Rare glial tumors

Soffietti, Riccardo; Rudà, Roberta; Reardon, David A.

doi:10.1016/b978-0-12-802997-8.00024-4

Cited by 13 publications

(14 citation statements)

References 161 publications

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“…However, an ependymal or tanycyte-derived origin of astroblastoma is considered by many authors based on the electron microscopic features [13,14]. Tanycytes are suggested to be glial precursor cells that may occur during normal human embryogenesis, which explains the existence of congenital astroblastoma [15,16]. Astroblastoma is often mixed with other types of tumor cells such as glioblastomas or anaplastic astrocytomas, and the pseudo-chrysanthemum cluster structure also appears in other tumors, leading to controversy about its existence [17].…”

Section: Discussionmentioning

confidence: 99%

Cerebral astroblastoma with oligodendroglial-like cells：A case report

Gu¹,

Yu²,

Xu³

et al. 2020

Preprint

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Background: Astroblastoma is a rare tumor of the central nervous system, with unclear biological behavior and origin. Its histopathological features have been well established. However, to the best of our knowledge, astroblastoma with oligodendroglial-like cells have not been reported. Case presentation: Herein, we reported a case of astroblastoma with atypical pathological features. A 15-year-old girl presented with nausea, vomiting, headache and visual disturbance. Magnetic resonance imaging (MRI) revealed a large neoplasm in the left temporal lobe. Histologically, the tumor showed solid and pseudopapillary structure. The tumor cells were radially arranged around blood vessels, with a single, prominent process, and as astroblastic pseudorosettes. More importantly, typical oligodendroglial-like cells were observed. In addition to membrane staining for EMA, immunohistochemical staining also showed that the tumor cells were positive for GFAP and Vimentin. The oligodendroglial-like cells were positive for GFAP, Vimentin, and Olig-2. The Ki-67 labeling index was about 4%. Sequencing for IDH1 codon 132 and IDH2 codon 172 gene mutations showed negative results. Furthermore, fluorescent analysis revealed neither 1p nor 19q deletion in the lesion. Based on these findings, the tumor was finally diagnosed as astroblastoma. Conclusions: Herein, we reported an extremely rare case of astroblastoma, which was morphologically characterized by the appearance of oligodendroglial-like cells.

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Section: Discussionmentioning

confidence: 99%

Cerebral astroblastoma with oligodendroglial-like cells：A case report

Gu¹,

Yu²,

Xu³

et al. 2020

Preprint

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“…17,33,39 Males are more commonly affected by DIA/DIG than females (1.5–1.7:1), and patients almost always present with rapidly enlarging head circumference and, sometimes, vomiting and seizures. 3,17,29,34 Classically, DIA/DIGs have been described as massive cystic lesions that emanate from firm nodules that are invariably supratentorial and often attached to the overlying dura. 2–4,8,13,23,36,39 The tumor nodules tend to be homogeneously enhancing, hypodense on T2-weighted imaging, and are not associated with significant vasogenic edema.…”

Section: Discussionmentioning

confidence: 99%

“…6,17,22,38,39 Significant progress has been made in understanding the molecular and genetic pathways underlying DIA/DIGs, yet the genetic and molecular pathways and the cellular origin of these tumors is not yet established. 26,29 A few studies have investigated this issue by interrogating known tumor suppressor genes and oncogenes using immunohistochemistry, chromogenic in situ hybridization, or genome-wide DNA copy number analysis with multiplex ligation-dependent probe amplification. However, because of the rarity of DIA/DIG, these studies lack sufficient sample size to be extrapolated to entire patient populations.…”

Section: Discussionmentioning

confidence: 99%

“…1,29,33,34,39 While both tumors are characterized histologically by prominent desmoplastic stroma, DIA is characterized by a neoplastic population restricted to astrocytes, while DIG includes a population of neoplastic neuronal cells of variable maturity. In light of reportedly similar clinical, radiological, and pathological features, DIG and DIA are considered to be part of the same entity.…”

