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Romanian Journal of Morphology and... 2018Astroblastoma is a poorly defined central nervous system (CNS) tumor, included along with polar spongioblastoma and gliomatosis cerebri in the group of neuroepithelial... (Review)
Review
BACKGROUND
Astroblastoma is a poorly defined central nervous system (CNS) tumor, included along with polar spongioblastoma and gliomatosis cerebri in the group of neuroepithelial tumors of uncertain origin in the June 2016 World Health Organization (WHO) Classification of tumors of the CNS. They are rare neoplasms that affect primarily patients of young ages. The purpose of this research is to highlight the uniqueness and rareness of this pathology and to emphasize on the particularities of one case managed in our Clinic.
CASE DESCRIPTION
We present the case of a 54-year-old patient with a history of seizures since the age of six years old, who presented on admission with progressive worsening and unresponsiveness to treatment, starting six months prior to presentation. Brain imaging shows a right frontal mass compressing neighboring structures. Gross total resection of the tumor was performed, and histopathological examination of the surgical sample together with immunohistochemistry highlighted the presence of a low-grade astroblastoma.
CONCLUSIONS
We summarized data from the literature in order to highlight aspects of this affliction: clinical presentation, imagery, surgical treatment and pathology, hoping that this will aid physicians in finding useful information on this subject, which can guide them to a good outcome. We also discussed differential diagnosis, as this type of tumor shares common features with ependymoma, meningioma, astrocytoma, etc.
Topics: Brain Neoplasms; Cell Proliferation; Female; Humans; Magnetic Resonance Imaging; Middle Aged; Neoplasm Proteins; Neoplasms, Neuroepithelial
PubMed: 30845307
DOI: No ID Found -
Magyar Onkologia 2005Intraocular tumours may be benign or malignant. The latter are more numerous, and endanger not only vision but life as well. Two of them deserve special attention:... (Review)
Review
Intraocular tumours may be benign or malignant. The latter are more numerous, and endanger not only vision but life as well. Two of them deserve special attention: melanoma malignum oculi in adults and retinoblastoma in children. Melanoma malignum may arise from all three areas of the uvea: the iris, the ciliary body and the choroid. The more malignant growths are those which are situated closer to the posterior pole. Histologically the epitheloid cell-type of melanoma is more malignant than those containing only spindle cells. Their treatment depends on the size: in the case of large tumours enucleation is required, while for the smaller ones, radiation therapy can be applied. Retinoblastoma is most common in children of 1-2 years of age. It has familial and sporadic forms. Sixty-seven percent of the inherited-type cases are bilateral. An early symptom in small children is strabismus. A white tissue mass growing into the vitreous is seen on the fundus. A diagnostic feature that can be detected by ultrasound examination is calcification. The tumour may also present intracranially, therefore CT of the skull should be performed in each case. Histologically the tumour contains malignant neuroepithelial cells, which may form a rosette. In the case of large tumours the treatment is enucleation; in bilateral processes the bulbus with the larger mass is removed and the other eye is treated with radiation therapy. In both cases chemotherapy is used according to a prescribed schedule. Metastases to the eye occur most frequently from carcinomas of the breast, lungs or gastrointestinal tract. These are treated with radiotherapy, chemotherapy and hormone therapy. Primary intraocular lymphoma often occurs bilaterally, and may be accompanied by primary lymphoma of the central nervous system (CNS). Some benign tumours are found by chance on routine eye examinations, others due to subjective and objective symptoms.
Topics: Eye Neoplasms; Humans; Lymphoma; Melanoma; Retinal Neoplasms; Retinoblastoma
PubMed: 15902327
DOI: No ID Found -
Journal of Neuro-oncology May 2023Astrocytomas and oligodendrogliomas are mainly diffuse primary brain tumors harboring a diagnostic and prognostically favorable isocitrate dehydrogenase mutation. They... (Review)
Review
PURPOSE
Astrocytomas and oligodendrogliomas are mainly diffuse primary brain tumors harboring a diagnostic and prognostically favorable isocitrate dehydrogenase mutation. They are still incurable besides growing molecular knowledge and therapy options. Circumscribed astrocytomas are also discussed here, although they represent a separate entity despite similarities in the nomenclature.
