Natural Course and Prognosis of Primary Spinal Glioblastoma

Amelot, Aymeric; Terrier, Louis-Marie; Mathon, Bertrand; Joubert, Christophe; Picart, Thiébaud; Jecko, Vincent; Bauchet, Luc; Bernard, Florian; Castel, Xavier; Chenin, Louis; Cook, Ann-Rose; Emery, Évelyne; Dominique, Figarella-Branger; Gauchotte, Guillaume; Graillon, Thomas; Jouvet, Anne; Kalamaridès, Michel; Knafo, Steven; Lazard, Arnaud; Lubrano, V.; Mokhtari, Karima; Rigau, Valérie; Vincent, Roualdes; Rousseau, Audrey; Seizeur, Romuald; Uro‐Coste, Emmanuelle; Voirin, Jimmy; Philippe, Metellus; Pallud, Johan; Zemmoura, Ilyess

doi:10.1212/wnl.0000000000206834

Cited by 5 publications

(2 citation statements)

References 40 publications

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“…We processed the FBTDB and, conducted a nationwide population-based study of all histopathologically con rmed meningioma in France. Detailed methodology has previously been published [6][7][8][9][10][11][12]. In summary, FBTDB collects and records data of patients operated on for a PCNST.…”

Section: Methodsmentioning

confidence: 99%

WITHDRAWN: Descriptive epidemiology of 30,223 histopathologically confirmed meningiomas in France: 2006–2015

Depond,

Zouaoui,

Darlix

et al. 2023

Preprint

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Purpose To describe the epidemiology of meningioma operated in France between 2006 and 2015. Methods We processed the French Brain Tumour Database (FBTDB) to conduct a nationwide population-based study of all histopathologically confirmed meningioma in France. Descriptive methods were implemented. Results 30,223 meningiomas cases were operated on 28,424 patients, in 61 centres. Meningioma was 3 times more common in women (74.1% vs. 25.9%). Mean age at surgery was 58.5±13.9 years; 57.9±13.5 years for women vs.59.9 ±14.8 years for men (p<0.001). The incidence of meningioma surgery increased with age. Grade 1, 2, and 3 meningiomas accounted for 83.9%, 13.91% and, 2.19% respectively. The proportion of grade 2 significantly grew over the study period (p<0.001). There was a significant variability of meningioma grading by institutions, especially for grade 2 which spanned from 5.1% up to 22.4% (p <0.001). 89.05% of the patients had solely one meningioma surgery, 8.52% two and, 2.43% three or more. The number of surgeries was associated with the grade of malignancy (p <0.001). Conclusion The FBTBD is an effective source that comforts previous studies and, bring new information from 30,223 meningiomas cases operated on over a 10-year-long period. Three quarters of meningioma were removed in women. The incidence of meningioma surgery increased with age and, peaked at 58.5 years. They were predominantly benign with meningothelial subtype being the most common. However, the rate of grade 2 has significantly grown over the time, on contrary to malignant meningioma proportion, which remained rare and stable over time around 2%.

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Section: Methodsmentioning

confidence: 99%

WITHDRAWN: Descriptive epidemiology of 30,223 histopathologically confirmed meningiomas in France: 2006–2015

Depond,

Zouaoui,

Darlix

et al. 2023

Preprint

Self Cite

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“…A French nationwide study reported a median OS time of 13.1 months and a median PFS time of 8.0 months for primary spinal GBM among a generally older population (mean age: 37 years, standard deviation (SD): 16.5). Remarkably, patient #8 achieved an OS time of 22 months post-larotrectinib treatment, significantly exceeding the OS time observed in the French study [ 1 ].…”

Section: Discussionmentioning

confidence: 68%

NTRK-fused central nervous system tumours: clinicopathological and genetic insights and response to TRK inhibitors

