Rare Neuronal, Glial and Glioneuronal Tumours in Adults

Crainic, Nicolas; Furtner, Julia; Pallud, Johan; Bielle, Franck; Lombardi, Giuseppe; Rudà, Roberta; Idbaïh, Ahmed

doi:10.3390/cancers15041120

Cited by 3 publications

(2 citation statements)

References 168 publications

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“…Glioneuronal tumors are uncommon primary central nervous system (CNS) tumors occurring during childhood and young adulthood with an estimated incidence of 0.4% of all CNS tumors, consisting of a mixture of well-differentiated neoplastic neural and glial components [ 1 ]. Ganglioma and Dysembryoplastic neuroepithelial tumors (DNET) represent the majority of this glioneuronal group, and descriptions of many other newly classified lesions (MVNT, DGONC, MGT and others) are limited to case reports and small case series [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

“…Ganglioma and Dysembryoplastic neuroepithelial tumors (DNET) represent the majority of this glioneuronal group, and descriptions of many other newly classified lesions (MVNT, DGONC, MGT and others) are limited to case reports and small case series [ 2 ]. According to the previous 2016 WHO classification, these tumors were classified into three grades: grade I (benign or low-grade tumors), grade II (atypical tumors) and grade III (anaplastic tumors) [ 1 ]. In the WHO CNS5 revision published in 2021, all tumors with a neuronal component remain grouped together, and three new types have been added: DGONC (provisional) tumor, myxoid glioneuronal tumor and multinodular and vacuolating neuronal tumor [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

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Infratentorial Relapsing Neuroglial Tumors in Adults: Management and Unsolved Issues—A Systematic Review

Brunasso,

Avallone,

Florena

et al. 2024

Brain Sciences

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(1) Background: Gangliogliomas are rare tumors accounting for about 0.4% of all central nervous system tumors. They are usually located in the temporal lobes of children and young adults, though such tumors in the infratentorial region and adult-age patients rarely reported. (2) Methods: A systematic review on ganglioglioma with infratentorial location in the adult population was conducted in accordance with the PRISMA guidelines. A total of 275 articles were found, and 23 were included. Demographic data, the location and histology of the lesion, pre-operative neurological status, the type of surgery, recurrence, radiotherapy/chemotherapy adjuvant treatments, neurological outcomes and follow-up information were collected. We also presented an illustrative case. (3) Results: A total of 27 patients were included. In 51%, the location was the cerebellum; in 40%, it was the fourth ventricle; in 11%, it was brainstem; and in 4%, it was the cerebellopontine angle. STR was performed in 44%, GTR in 26% and biopsy in 15% of the cases. Adjuvant radiotherapy was found in 22% of cases. Disease recurrence occurred in 15% of patients between 1 and 12 months after surgery with a diagnosis of high-grade ganglioglioma, while in six cases, no disease recurrence was documented. (4) Conclusions: Infratentorial glioneuronal tumors are rare findings in the adult population. Histopathological characterization does not seem to fully reflect their true behavior. Future studies are warranted for better characterizing histopathological findings and treatment.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Infratentorial Relapsing Neuroglial Tumors in Adults: Management and Unsolved Issues—A Systematic Review

Brunasso,

Avallone,

Florena

et al. 2024

Brain Sciences

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The Role of Radiotherapy, Chemotherapy, and Targeted Therapies in Adult Intramedullary Spinal Cord Tumors

Esparragosa Vazquez,

Ducray

2024

Cancers

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Intramedullary primary spinal cord tumors are rare in adults and their classification has recently evolved. Their treatment most frequently relies on maximal safe surgical resection. Herein, we review, in light of the WHO 2021 classification of central nervous system tumors, the knowledge regarding the role of radiotherapy and systemic treatments in spinal ependymomas, spinal astrocytomas (pilocytic astrocytoma, diffuse astrocytoma, spinal glioblastoma IDH wildtype, diffuse midline glioma H3-K27M altered, and high-grade astrocytoma with piloid features), neuro-glial tumors (ganglioglioma and diffuse leptomeningeal glioneuronal tumor), and hemangioblastomas. In spinal ependymomas, radiotherapy is recommended for incompletely resected grade 2 tumors, grade 3 tumors, and recurrent tumors not amenable to re-surgery. Chemotherapy is used in recurrent cases. In spinal astrocytomas, radiotherapy is recommended for incompletely resected grade 2 astrocytomas and grade 3 or 4 tumors as well as recurrent tumors. Chemotherapy is indicated for newly diagnosed high-grade astrocytomas and recurrent cases. In hemangioblastomas not amenable to surgery, radiotherapy is an effective alternative option. Targeted therapies are playing an increasingly important role in the management of some intramedullary primary spinal cord tumor subtypes. BRAF and/or MEK inhibitors have demonstrated efficacy in pilocytic astrocytomas and glioneuronal tumors, belzutifan in von Hippel–Lindau-related hemangioblastomas, and promising results have been reported with ONC201 in diffuse midline glioma H3-K27M altered.

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Subependymal Giant Cell Astrocytoma: The Molecular Landscape and Treatment Advances

Pucko,

Sulejczak,

Ostrowski

2024

Cancers

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Subependymal giant cell astrocytoma (SEGA) is most often found in patients with TSC (Tuberous Sclerosis Complex). Although it has been classified as a benign tumor, it may create a serious medical problem leading to grave consequences, including young patient demise. Surgery and chemotherapy belong to the gold standard of treatment. A broader pharmacological approach involves the ever-growing number of rapalogs and ATP-competitive inhibitors, as well as compounds targeting other kinases, such as dual PI3K/mTOR inhibitors and CK2 kinase inhibitors. Novel approaches may utilize noncoding RNA-based therapeutics and are extensively investigated to this end. The purpose of our review was to characterize SEGA and discuss the latest trends in the diagnosis and therapy of this disease.

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Rare Neuronal, Glial and Glioneuronal Tumours in Adults

Cited by 3 publications

References 168 publications

Infratentorial Relapsing Neuroglial Tumors in Adults: Management and Unsolved Issues—A Systematic Review

Infratentorial Relapsing Neuroglial Tumors in Adults: Management and Unsolved Issues—A Systematic Review

The Role of Radiotherapy, Chemotherapy, and Targeted Therapies in Adult Intramedullary Spinal Cord Tumors

Subependymal Giant Cell Astrocytoma: The Molecular Landscape and Treatment Advances

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