METHODS
We reviewed clinical trials, preclinical approaches as well as guideline recommendations form the major scientific Neuro-Oncology organizations for astrocytomas and oligodendrogliomas according to PRISMA guidelines.
RESULTS
After histopathological diagnosis and eventually a maximal safe resection, patients with good prognostic factors may be followed by magnetic resonance imaging (MRI). If further treatment is necessary, either after diagnosis or at progression, diffuse astrocytomas and oligodendrogliomas are mainly treated with combined radiochemotherapy or maximal safe resection followed by combined radiochemotherapy according to current guidelines based on randomized trials. Circumscribed gliomas like pilocytic astrocytomas, CNS WHO grade 1, or pleomorphic xanthoastrocytomas, CNS WHO grade 2, are often treated with surgery alone. Current approaches for therapy optimization include decision of the best chemotherapy regimen. The IDH mutation presents a rational target for small molecule inhibition and immune therapy in diffuse astrocytomas and oligodendrogliomas, while the BRAF pathway is frequently mutated and treatable in circumscribed gliomas.
CONCLUSION
Despite establishment of standard treatment approaches for gliomas that include resection, radio- and chemotherapy, there is a lack of effective treatments for progressive disease. Immune- and targeted therapies are currently investigated.
Topics: Humans; Oligodendroglioma; Astrocytoma; Glioma; Magnetic Resonance Imaging; Mutation
PubMed: 36566461
DOI: 10.1007/s11060-022-04216-z -
Indian Journal of Pathology &... May 2022The newest revision of the WHO classification of tumors of the central nervous system, also known as WHO 5 edition, introduces substantial changes, especially within the... (Review)
Review
The newest revision of the WHO classification of tumors of the central nervous system, also known as WHO 5 edition, introduces substantial changes, especially within the glial tumor category and separates adult-type and pediatric-type glial tumors into different categories for the first time. In addition, another category of glial tumors, "Circumscribed Astrocytic Gliomas" were also created. This group includes pilocytic astrocytoma, pleomorphic xanthoastrocytoma, subependymal giant cell astrocytoma, chordoid glioma, astroblastoma, and the highly nebulous novel entity high-grade astrocytoma with piloid features. We present a brief and critical review of the pathological and molecular characteristics of these often well-demarcated tumors that can occur in adults as well as in the pediatric population.
Topics: Adult; Astrocytoma; Brain Neoplasms; Child; Glioma; Humans; Precancerous Conditions; World Health Organization
PubMed: 35562132
DOI: 10.4103/ijpm.ijpm_1019_21 -
Acta Neuropathologica Communications Jan 2023Gliomas with FGFR3::TACC3 fusion mainly occur in adults, display pathological features of glioblastomas (GB) and are usually classified as glioblastoma, IDH-wildtype....
BACKGROUND
Gliomas with FGFR3::TACC3 fusion mainly occur in adults, display pathological features of glioblastomas (GB) and are usually classified as glioblastoma, IDH-wildtype. However, cases demonstrating pathological features of low-grade glioma (LGG) lead to difficulties in classification and clinical management. We report a series of 8 GB and 14 LGG with FGFR3:TACC3 fusion in order to better characterize them.
METHODS
Centralized pathological examination, search for TERT promoter mutation and DNA-methylation profiling were performed in all cases. Search for prognostic factors was done by the Kaplan-Meir method.
RESULTS
TERT promoter mutation was recorded in all GB and 6/14 LGG. Among the 7 cases with a methylation score > 0.9 in the classifier (v12.5), 2 were classified as glioblastoma, 4 as ganglioglioma (GG) and 1 as dysembryoplastic neuroepithelial tumor (DNET). t-SNE analysis showed that the 22 cases clustered into three groups: one included 12 cases close to glioblastoma, IDH-wildtype methylation class (MC), 5 cases each clustered with GG or DNET MC but none with PLNTY MC. Unsupervised clustering analysis revealed four groups, two of them being clearly distinct: 5 cases shared age (< 40), pathological features of LGG, lack of TERT promoter mutation, FGFR3(Exon 17)::TACC3(Exon 10) fusion type and LGG MC. In contrast, 4 cases shared age (> 40), pathological features of glioblastoma, and were TERT-mutated. Relevant factors associated with a better prognosis were age < 40 and lack of TERT promoter mutation.
