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Neurosurgery Sep 1984The term "diffuse cerebellar astrocytoma" was originally introduced by Russell and Rubinstein to describe that minority of cerebellar tumors microscopically similar to...
The term "diffuse cerebellar astrocytoma" was originally introduced by Russell and Rubinstein to describe that minority of cerebellar tumors microscopically similar to the cerebral hemispheric astrocytoma. The aim of this study was to verify some recent reports warning against the less favorable long term prognosis of the "diffuse" form of the cerebellar astrocytoma. We reviewed our series of cerebellar astrocytomas in children and collected 49 cases operated on before 1966 (i.e., with a follow-up ranging from a minimum of 15 years to 29 years). In addition, we reexamined all recurrent cerebellar astrocytomas observed during a 30-year period, looking for the eventual occurrence of the "diffuse" form. In the first group, 10 cases (20%) were classified as "diffuse" and 39 cases (80%) were classified as "classic". However, in 3 cases largely featuring a diffuse pattern, we also noted some areas of the classic type. These two subgroups showed no significant differences in patient sex and age, the incidence of macrocysts, the surgical technique used, the eventual x-ray therapy, and the long term functional results (P greater than 0.05). When we studied recurrent tumors that were reoperated on, we did not find any case showing histologically the "diffuse" pattern. To conclude, we cannot agree with the pessimism about the ominous long term prognosis of the diffuse cerebellar astrocytoma. In our opinion, the so-called diffuse cerebellar astrocytoma does not exist as a separate clinicopathological entity.
Topics: Adolescent; Astrocytoma; Cerebellar Neoplasms; Cerebellum; Child; Child, Preschool; Female; Humans; Infant; Male; Neoplasm Recurrence, Local; Prognosis
PubMed: 6483145
DOI: 10.1227/00006123-198409000-00004 -
Neurological Sciences : Official... Jul 2022Pilocytic astrocytoma (PA) rarely spreads along neuraxis, and association with superficial siderosis (SS) and chronic signs of intracranial hypertension is exceptional.
BACKGROUND
Pilocytic astrocytoma (PA) rarely spreads along neuraxis, and association with superficial siderosis (SS) and chronic signs of intracranial hypertension is exceptional.
CASE REPORT
A 48-year-old woman presented with slow onset hearing loss in the past year. Clinical examination revealed dysarthria, positive Romberg test, and severe optic neuropathy. Cerebrospinal fluid (CSF) analysis showed numerous red blood cells, increased proteins and LDH, and high opening pressure. Brain and spine MRI demonstrated extensive superficial siderosis, bone remodeling of the skull base and spine, and diffuse nodular leptomeningeal enhancement. Histological examination of a nodule in the dorsal spine evidenced PA.
CONCLUSION
We report a case of PA associated with dural remodeling and SS. The mechanism of SS is unclear but might be related to meningeal tumor infiltration and altered CSF composition and resorption.
Topics: Astrocytoma; Brain; Female; Humans; Intracranial Hypertension; Magnetic Resonance Imaging; Middle Aged; Siderosis
PubMed: 35460451
DOI: 10.1007/s10072-022-06077-w -
CNS Oncology Nov 2019Pleomorphic xanthoastrocytoma (PXA) is a rare primary CNS tumor. Recent advances in the molecular characterization are helping to define subtypes of tumor. The discovery... (Review)
Review
Pleomorphic xanthoastrocytoma (PXA) is a rare primary CNS tumor. Recent advances in the molecular characterization are helping to define subtypes of tumor. The discovery of mutations within a substantial percentage of PXA fosters a clearer understanding of the pathophysiology of these tumors with clear prognostic and therapeutic implications. These findings are expected to provide insight into the spectrum of clinical behavior observed in PXA, ranging from cure with surgery to diffuse dissemination throughout the neuraxis. This review details the clinical presentation including radiographic appearance of PXA. Pathology, including molecular pathology is discussed. Therapeutic management including surgical resection, radiotherapy and systemic therapies are reviewed.
