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MedGenMed : Medscape General Medicine Dec 2004Pilomyxoid astrocytoma (PMA) is a recently described type of brain tumor. PMA shares similar features with pilocytic astrocytoma (PA), the most common central nervous... (Review)
Review
Pilomyxoid astrocytoma (PMA) is a recently described type of brain tumor. PMA shares similar features with pilocytic astrocytoma (PA), the most common central nervous system (CNS) tumor in the pediatric population, yet displays subtle histologic differences. Previous studies have shown PMA to behave more aggressively than PA, with shorter progression-free and overall survival as well as a higher rate of recurrence and CNS dissemination. These findings suggest that PMA may be a unique and distinct neoplasm. This review summarizes the histologic, clinical, and radiographic characteristics of PMA. In addition, the current treatment options and research endeavors involving this disease are described. Increased recognition of PMA within the medical community has the potential to affect the treatment and prognosis of pediatric low-grade astrocytomas.
Topics: Astrocytoma; Brain; Brain Neoplasms; Humans; Radiography
PubMed: 15775869
DOI: No ID Found -
Oncology (Williston Park, N.Y.) Apr 1998Traditionally, cytotoxic drugs have played a limited role in the treatment of brain tumors, but important advances in chemotherapy have occurred during the past decade.... (Review)
Review
Traditionally, cytotoxic drugs have played a limited role in the treatment of brain tumors, but important advances in chemotherapy have occurred during the past decade. Certain central nervous system (CNS) malignancies are remarkably chemosensitive. These include primary CNS lymphoma, medulloblastoma, oligodendroglioma, and intracranial germ-cell tumors. This review focuses on advances in the chemotherapy of these chemosensitive tumors and also discusses the potential use of chemotherapeutic agents, both cytotoxic and cytostatic, in other brain tumors, such as glioblastomas and anaplastic astrocytomas. In addition, a brief description of future directions that may hold promise, including high-dose chemotherapy with stem-cell rescue, blood-brain barrier disruption, and regional treatment using controlled-release biodegradable polymers, is included.
Topics: Antineoplastic Agents; Astrocytoma; Brain Neoplasms; Germinoma; Glioblastoma; Humans; Lymphoma; Medulloblastoma; Oligodendroglioma
PubMed: 9575527
DOI: No ID Found -
The Cochrane Database of Systematic... May 2014Standard care of adjuvant treatment for anaplastic oligodendrogliomas (AO) and anaplastic oligoastrocytomas (AOA) is not yet well defined. The benefit of adjuvant... (Review)
Review
BACKGROUND
Standard care of adjuvant treatment for anaplastic oligodendrogliomas (AO) and anaplastic oligoastrocytomas (AOA) is not yet well defined. The benefit of adjuvant chemotherapy and radiotherapy (RT), given as single modalities or sequentially, is still unclear. Furthermore, insight into the predictive and prognostic impact of various biomarkers is surging.
OBJECTIVES
To compare postoperative sequential RT and chemotherapy to RT alone in adults with newly diagnosed AO or mixed AOA. To evaluate the predictive and prognostic impact of the following biomarkers: codeletion of chromosomes 1p and 19q, O(6)-methylguanine-DNA methyltransferase (MGMT) promotor methylation and isocitrate dehydrogenase (IDH)-1 and -2 mutations.
SEARCH METHODS
We searched the Cochrane Central Register for Controlled Trials (CENTRAL, Issue 1, 2014), MEDLINE (2006 to March week 2, 2014) and EMBASE (2006 to week 11, 2014). We scanned reference lists from relevant studies for any additional articles.
SELECTION CRITERIA
We included randomized controlled trials (RCTs) of adults with AO, AOA or anaplastic astrocytoma (AA) comparing adjuvant treatment of chemotherapy, RT, or sequential chemotherapy and RT. We excluded no specific chemotherapy regimens.
DATA COLLECTION AND ANALYSIS
We critically appraised and extracted data from relevant studies. Based on the differences in participant selection with respect to the definition of AO (two versus three high-risk anaplastic features), the inclusion of AA and sequence of treatment (RT and chemotherapy), we could not consider the results from the three RCTs for meta-analysis.
