal hyalinization, and with astrocytic differentiation. Our aim was to present six pediatric cases to contribute to the characterization of this group of tumors. Material and methods: Six female patients aged 4 to 12 years with CNS tumors with MN1 alteration identified using genome-wide methylation arrays and/or RT-PCR were included. Clinicopathological, morphological, immunohistochemical, and molecular findings were analyzed. Results: Tumor location was the parietal lobe in four and the intramedullary spinal cord in two. Two were morphologically diagnosed as ependymomas, one as gliofibroma, one as a HGNET-MN1 altered and the other two were difficult to classify. All were well-defined tumors, with a cystic component in three. Only two tumors had extensive stromal hyalinization, three had pseudopapillary formations, and four had other patterns. Multinucleated, clear, and rhabdoid cells were present. Necrosis and histiocyte clusters were also observed. Proliferative index was >10 in four. GFAP, EMA, CK, and SYN were variable, while Olig2 staining was mostly positive. Four of six patients with supratentorial tumors and complete resections were alive and tumor free after 2 to 10 years of follow-up. The two cases with medullary involvement and incomplete resections were alive and undergoing treatment 2 years after surgery. Conclusion: Neuroepithelial-MN1 tumors are challenging and suspicion requires molecular confirmation. Our pediatric data contribute to the knowledge for accurate diagnosis. Although further studies with a larger number of cases should be conducted in order to draw more robust conclusions regarding clinico-pathological features, here we present valuable pediatric data to increase the knowledge that may lead to the accurate management of this group of tumors. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Astroblastomas are central nervous system tumours with unknown cell of origin and clinical behaviour. These tumours occur most commonly in cerebral hemispheres with spinal astroblastomas being very rare. We report a case of spinal astroblastoma which harboured MN1 alteration. [ABSTRACT FROM AUTHOR]

Introduction: Astroblastoma, MN1-altered (old name: high-grade neuroepithelial tumor/HGNET with MN1 alteration) is a recently described central nervous system tumor mostly affecting pediatric patients and profoundly young girls. Differential pathological diagnoses of these tumors include ependymoma, pleomorphic xanthoastrocytoma, embryonal tumor with multilayered rosettes, meningioma, and even glioblastoma. As the treatment approaches to these tumors differ, it is essential to increase the awareness about these tumors in the neurosurgical community. Clinical presentation: A 7-year-old female patient admitted with a 7-day history of headache, nausea, and vomiting. A contrasted MRI scan revealed a left parietal 4 × 4 × 5 cm mass with central necrosis and peripheral contrast enhancement. The tumor's histopathological findings were suggestive of a metastatic carcinoma with unknown primary, yet further genetic analysis revealed MN1 alteration. Peculiarly, the tumor pathomorphological features were not compatible with astroblastomas and exerted features strongly indicating a metastatic cancer; however, systemic PET and whole-body MRI failed to detect a primary malignancy. Outcome and conclusions: Eighteen months after gross-total tumor resection, an in-field and out-field multifocal recurrence developed which required a second surgery and subsequent chemo-radiotherapy. The patient is doing well for 1 year after the second treatment regimen at the time of this report. Despite the final cIMPACT6 classification in 2020 advised to define all MN1 altered brain tumors as astroblastomas, there exist prognostic differences in MN1-altered tumors with and without morphological features of astroblastoma. Rare morphological variants of MN1-altered tumors shall be recognized for their future prognostic and clinical classification. HGNET with MN1 alteration seems still be a more proper definition of such malignancies as an umbrella term. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