CONCLUSION
Among gliomas with FGFR3::TACC3 fusion, age, TERT promoter mutation, pathological features, DNA-methylation profiling and fusion subtype are of interest to determine patients' risk.
Topics: Adult; Humans; Child; Glioblastoma; Mutation; Glioma; Brain Neoplasms; Prognosis; Ganglioglioma; Epigenesis, Genetic; DNA; Isocitrate Dehydrogenase; Receptor, Fibroblast Growth Factor, Type 3; Microtubule-Associated Proteins
PubMed: 36647073
DOI: 10.1186/s40478-023-01506-z -
Head and Neck Pathology Jun 2023Although the definitions of sinonasal neuroendocrine and neuroectodermal neoplasms did not change substantially in the 5th edition WHO Classification of Head and Neck... (Review)
Review
Proceedings of the 2023 North American Society of Head and Neck Pathology Companion Meeting, New Orleans, LA, March 12, 2023: Navigating New Developments in High Grade Sinonasal Neuroendocrine and Neuroectodermal Neoplasms.
Although the definitions of sinonasal neuroendocrine and neuroectodermal neoplasms did not change substantially in the 5th edition WHO Classification of Head and Neck Tumours, the diagnosis of olfactory neuroblastoma (ONB), small cell neuroendocrine carcinoma, and large cell neuroendocrine carcinoma remains quite challenging in practice. Ambiguities surrounding the amount of keratin expression allowable in ONB and the amount of neuroendocrine differentiation seen in sinonasal undifferentiated carcinoma (SNUC) lead to significant diagnostic discrepancies at the high grade end of this tumor spectrum. Furthermore, a group of problematic neuroepithelial tumors that show overlapping features of ONB and neuroendocrine carcinoma have never been recognized in formal classification schemes. Since publication of the 5th edition WHO, two new tumor entities have been proposed that help resolve these problems. Olfactory carcinoma is defined by high grade keratin-positive neuroectodermal cells with frequent intermixed glands and shows recurrent Wnt pathway, ARID1A, and RUNX1 alterations. IDH2-mutant sinonasal carcinoma is a molecularly-defined category that encompasses tumors with undifferentiated (SNUC), large cell neuroendocrine, and neuroepithelial phenotypes. This review will provide a practical overview of these emerging entities and their application to diagnostic challenges in the post-WHO sinonasal neuroendocrine and neuroectodermal tumor classification.
Topics: Humans; New Orleans; Carcinoma, Neuroendocrine; Paranasal Sinus Neoplasms; Maxillary Sinus Neoplasms; Esthesioneuroblastoma, Olfactory; Keratins; Nose Neoplasms; Nasal Cavity
PubMed: 37184733
DOI: 10.1007/s12105-023-01548-8 -
The Mount Sinai Journal of Medicine,... 2010Malignant gliomas are the most common primary brain tumors in adults and carry a dismal prognosis. Despite aggressive therapy with maximal safe surgical resection,... (Review)
Review
Malignant gliomas are the most common primary brain tumors in adults and carry a dismal prognosis. Despite aggressive therapy with maximal safe surgical resection, radiation and chemotherapy, these tumors invariably are refractory to or become resistant to treatment and recur. Gliomas are highly infiltrative cancers and display remarkable genetic heterogeneity making them challenging to treat. Recent progress has been made in understanding the molecular and genetic composition of these tumors and from this, promising new targets for therapy have emerged. In particular, anti-angiogenesis therapies have led to modest success in disease control. In addition, the growing body of research in cancer immunology as well as cancer stem cells has made inroads in our understanding of tumorgenesis. Translational research has been particularly crucial to the development of these therapies as much preclinical and clinical work is needed to develop the rationale for treatments, to develop biomarkers of drug activity and to elucidate mechanisms of resistance. This brief overview will discuss some of the pivotal advances made in the pursuit of improved outcomes and survival for patients with this devastating disease.