Topics: Astrocytoma; Brain Neoplasms; Combined Modality Therapy; Humans; Prognosis
PubMed: 31535562
DOI: 10.2217/cns-2019-0009 -
AJNR. American Journal of Neuroradiology Jun 2024T2-FLAIR mismatch is a highly specific imaging biomarker of -mutant diffuse astrocytoma in adults. It has however also been described in -altered low grade tumors. Our...
BACKGROUND AND PURPOSE
T2-FLAIR mismatch is a highly specific imaging biomarker of -mutant diffuse astrocytoma in adults. It has however also been described in -altered low grade tumors. Our aim was to assess the diagnostic power of the T2-FLAIR mismatch in -mutant astrocytoma and -altered low-grade tumors in children and correlate this mismatch with histology.
MATERIALS AND METHODS
We evaluated MR imaging examinations of all pediatric patients, performed at the Princess Máxima Center for Pediatric Oncology and the University Medical Center Utrecht between January 2012 and January 2023, with the histomolecular diagnosis of -mutant astrocytoma, diffuse astrocytoma -altered, or angiocentric glioma, and the presence of T2-FLAIR mismatch was assessed. Histologically, the presence of microcysts in the tumor (a phenomenon suggested to be correlated with T2-FLAIR mismatch in -mutant astrocytomas in adults) was evaluated.
RESULTS
Nineteen pediatric patients were diagnosed with either -mutant astrocytoma ( = 8) or -altered tumor ( = 11: diffuse astrocytoma or -altered = 8; or angiocentric glioma = 3). T2-FLAIR mismatch was present in 11 patients, 3 (38%) in the -mutant group and 8 (73%) in the group. No correlation was found between T2-FLAIR mismatch and the presence of microcysts or an enlarged intercellular space in either -mutant astrocytoma (= .38 and = .56, respectively) or -altered tumors (= .36 and = .90, respectively).
CONCLUSIONS
In our pediatric population, T2-FLAIR mismatch was more often found in -altered tumors than in -mutant astrocytomas. In contrast to what has been reported for -mutant astrocytomas in adults, no correlation was found with microcystic changes in the tumor tissue. This finding challenges the hypothesis that such microcystic changes and/or enlarged intercellular spaces in the tissue of these tumors are an important part of explaining the occurrence of the T2-FLAIR mismatch.
Topics: Humans; Astrocytoma; Child; Brain Neoplasms; Male; Female; Adolescent; Child, Preschool; Glioma; Magnetic Resonance Imaging; Proto-Oncogene Proteins c-myb; Trans-Activators; Biomarkers, Tumor; Isocitrate Dehydrogenase; Infant; Mutation; Retrospective Studies; Proto-Oncogene Proteins
PubMed: 38724203
DOI: 10.3174/ajnr.A8203 -
Scientific Reports Jan 2019Diffuse astrocytoma (including glioblastoma) is morbid with a worse prognosis than other types of glioma. Therefore, we sought to build a progression-associated score to...
Diffuse astrocytoma (including glioblastoma) is morbid with a worse prognosis than other types of glioma. Therefore, we sought to build a progression-associated score to improve malignancy and prognostic predictions for astrocytoma. The astrocytoma progression (AP) score was constructed through bioinformatics analyses of the training cohort (TCGA RNA-seq) and included 18 genes representing distinct aspects of regulation during astrocytoma progression. This classifier could successfully discriminate patients with distinct prognoses in the training and validation (REMBRANDT, GSE16011 and TCGA-GBM Microarray) cohorts (P < 0.05 in all cohorts) and in different clinicopathological subgroups. Distinct patterns of somatic mutations and copy number variation were also observed. The bioinformatics analyses suggested that genes associated with a higher AP score were significantly involved in cancer progression-related biological processes, such as the cell cycle and immune/inflammatory responses, whereas genes associated with a lower AP score were associated with relatively normal nervous system biological processes. The analyses indicated that the AP score was a robust predictor of patient survival, and its ability to predict astrocytoma malignancy was well elucidated. Therefore, this bioinformatics-based scoring system suggested that astrocytoma progression could distinguish patients with different underlying biological processes and clinical outcomes, facilitate more precise tumour grading and possibly shed light on future classification strategies and therapeutics for astrocytoma patients.