MN1 alteration characterizes a recently described group of neuroepithelial tumors with varied morphological features. In cIMPACT-NOW update 6, only those with astroblastoma morphology has been accepted as a newly recognized tumor type, whereas the rest of morphological variants are considered lesions sub-judice. We perform an individual patient data meta-analysis of MN1-altered neuroepithelial tumors comprising a total of 73 cases, in order to study the survival data and predictive markers for better diagnosis and management of this rare molecular entity. The 5- and 10-year progression-free survival are 38% and 0%, whereas the 5- and 10-year overall survival are 89% and 55%, respectively. Among all the morphological variants of MN1-altered tumor, astroblastoma morphology is significantly associated with an improved overall survival, emphasizing the importance of providing an integrated histologic and molecular diagnosis. Histological grading within the molecularly-defined MN1-altered astroblastoma remains controversial. In tumors with astroblastoma morphology, the odds of MN1-altered status among patients less than 15-year-old is 10.5 times that of those 15-year-old and older, and female of 9.4 times that of the male gender. Gross tumor resection appears as main treatment modality for better disease control based on observational data. [ABSTRACT FROM AUTHOR]

A 4-year-old girl presented to the hospital with a progressive headache, difficulty walking, and persistent daily vomiting for 3 weeks. Papilledema was observed on fundoscopic examination. A large left cerebellovermian tumor with "bubbly" appearance was discovered. Total removal of the tumor mass was performed, and a diagnosis of low-grade astroblastoma was made. Adjuvant radiotherapy was performed due to the risk of recurrence. The patient is disease-free and has been kept on close follow-up for 6 months. The occurrence of posterior fossa astroblastoma has been rarely reported in the literature. Thus, when a "bubby" appearance enhancing cystic solid tumor is located on the cerebellar hemisphere in a child, an astroblastoma should also be included in the differential diagnosis. [ABSTRACT FROM AUTHOR]

Astroblastomas are unique tumours with unresolved issues in terms of their origin, molecular biology, clinical behaviour, and response to treatment. To decipher the characteristics of this tumour, we reviewed cases histologically diagnosed as astroblastoma in our institute over the past 8 years, with immunohistochemistry, and performed fluorescence in situ hybridisation (FISH), for the newly emerged MN1 rearrangement which was reported in central nervous system high-grade neuroepithelial tumours. The mean age at diagnosis was 18.6 years with all cases seen in females and with supratentorial localisation. The tumours showed typical circumscription and bubbly appearance on imaging. The cohort included eight cases diagnosed as astroblastoma (two low grades; six anaplastic) based on histology and proliferative index. The tumours displayed characteristic astroblastic pseudorosettes with hyalinised vascular core and variable immunopositivity for glial fibrillary acidic protein, pan cytokeratin, and epithelial membrane antigen. MN1 break-apart by FISH was found in 5/8 of our cases (62.5%), which included 2 low-grade and 3 anaplastic tumours. Tumour recurrence was noted in three cases, with MN1 alteration in two. We account for one of the few series to study the MN1 rearrangement in astroblastoma and conclude that MN1 alteration is seen in a subset of these tumours. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Astroblastoma (AB) is a rare tumor with significant dilemma regarding diagnostic criteria, behavior, and optimum treatment. Materials and methods: We searched PubMed, Google Search, and Cochrane Library for eligible studies with the following search words: astroblastoma, high-grade astroblastoma, and anaplastic astroblastoma till July 1, 2016, published in English language and collected data regarding age, sex, site of disease, pathological grade, treatment received, and survival. Results: Data of 152 patients were retrieved from 63 publications. Median age was 16 years (range 0-71). Females were affected twice more frequently than male (70.3 vs. 29.7%). Tumors were most commonly located in the frontal (39%) followed by parietal lobe (26.7%). Fifty-two and 25% of the patients had headache and seizure at presentation, 76.3% of the patients underwent a gross total resection, 41 out of 89 had a high-grade tumor, and 56 patients received adjuvant radiation with a median dose of 54 Gy (range 20-72). Adjuvant chemotherapy was used in 23 patients. Temozolomide was the most common drug used in 30% of the patients. A combination of cisplatin, etoposide with vincristine, or ifosfamide was used in 17%. Median follow-up duration was 37 months (range 1-238). Median progression-free survival and OS were 36 and 184 months, respectively. Patients with a higher-grade tumor had significantly worse OS with HR 5.260 and p = 0.001. Forty patients experienced local progression. Sixty-five percent patients underwent surgery while 50% underwent radiation as salvage. Conclusion: AB has two distinct grades with higher-grade tumors having significantly poor survival. Maximal safe surgery followed by adjuvant radiation and temozolomide should be advocated for these tumors. [ABSTRACT FROM AUTHOR]