Topics: Epigenomics; Glioblastoma; Glioma; Humans; Immunotherapy; Neovascularization, Pathologic; Signal Transduction; Stem Cell Transplantation; Translational Research, Biomedical; Vaccines, Subunit
PubMed: 21105127
DOI: 10.1002/msj.20223 -
Cancer Research Dec 2018Glioblastoma (GBM) is a highly aggressive form of cancer that is resistant to standard therapy with concurrent radiation and temozolomide, two agents that work by... (Review)
Review
Glioblastoma (GBM) is a highly aggressive form of cancer that is resistant to standard therapy with concurrent radiation and temozolomide, two agents that work by inducing DNA damage. An underlying cause of this resistance may be a subpopulation of cancer stem-like cells that display a heightened DNA damage response (DDR). Although this DDR represents an attractive therapeutic target for overcoming the resistance of GBMs to radiotherapy, until now, the cause of this DDR upregulation has not been understood. In a previous issue of , Carruthers and colleagues investigated DNA replication stress as an underlying mechanism responsible for upregulation of the DDR and hence the radiation resistance of glioma stem-like cells. Furthermore, the authors explore the efficacy of combined ataxia telangiectasia and Rad3-related kinase and PARP inhibitors as a strategy to leverage these mechanisms and overcome radiation resistance..
Topics: Cell Line, Tumor; DNA Damage; Glioblastoma; Glioma; Humans; Neoplastic Stem Cells
PubMed: 30498082
DOI: 10.1158/0008-5472.CAN-18-2439 -
The British Journal of Radiology Feb 2022The peritoneum is a unique serosal membrane, which can be the site of primary tumors and, more commonly, secondary pathologic processes. Peritoneal carcinomatosis is the... (Review)
Review
The peritoneum is a unique serosal membrane, which can be the site of primary tumors and, more commonly, secondary pathologic processes. Peritoneal carcinomatosis is the most common malignant condition to affect the peritoneal cavity, and the radiologist plays an important role in making the diagnosis and assessing the extent of disease, especially in sites that may hinder surgery. In this review, we address the role of the radiologist in the setting of peritoneal pathology, focusing on peritoneal carcinomatosis as this is the predominant malignant process, followed by revising typical imaging findings that can guide the differential diagnosis.We review the most frequent primary and secondary peritoneal tumor and tumor-like lesions, proposing a systemic approach based on clinical history and morphological appearance, namely distinguishing predominantly cystic from solid lesions, both solitary and multiple.
Topics: Ascites; Ascitic Fluid; Carcinoma; Desmoplastic Small Round Cell Tumor; Diagnosis, Differential; Echinococcosis; Endometriosis; Female; Humans; Lymphangioma; Lymphoma; Magnetic Resonance Imaging; Mesothelioma; Neoplasms, Neuroepithelial; Peritoneal Neoplasms; Peritoneum; Peritonitis; Positron Emission Tomography Computed Tomography; Pseudomyxoma Peritonei; Splenosis; Tomography, X-Ray Computed
PubMed: 34767464
DOI: 10.1259/bjr.20210346 -
Journal of Cellular Physiology Dec 2008Over the past decades considerable advances have been made in neurosurgery, radiotherapy and chemotherapy resulting in improved survival and cure rates for children with... (Review)
Review
Over the past decades considerable advances have been made in neurosurgery, radiotherapy and chemotherapy resulting in improved survival and cure rates for children with brain tumors. Here we review four of the most common subtypes of pediatric brain tumors, low-grade and high-grade astrocytomas, medulloblastomas and ependymomas, highlighting their molecular features regarding their tumor biology, and promising potential therapeutic targets that may hold promise for finding new "molecular targeted" drugs. Importantly, appropriate clinical trial design will play a critical role in the evaluation of new and novel treatment approaches for pediatric brain tumors.
Topics: Astrocytoma; Brain Neoplasms; Child; Clinical Trials as Topic; Ependymoma; Glioma; Humans; Medulloblastoma
PubMed: 18651562
DOI: 10.1002/jcp.21544