Topics: Adult; Aged; Aged, 80 and over; Astrocytoma; Computational Biology; Female; Humans; Machine Learning; Male; Microarray Analysis; Middle Aged; Neoplasm Grading; Pathology, Molecular; Prognosis; Survival Analysis; Young Adult
PubMed: 30643174
DOI: 10.1038/s41598-018-36471-4 -
World Neurosurgery Aug 2018Collision tumors are often difficult to distinguish from intratumoral heterogeneity in diffuse gliomas.
BACKGROUND
Collision tumors are often difficult to distinguish from intratumoral heterogeneity in diffuse gliomas.
CASE DESCRIPTION
We report the case of a 44-year-old woman admitted for intracranial hypertension. Magnetic resonance imaging revealed a right intra-axial frontal mass, composed of a hypervascular nodular portion contrasting with a large nonenhanced infiltrative and muliticystic portion. Histopathologic examination showed the occurrence of two morphologically different gliomas. The largest component corresponded to an anaplastic astrocytoma, IDH1-mutated. The second corresponded to a leptomeningeal nodule, reminiscent of a pleomorphic xanthoastrocytoma. Both tumoral components exhibited anaplastic features, World Health Organization grade III. Immunohistochemical and molecular studies showed that the 2 components were identical, IDH1 R132H mutated but without BRAF V600E mutation. Tumor progression was assessed 2 years after surgery, after radiotherapy and chemotherapy, showing supratentorial leptomeningeal dissemination.
CONCLUSIONS
Collision tumors and combined neoplasms have been rarely described in the brain and only 4 similar articles report the synchronous occurrence of 2 primary gliomas. A review of the literature is proposed, focusing on criteria that could be used to discriminate them.
Topics: Arginine; Astrocytoma; Brain Neoplasms; Histidine; Humans; Isocitrate Dehydrogenase; Magnetic Resonance Imaging; Mutation
PubMed: 29859360
DOI: 10.1016/j.wneu.2018.05.156 -
Seminars in Neurology Feb 2018Estimating the malignancy level of tumors is key to management, and has been part of oncology practice for the past ∼100 years. A central aspect of assessing... (Review)
Review
Estimating the malignancy level of tumors is key to management, and has been part of oncology practice for the past ∼100 years. A central aspect of assessing malignancy level is based on histological "grading"-a process in which a pathologist evaluates microscopic features of a tumor and interprets those findings in light of large prognostic studies. For the diffuse astrocytic gliomas, there have been many such studies over the past century and these have proven useful in estimating prognosis for patients. With the advent of molecular genetics, molecular diagnostic testing has been added to histological evaluation in the armamentarium of the pathologist, and the recent World Health Organization (WHO) Classification of Tumors of the Central Nervous System encourages testing for isocitrate dehydrogenase (IDH) gene status in the classification of diffuse astrocytic gliomas. The present review catalogues a large series of diffuse astrocytic glioma grading studies over the past few decades, and compares the prognostic value of such grading schema before and after the emergence of IDH testing. The review concludes that novel approaches to diffuse astrocytic tumor grading are required in the era of IDH testing.