Background: Astroblastoma is a rare tumor of uncertain origin most commonly presenting in the cerebrum of children and young adults. The literature contains only case reports and small series regarding its radiologic features. This systematic review is the largest study of imaging findings of astroblastoma to date and serves to identify features that might differentiate it from other neoplasms.Objective: This study describes the imaging features of astroblastoma based on a systematic review of the literature and two new cases.Materials and Methods: We conducted a PubMed and Google Scholar database search that identified 59 publications containing 125 cases of pathology-confirmed astroblastoma, and we also added two new cases from our own institution. Data collected include patient age, gender, tumor location, morphology, calcifications and calvarial changes. We recorded findings on CT, MRI, diffusion-weighted imaging (DWI), MR spectroscopy, positron emission tomography (PET) and catheter angiography.Results: Age at diagnosis ranged 0-70 years (mean 18 years; median 14 years). Female-to-male ratio was 8:1. Of 127 cases, 66 reported CT, 78 reported MRI and 47 reported both findings. Not all authors reported all features, but the tumor features reported included supratentorial in 96% (122/127), superficial in 72% (48/67), well-demarcated in 96% (79/82), mixed cystic-solid in 93% (79/85), and enhancing in 99% (78/79). On CT, 84% (26/31) of astroblastomas were hyperattenuated, 73% (27/37) had calcifications and 7 cases reported adjacent calvarial erosion. Astroblastomas were hypointense on T1-W in 58% (26/45) and on T2-W in 50% (23/46) of MRI sequences. Peritumoral edema was present in 80% (40/50) of cases but was typically described as slight. Six cases included DWI findings, with 100% showing restricted diffusion. On MR spectroscopy, 100% (5/5) showed nonspecific tumor spectra with elevated choline and decreased N-acetylaspartate (NAA). PET revealed nonspecific reduced uptake of [F-18] 2-fluoro-2-deoxyglucose ((18)F-FDG) and increased uptake of [11C]-Methionine in 100% (3/3) of cases. Catheter angiography findings (n=12) were variable, including hypervascularity in 67%, arteriovenous shunting in 33% and avascular areas in 25%.Conclusion: Astroblastomas occur most often in adolescent girls. Imaging often shows a supratentorial, superficial, well-defined, cystic-solid enhancing mass. On CT, most are hyperattenuated, have calcifications, and may remodel adjacent bone if superficial. MRI characteristically reveals a hypointense mass on T1-W and T2-W sequences with restricted diffusion. MR spectroscopy, PET and catheter angiography findings are nonspecific. [ABSTRACT FROM AUTHOR]

Astroblastoma is a rare neuroepithelial neoplasm of unknown origin, usually occurring in children and young adults. Here we report a case of astroblastoma with uncommon features in an 18-year-old female. The tumor was a well-circumscribed cystic and solid mass with marked gadolinium enhancement in the right frontal lobe. Cytological examination showed polarized monopolar cells with diminished cohesiveness. Tumor cells possessed eccentric round to oval nuclei with abundant eosinophilic cytoplasm, sometimes having cytoplasmic processes. Histopathologically, the tumor showed perivascular pseudorosettes with prominent vascular sclerosis. Foam cells were frequently infiltrated around blood vessels and among tumor cells. In some areas, a solid growth pattern of plump tumor cells with abundant inclusion-like eosinophilic cytoplasm showing rhabdoid appearance was observed. The immunohistochemical study revealed strong and diffuse positivity for vimentin and epithelial membrane antigen. Tumor cells were focally positive for glial fibrillary acidic protein and cytokeratin AE1/AE3. Nuclear immunoreactivity for INI1 protein was evident. The Ki-67 labeling index was 10.8 %. This tumor was finally diagnosed as low-grade astroblastoma and the patient had no evidence of recurrence without postoperative radiotherapy or chemotherapy during the last 6 months of follow-up. This report describes novel cytological, histopathological, and immunohistochemical features of the rare tumor. [ABSTRACT FROM AUTHOR]

oblastomas 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. [ABSTRACT FROM AUTHOR]