MAIN RESULTS
Three RCTs, with 931 participants, tested different neoadjuvant treatments: RT alone; sequential RT and procarbazine, lomustine and vincristine (PCV) chemotherapy; PCV chemotherapy alone; and temozolomide chemotherapy alone. None of the studies blinded participants or personnel, and, therefore, are considered at high risk of performance and detection bias. The studies were otherwise at low risk of bias. One study, the European Organisation for Research and Treatment of Cancer (EORTC) trial, demonstrated a statistically significant overall survival (OS) benefit for RT plus PCV, with a median OS of 3.5 years compared with 2.6 years in the RT alone arm (P value = 0.018). This result was reported 10 years after the conclusion of the enrolment, and was not apparent in the original 2008 Cochrane review. Furthermore, with retrospective evaluation of biomarkers, codeletion of complete chromosome arms 1p and 19q and IDH-1 or -2 mutation were independent prognostic factors for OS in two of the RCTs (Radiation Therapy Oncology Group (RTOG) and EORTC), and were predictive for OS in one trial (RTOG). The third trial (NOA-04) evaluated these biomarkers prospectively and found them prognostic for progression-free survival.
AUTHORS' CONCLUSIONS
Early PCV, either before or after RT, appears to improve OS of participants with AO or AOA. Use of biomarkers including codeletion of chromosomes 1p and 19q with or without IDH-1 or -2 mutation identify a subset of people with increased sensitivity to combined PCV and RT. The important role of biomarkers was supported in all of the RCTs examined, and prospective evaluation should be undertaken in future studies. However, PCV was associated with significant grade 3 and 4 toxicities, and whether temozolomide can be substituted for this remains unclear.
Topics: Adult; Astrocytoma; Brain Neoplasms; Chemotherapy, Adjuvant; Humans; Oligodendroglioma; Randomized Controlled Trials as Topic
PubMed: 24833028
DOI: 10.1002/14651858.CD007104.pub2 -
Neuropathology and Applied Neurobiology Dec 2022Anaplastic ganglioglioma is a rare tumour, and diagnosis has been based on histological criteria. The 5th edition of the World Health Organization Classification of...
AIMS
Anaplastic ganglioglioma is a rare tumour, and diagnosis has been based on histological criteria. The 5th edition of the World Health Organization Classification of Tumours of the Central Nervous System (CNS WHO) does not list anaplastic ganglioglioma as a distinct diagnosis due to lack of molecular data in previous publications. We retrospectively compiled a cohort of 54 histologically diagnosed anaplastic gangliogliomas to explore whether the molecular profiles of these tumours represent a separate type or resolve into other entities.
METHODS
Samples were subjected to histological review, desoxyribonucleic acid (DNA) methylation profiling and next-generation sequencing. Morphological and molecular data were summarised to an integrated diagnosis.
RESULTS
The majority of tumours designated as anaplastic gangliogliomas resolved into other CNS WHO diagnoses, most commonly pleomorphic xanthoastrocytoma (16/54), glioblastoma, isocitrate dehydrogenase protein (IDH) wild type and diffuse paediatric-type high-grade glioma, H3 wild type and IDH wild type (11 and 2/54), followed by low-grade glial or glioneuronal tumours including pilocytic astrocytoma, dysembryoplastic neuroepithelial tumour and diffuse leptomeningeal glioneuronal tumour (5/54), IDH mutant astrocytoma (4/54) and others (6/54). A subset of tumours (10/54) was not assignable to a CNS WHO diagnosis, and common molecular profiles pointing to a separate entity were not evident.
CONCLUSIONS
In summary, we show that tumours histologically diagnosed as anaplastic ganglioglioma comprise a wide spectrum of CNS WHO tumour types with different prognostic and therapeutic implications. We therefore suggest assigning this designation with caution and recommend comprehensive molecular workup.
Topics: Child; Humans; Ganglioglioma; Retrospective Studies; Glioma; Astrocytoma; Brain Neoplasms; Central Nervous System Neoplasms; Isocitrate Dehydrogenase
PubMed: 35977725
DOI: 10.1111/nan.12847 -
Clinical Neurology and Neurosurgery Aug 2016Anaplastic pilocytic astrocytoma (APA) is an exceptionally rare type of high-grade glioma in adults. Establishing histopathological diagnosis is challenging and its... (Review)
Review
INTRODUCTION
Anaplastic pilocytic astrocytoma (APA) is an exceptionally rare type of high-grade glioma in adults. Establishing histopathological diagnosis is challenging and its clinical and radiological appearance insidious. By this case series and first literature review we investigated the various clinical, neuroradiological, and histopathological features of APA in adults.