Kim,

Park,

Kim

et al. 2024

acta neuropathol commun

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Background Neurotrophic tropomyosin receptor kinase (NTRK) gene fusions are found in 1% of gliomas across children and adults. TRK inhibitors are promising therapeutic agents for NTRK-fused gliomas because they are tissue agnostic and cross the blood–brain barrier (BBB). Methods We investigated twelve NGS-verified NTRK-fused gliomas from a single institute, Seoul National University Hospital. Results The patient cohort included six children (aged 1–15 years) and six adults (aged 27–72 years). NTRK2 fusions were found in ten cerebral diffuse low-grade and high-grade gliomas (DLGGs and DHGGs, respectively), and NTRK1 fusions were found in one cerebral desmoplastic infantile ganglioglioma and one spinal DHGG. In this series, the fusion partners of NTRK2 were HOOK3, KIF5A, GKAP1, LHFPL3, SLMAP, ZBTB43, SPECC1L, FKBP15, KANK1, and BCR, while the NTRK1 fusion partners were TPR and TPM3. DLGGs tended to harbour only an NTRK fusion, while DHGGs exhibited further genetic alterations, such as TERT promoter/TP53/PTEN mutation, CDKN2A/2B homozygous deletion, PDGFRA/KIT/MDM4/AKT3 amplification, or multiple chromosomal copy number aberrations. Four patients received adjuvant TRK inhibitor therapy (larotrectinib, repotrectinib, or entrectinib), among which three also received chemotherapy (n = 2) or proton therapy (n = 1). The treatment outcomes for patients receiving TRK inhibitors varied: one child who received larotrectinib for residual DLGG maintained stable disease. In contrast, another child with DHGG in the spinal cord experienced multiple instances of tumour recurrence. Despite treatment with larotrectinib, ultimately, the child died as a result of tumour progression. An adult patient with glioblastoma (GBM) treated with entrectinib also experienced tumour progression and eventually died. However, there was a successful outcome for a paediatric patient with DHGG who, after a second gross total tumour removal followed by repotrectinib treatment, showed no evidence of disease. This patient had previously experienced relapse after the initial surgery and underwent autologous peripheral blood stem cell therapy with carboplatin/thiotepa and proton therapy. Conclusions Our study clarifies the distinct differences in the pathology and TRK inhibitor response between LGG and HGG with NTRK fusions.

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Astrocytomas of the spinal cord

Tonn,

Teske,

Karschnia

2024

Neuro-Oncology Advances

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Tumors of astrocytic origin represent one of most frequent entities among the overall rare group of spinal cord gliomas. Initial clinical symptoms are often unspecific, and sensorimotor signs localizing to the spinal cord occur with progressing tumor growth. On MRI, a hyperintense intrinsic spinal cord signal on T2-weighted sequences with varying degree of contrast enhancement raises suspicion for an infiltrative neoplasm. Blood and CSF analysis serves to exclude an infectious process, nutritional deficits, or metabolic disorders. When such other differential diagnoses have been ruled out, a neuropathological tissue-based analysis is warranted to confirm the diagnosis of a spinal cord astrocytoma and guide further patient management. As such, maximal safe resection forms the basis of any treatment. Meticulous pre-operative planning is necessary to weight the potential improvement in survival against the risk of functional deterioration. Intraoperative neuromonitoring and ultrasound may aid in achieving a more extensive resection. Depending on the assigned WHO tumor grade spanning from grade 1 to grade 4, the use of radiotherapy and chemotherapy might be indicated but also wait-and-scan approaches appear reasonable in tumors of lower grade. Close imaging follow-up is necessary given that recurrence inevitably occurs in astrocytomas of grade 2-4. Prognosis is so far dictated by tumor grade and histopathological findings, but also by age and clinical performance of the patient. Targeted therapies resting upon an in-depth tissue analysis are emerging in recurrent tumors, but no prospective study is available to far given the rarity of spinal cord astrocytomas.

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Natural Course and Prognosis of Primary Spinal Glioblastoma

Cited by 5 publications

References 40 publications

WITHDRAWN: Descriptive epidemiology of 30,223 histopathologically confirmed meningiomas in France: 2006–2015

WITHDRAWN: Descriptive epidemiology of 30,223 histopathologically confirmed meningiomas in France: 2006–2015

NTRK-fused central nervous system tumours: clinicopathological and genetic insights and response to TRK inhibitors

Astrocytomas of the spinal cord

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