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. 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. 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. [ABSTRACT FROM AUTHOR]

Astroblastoma is a very rare primary glial tumor occurring in children and young adults that is almost exclusively supratentorial in location. We report an extremely unusual presentation of a densely calcified posterior fossa astroblastoma with disseminated spinal and supratentorial metastasis. The mass exhibited neoplastic bone formation, which has not been reported, although calcifications are commonly seen in astroblastomas. A companion case of a low-grade astroblastoma that demonstrated classic histologic features but nonspecific and atypical imaging findings is also included. These cases expand the imaging and pathologic spectrum of this controversial tumor that shows highly variable biologic behavior and is difficult to distinguish from ependymoma. [ABSTRACT FROM AUTHOR]

A 17-year-old male patient underwent surgery five times (four consecutive intracranial tumor removal surgeries and a final spinal tumor removal surgery). After the third surgery, this case was reported as a low-grade astroblastoma that is characterized by perivascular pseudorosettes consisting of elongated tumor cells arranged around the blood vessels. However, the fourth and fifth surgical specimens demonstrated very interesting histological changes in the astroblastoma. Through the course of relapses, the constituent cells of the astroblastic perivascular rosettes became smaller and rounder, and a multilayered cell arrangement was observed. The nucleus-to-cytoplasm ratio increased, and the compact intervascular cells ultimately lost glial fibrillary acidic protein (GFAP) expression. These undifferentiated cells showed high MIB-1 indices and an increased olig2 index. On the other hand, the cells in all the surgical specimens were positive for certain neuronal markers such as NSE, TUJ1, and nestin. Some astroblastomas may be more immature than the usual astrocytes; however, it is necessary to study more cases to confirm this. [ABSTRACT FROM AUTHOR]

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. 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. 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. 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. [ABSTRACT FROM AUTHOR]

Background. Astroblastoma is a rare glial tumour about which little is known.Method. We report a case of cerebral high grade astroblastoma and discuss the clinical, histopathological, surgical, radiological and prognostic features of this tumour, in the light of the pertinent literature.Result. Present patient had an initial histological diagnosis of glioblastoma multiforme. Three years later an histological revaluation was performed and revealed a high grade astroblastoma. Our patient underwent surgical removal and radiotherapy; five years after the operation he is alive and without evidence of recurrence.Interpretation. Classification and histogenesis of this tumour is still debated. The lack of a clinicopathological correlation makes the prognosis of this tumour unpredictable. The optimal management is not defined, but total resection and post-operative radiotherapy seem to be the effective means to treat the astroblastoma. [ABSTRACT FROM AUTHOR]

Astroblastoma is a rare and controversial tumor about which little is known. We have made the diagnosis in seven patients since 1990 at Memorial Sloan-Kettering Cancer Center. Four patients had astroblastomas with anaplastic features, whereas three patients had tumors which were well-differentiated. All three patients with low grade lesions are alive and recurrence free after surgery alone (mean follow-up 29 months). All four patients with anaplastic astroblastoma were treated with surgery, radiotherapy and chemotherapy. One died of infection during induction chemotherapy. Two others died of tumor progression at 28 and 42 months. Radiographic response to chemotherapy was seen in one patient. The results of our series and other reports suggest that anaplastic histology is a prognostic factor in the setting of astroblastoma. More aggressive treatment is necessary for patients with anaplastic astroblastoma although the precise role of irradiation and chemotherapy cannot be defined at this time. [ABSTRACT FROM AUTHOR]