METHODS
An in hospital screening of the database from the Institute of Pathology was conducted to identify cases of APA. Further, we performed a literature review in PubMed using the keywords "anaplastic/malignant/atypical AND pilocytic astrocytoma" and "anaplastic astrocytoma/glioblastoma AND Rosenthal fibers" and summarized the current knowledge about APA in adults.
RESULTS
Over the last decade we were able to identify 3 adult patients with APA in our hospital. According to the pertinent literature, the prognosis of APA in adults (documented survival of up to 10 years) appears to be better than in other high-grade gliomas. Few cases were associated with neurofibromatosis type 1, which seems to predispose for development of APA. Although molecular genetics is still of limited value for differentiation of APA from other high-grade glioma, advanced neuroimaging techniques such as magnetic resonance perfusion imaging and spectroscopy allow improved differential work-up. In particular, APA in adults has the ability to mimic various neurological diseases such as tumefactive demyelinating lesions, low-, or high-grade gliomas.
CONCLUSIONS
Although currently not explicitly recognized as a distinct clinico-pathologic entity it seems that adult APA behaves differently from conventional high-grade glioma and should be included in differential diagnostics to enable adequate patient care. However, further studies are needed to better understand this extremely rare disease.
Topics: Adult; Astrocytoma; Brain Neoplasms; Female; Humans; Male; Middle Aged
PubMed: 27341279
DOI: 10.1016/j.clineuro.2016.06.005 -
BMC Cancer Jun 2017Co-occurrence of multiple sclerosis (MS) and glial tumours (GT) is uncommon although occasionally reported in medical literature. Interpreting the overlapping radiologic... (Review)
Review
BACKGROUND
Co-occurrence of multiple sclerosis (MS) and glial tumours (GT) is uncommon although occasionally reported in medical literature. Interpreting the overlapping radiologic and clinical characteristics of glial tumours, MS lesions, and progressive multifocal leukoencephalopathy (PML) can be a significant diagnostic challenge.
CASE PRESENTATION
We report a case of anaplastic astrocytoma mimicking PML in a 27-year-old patient with a 15-year history of MS. She was treated with interferon, natalizumab and finally fingolimod due to active MS. Follow-up MRI, blood and cerebrospinal fluid examinations, and biopsy were conducted, but only the latter was able to reveal the cause of progressive worsening of patient's disease.
CONCLUSIONS
Anaplastic astrocytoma misdiagnosed as PML has not yet been described. We suppose that the astrocytoma could have evolved from a low grade glioma to anaplastic astrocytoma over time, as the tumour developed adjacent to typical MS plaques. The role of the immunomodulatory treatment as well as other immunological factors in the malignant transformation can only be hypothesised. We discuss clinical, laboratory and diagnostic aspects of a malignant GT, MS lesions and PML. The diagnosis of malignant GT must be kept in mind when an atypical lesion develops in a patient with MS.
Topics: Adult; Astrocytoma; Biomarkers; Brain; Diagnosis, Differential; Female; Humans; Immunohistochemistry; Leukoencephalopathy, Progressive Multifocal; Magnetic Resonance Imaging; Magnetic Resonance Spectroscopy; Positron-Emission Tomography; Symptom Assessment
PubMed: 28629398
DOI: 10.1186/s12885-017-3415-1 -
Survey of Ophthalmology 1993On routine examination, a 61-year-old man was found to have a deep peripapillary hemorrhage surrounding his left optic nerve head. Further examination revealed a left...
On routine examination, a 61-year-old man was found to have a deep peripapillary hemorrhage surrounding his left optic nerve head. Further examination revealed a left sixth nerve paresis, a subtle right homonymous quadrantanopia, and jerky pursuit to the left. The diagnostic work-up was delayed by the patient because of business commitments. He returned confused and obtunded. Neuro-imaging showed a large frontal mass, which turned out to be an anaplastic astrocytoma. Diagnosis of the lesion had been obscured by three false localizing signs. Discussion deals with the definition of Terson's syndrome and the occurrence of peripapillary hemorrhages. Other causes of peripapillary hemorrhages are illustrated.