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confidence: 99%

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Novel suprasellar location of desmoplastic infantile astrocytoma and ganglioglioma: a single institution’s experience

Naylor¹,

Wohl²,

Raghunathan³

et al. 2018

Journal of Neurosurgery: Pediatrics

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OBJECTIVEThe aim of this study was to describe the clinical presentation, imaging appearance, and differential outcomes based on tumor location in 7 patients with desmoplastic infantile astrocytoma and desmoplastic infantile gangliogliomas (DIA/DIG).METHODSData of 7 patients with histopathology-proven DIA/DIGs and preoperative imaging were retrospectively reviewed, and age, sex, clinical presentation, imaging characteristics, tumor location, surgical procedure, postoperative morbidity, and overall mortality were recorded.RESULTSTwo subgroups of patients with DIA/DIGs were found to exist based on whether their tumor was located in the cerebral hemispheres or suprasellar region. Nearly all patients presented with rapidly enlarging head circumference regardless of tumor location. However, ocular abnormalities, including nystagmus and preference for downward gaze, were specific for patients with suprasellar disease. These patients experienced significant postoperative complications and had poor long-term outcomes. In contrast, patients with hemispheric tumors underwent more extensive resection than patients with suprasellar tumors, had uneventful postoperative courses, and had no documented long-term comorbidities.CONCLUSIONSPostoperative course and long-term outcome for patients with DIA/DIGs were correlated to the anatomical location and radiographic appearance of their tumor at presentation, despite having histologically and molecularly indistinguishable, WHO grade I tumors.

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“…As for management ( 60 ), GTR, when feasible, is the standard of care but frequently a local progression occurs. Eighty percent of patients undergo reoperation alone or followed by radiotherapy, while 20% of patients receive radiotherapy alone.…”

Section: Glioneuronal Tumors and Rare Glial Tumorsmentioning

confidence: 99%

Rare Primary Central Nervous System Tumors in Adults: An Overview

et al. 2020

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Overall, tumors of primary central nervous system (CNS) are quite common in adults with an incidence rate close to 30 new cases/100,000 inhabitants per year. Significant clinical and biological advances have been accomplished in the most common adult primary CNS tumors (i.e., diffuse gliomas). However, most CNS tumor subtypes are rare with an incidence rate below the threshold defining rare disease of 6.0 new cases/100,000 inhabitants per year. Close to 150 entities of primary CNS tumors have now been identified by the novel integrated histomolecular classification published by the World Health Organization (WHO) and its updates by the c-IMPACT NOW consortium (the Consortium to Inform Molecular and Practical Approaches to CNS Tumor Taxonomy). While these entities can be better classified into smaller groups either by their histomolecular features and/or by their location, assessing their treatment by clinical trials and improving the survival of patients remain challenging. Despite these tumors are rare, research, and advances remain slower compared to diffuse gliomas for instance. In some cases (i.e., ependymoma, medulloblastoma) the understanding is high because single or few driver mutations have been defined. The European Union has launched European Reference Networks (ERNs) dedicated to support advances on the clinical side of rare diseases including rare cancers. The ERN for rare solid adult tumors is termed EURACAN. Within EURACAN, Domain 10 brings together the European patient advocacy groups (ePAGs) and physicians dedicated to improving outcomes in rare primary CNS tumors and also aims at supporting research, care and teaching in the field. In this review, we discuss the relevant biological and clinical characteristics, clinical management of patients, and research directions for the following types of rare primary CNS tumors: medulloblastoma, pineal region tumors, glioneuronal and rare glial tumors, ependymal tumors, grade III meningioma and mesenchymal tumors, primary central nervous system lymphoma, germ cell tumors, spinal cord tumors and rare pituitary tumors.

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Rare glial tumors

Cited by 13 publications

References 161 publications

Cerebral astroblastoma with oligodendroglial-like cells：A case report

Cerebral astroblastoma with oligodendroglial-like cells：A case report

Novel suprasellar location of desmoplastic infantile astrocytoma and ganglioglioma: a single institution’s experience

Rare Primary Central Nervous System Tumors in Adults: An Overview

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