Topics: Astrocytoma; Brain Neoplasms; Humans; Isocitrate Dehydrogenase; Neoplasm Grading
PubMed: 29548048
DOI: 10.1055/s-0038-1636430 -
Folia Neuropathologica 2016Pilocytic astrocytomas (PAs) are the most frequent primary astroglial tumours affecting children and adolescents. They occur sporadically or in association with a... (Review)
Review
Pilocytic astrocytomas (PAs) are the most frequent primary astroglial tumours affecting children and adolescents. They occur sporadically or in association with a genetically determined syndrome - neurofibromatosis type 1. Classic PA usually manifests as a well-circumscribed, often cystic, slowly growing tumour, which corresponds to WHO grade I. The majority of pilocytic tumours arise along the neuraxis, predominantly in the cerebellum. They are associated with favourable long-term outcome or spontaneous regression, even after incomplete resection. However, the behaviour and prognosis might also be related to tumour histology and location. Pilomyxoid astrocytoma (PMA) represents a variant of classical PA with more invasive growth and increased risk of recurrences and dissemination. Typically, PAs exhibit distinct histology with biphasic architecture of loose, microcystic and compact, fibrillary areas. However, some tumours arise in an uncommon location and display heterogeneous histopathological appearance. The morphological pattern of PA can mimic some other glial neoplasms, including oligodendroglioma, pleomorphic xanthoastrocytoma, ependymoma or diffuse astrocytoma. Not infrequently, the advanced degenerative changes, including vascular fibrosis, and recent and old haemorrhages, may mimic vascular pathology. Sometimes, the neoplastic piloid tissue can resemble reactive gliosis, related to long-standing non neoplastic lesions. Not infrequently, PA exhibits histological features typical for anaplasia, including necrosis, mitoses and glomeruloid vascular proliferation that can suggest a diffuse high-grade glioma. However, even those PAs that lack distinct histological features of anaplasia can behave unpredictably, in a more aggressive manner, with leptomeningeal spreading. Genetic alterations resulting in aberrant signalling of the mitogen-activated protein kinase (MAPK) pathway have been considered to underlie the development of PAs. The most commonly identified KIAA1549-BRAF fusion is important for appropriate tumour molecular diagnosis. In this paper we summarize the clinicopathological presentation of PAs, with emphasis on their heterogeneous morphology, based on our own experience in the field of surgical neuropathology and the literature data. Diagnosis of pilocytic tumours requires careful analysis of clinical, histopathological and molecular features to avoid misinterpretation of these benign neoplastic lesions.
Topics: Animals; Astrocytes; Astrocytoma; Brain Neoplasms; Glioma; Humans; Mitogen-Activated Protein Kinases; Neoplasm Recurrence, Local
PubMed: 27764513
DOI: 10.5114/fn.2016.62530 -
European Spine Journal : Official... Oct 2021Due to the rarity of diffuse spinal cord astrocytoma, an effective model is still lacking to stratify their prognosis. Here, we aimed to establish a prognostic model...
PURPOSE
Due to the rarity of diffuse spinal cord astrocytoma, an effective model is still lacking to stratify their prognosis. Here, we aimed to establish a prognostic model through comprehensively evaluating clinicopathological features and preoperative peripheral blood inflammatory markers in 89 cases.
METHODS
We performed univariate and multivariate Cox regression to identify prognosis factors. The Kaplan-Meier curves and ROC curves were employed to compare the prognostic value of selected factors.
RESULTS
In addition to clinicopathological factors, we revealed the preoperative peripheral blood leukocyte count, neutrophils-to-lymphocytes ratio (NLR), and platelet-to-lymphocyte ratio (PLR) were also significantly correlated with overall survival of spinal cord astrocytoma in univariate Cox regression, and NLR was still significant in multivariate Cox analysis. Further, we demonstrated that NLR ≤ 3.65 and preoperative McCormick score (MMS) ≤ 3 were independently correlated with better survival of WHO grade IV tumors. Meanwhile, Ki-67 < 10% and resection extent ≥ 90% were independent prognostic factors in WHO grade II/III tumors. Finally, we developed a prognostic model that had better predictive efficiencies than WHO grade and histological grade for 1-year (AUC = 76.6), 2- year (AUC = 80.9), and 3-year (AUC = 80.3) survival. This model could classify tumors into 4 classifications with increasingly poor prognosis: 1, WHO grade II/III, with Ki-67 < 10% and resection extent ≥ 90%; 2, WHO grade II/III, Ki-67 ≥ 10% or resection < 90%; 3, WHO grade IV, NLR ≤ 3.65 and MMS ≤ 3; 4, WHO grade IV, with NRL > 3.65 or MMS = 4.
CONCLUSION
We successfully constructed a comprehensive prognostic model including preoperative peripheral blood inflammatory markers, which can stratify diffuse spinal cord astrocytoma into 4 subgroups.
Topics: Astrocytoma; Humans; Lymphocytes; Prognosis; Retrospective Studies; Spinal Cord
PubMed: 33495960
DOI: 10.1007/s00586-021-06724-4 -
BMJ Case Reports Feb 2022
Topics: Adolescent; Astrocytoma; Brain Neoplasms; Ganglioglioma; Humans; Neurofibromatosis 1
PubMed: 35228248
DOI: 10.1136/bcr-2021-248372