Topics: Astrocytoma; Brain Neoplasms; Cerebral Cortex; Fluorescein Angiography; Fundus Oculi; Humans; Male; Middle Aged; Optic Disk; Papilledema; Retinal Hemorrhage; Tomography, X-Ray Computed
PubMed: 8484169
DOI: 10.1016/0039-6257(93)90066-g -
European Journal of Nuclear Medicine... Nov 2022Both amino acid positron emission tomography (PET) and magnetic resonance imaging (MRI) blood volume (BV) measurements are used in suspected recurrent high-grade...
Diagnostic yield of simultaneous dynamic contrast-enhanced magnetic resonance perfusion measurements and [F]FET PET in patients with suspected recurrent anaplastic astrocytoma and glioblastoma.
PURPOSE
Both amino acid positron emission tomography (PET) and magnetic resonance imaging (MRI) blood volume (BV) measurements are used in suspected recurrent high-grade gliomas. We compared the separate and combined diagnostic yield of simultaneously acquired dynamic contrast-enhanced (DCE) perfusion MRI and O-(2-[F]-fluoroethyl)-L-tyrosine ([F]FET) PET in patients with anaplastic astrocytoma and glioblastoma following standard therapy.
METHODS
A total of 76 lesions in 60 hybrid [F]FET PET/MRI scans with DCE MRI from patients with suspected recurrence of anaplastic astrocytoma and glioblastoma were included retrospectively. BV was measured from DCE MRI employing a 2-compartment exchange model (2CXM). Diagnostic performances of maximal tumour-to-background [F]FET uptake (TBR), maximal BV (BV) and normalised BV (nBV) were determined by ROC analysis using 6-month histopathological (n = 28) or clinical/radiographical follow-up (n = 48) as reference. Sensitivity and specificity at optimal cut-offs were determined separately for enhancing and non-enhancing lesions.
RESULTS
In progressive lesions, all BV and [F]FET metrics were higher than in non-progressive lesions. ROC analyses showed higher overall ROC AUCs for TBR than both BV and nBV in both lesion-wise (all lesions, p = 0.04) and in patient-wise analysis (p < 0.01). Combining TBR with BV metrics did not increase ROC AUC. Lesion-wise positive fraction/sensitivity/specificity at optimal cut-offs were 55%/91%/84% for TBR, 45%/77%/84% for BV and 59%/84%/72% for nBV. Combining TBR and best-performing BV cut-offs yielded lesion-wise sensitivity/specificity of 75/97%. The fraction of progressive lesions was 11% in concordant negative lesions, 33% in lesions only BV positive, 64% in lesions only [F]FET positive and 97% in concordant positive lesions.
CONCLUSION
The overall diagnostic accuracy of DCE BV imaging is good, but lower than that of [F]FET PET. Adding DCE BV imaging did not improve the overall diagnostic accuracy of [F]FET PET, but may improve specificity and allow better lesion-wise risk stratification than [F]FET PET alone.
Topics: Humans; Glioblastoma; Brain Neoplasms; Retrospective Studies; Positron-Emission Tomography; Astrocytoma; Tyrosine; Magnetic Resonance Imaging; Perfusion; Magnetic Resonance Spectroscopy
PubMed: 35907033
DOI: 10.1007/s00259-022-05917-3 -
Brain Pathology (Zurich, Switzerland) Jan 2021Pleomorphic xanthoastrocytoma (PXA) is a rare astrocytoma predominantly affecting children and young adults. We performed comprehensive genomic characterization on a...
Pleomorphic xanthoastrocytoma (PXA) is a rare astrocytoma predominantly affecting children and young adults. We performed comprehensive genomic characterization on a cohort of 67 patients with histologically defined PXA (n = 53, 79%) or anaplastic PXA (A-PXA, n = 14, 21%), including copy number analysis (ThermoFisher Oncoscan, n = 67), methylation profiling (Illumina EPIC array, n = 43) and targeted next generation sequencing (n = 32). The most frequent alterations were CDKN2A/B deletion (n = 63; 94%) and BRAF p.V600E (n = 51, 76.1%). In 7 BRAF p.V600 wild-type cases, alternative driver alterations were identified involving BRAF, RAF1 and NF1. Downstream phosphorylation of ERK kinase was uniformly present. Additional pathogenic alterations were rare, with TERT, ATRX and TP53 mutations identified in a small number of tumors, predominantly A-PXA. Methylation-based classification of 46 cases utilizing a comprehensive reference tumor allowed assignment to the PXA methylation class in 40 cases. A minority grouped with the methylation classes of ganglioglioma or pilocytic astrocytoma (n = 2), anaplastic pilocytic astrocytoma (n = 2) or control tissues (n = 2). In 9 cases, tissue was available from matched primary and recurrent tumors, including 8 with anaplastic transformation. At recurrence, two tumors acquired TERT promoter mutations and the majority demonstrated additional non-recurrent copy number alterations. Methylation class was preserved at recurrence. For 62 patients (92.5%), clinical follow-up data were available (median follow-up, 5.4 years). Overall survival was significantly different between PXA and A-PXA (5-year OS 80.8% vs. 47.6%; P = 0.0009) but not progression-free survival (5-year PFS 59.9% vs. 39.8%; P = 0.05). WHO grade remained a strong predictor of overall survival when limited to 38 cases defined as PXA by methylation-based classification. Our data confirm the importance of WHO grading in histologically and epigenetically defined PXA. Methylation-based classification may be helpful in cases with ambiguous morphology, but is largely confirmatory in PXA with well-defined morphology.
Topics: Adolescent; Adult; Aged; Astrocytoma; Brain Neoplasms; Child; Child, Preschool; Cohort Studies; DNA Methylation; Female; Humans; Male; Middle Aged; Neoplasm Grading; Young Adult
PubMed: 32619305
DOI: 10.1111/bpa.12874 -
AJNR. American Journal of Neuroradiology Dec 2021Compared with wild-type pleomorphic xanthoastrocytoma, -mutant pleomorphic xanthoastrocytoma showed a higher survival rate. In this study, we focused on finding...
BACKGROUND AND PURPOSE
Compared with wild-type pleomorphic xanthoastrocytoma, -mutant pleomorphic xanthoastrocytoma showed a higher survival rate. In this study, we focused on finding preoperative MR imaging differences between mutant and wild-type in pleomorphic xanthoastrocytoma and anaplastic pleomorphic xanthoastrocytoma.
MATERIALS AND METHODS
Twenty-three patients with pathologically confirmed pleomorphic xanthoastrocytoma or anaplastic pleomorphic xanthoastrocytoma in our hospital were retrospectively analyzed from January 2015 to December 2020. They were divided into a -mutant group (including 6 pleomorphic xanthoastrocytomas and 5 anaplastic pleomorphic xanthoastrocytomas) and a wild-type group (including 8 pleomorphic xanthoastrocytomas and 4 anaplastic pleomorphic xanthoastrocytomas). The preoperative MR imaging characteristics of these groups were statistically compared.
RESULTS
The wild-type pleomorphic xanthoastrocytoma group presented with more aggressive conventional and advanced MR imaging features than the mutant pleomorphic xanthoastrocytoma group, including greater mean maximum tumor diameter (3.1 [SD, 0.9] cm versus 1.7 [SD, 0.4 ] cm, < .05), more frequent heterogeneous contrast enhancement of solid portions (100% versus 0%, < .001), more obvious peritumoral edema (mean, [2.1 SD, 0.7] cm versus 0.6 [SD, 0.2] cm, < .01), and lower mean minimum relative ADC (896 [SD, 86] versus 988 [SD, 73], < .05) and mean relative ADC (1060 [SD, 159] versus 1248 [SD, 116], < .05) on DWI. However, there was no significant difference in either conventional or advanced MR imaging features between the wild-type anaplastic pleomorphic xanthoastrocytoma group and the mutant anaplastic pleomorphic xanthoastrocytoma group.
CONCLUSIONS
Neurosurgeons should carefully interpret MR images before an operation and select appropriate surgical strategies according to genotype prediction.
Topics: Astrocytoma; Brain Neoplasms; Humans; Magnetic Resonance Imaging; Mutation; Proto-Oncogene Proteins B-raf; Retrospective Studies
PubMed: 34725042
DOI: 10.3174/ajnr.A7324