Pediatric Chordomas: Results of a Multicentric Study of 40 Children and Proposal for a Histopathological Prognostic Grading System and New Therapeutic Strategies

Tauziède‐Espariat, Arnault; Monnien, Franck; Adle‐Biassette, Homa; Masliah‐Planchon, Julien; Maillot, Laetitia; Polivka, Marc; Laquerrière, Annie; Bouillot-Eimer, Sandrine; Gimbert, Édouard; Gauchotte, Guillaume; Coffinet, L.; Sevestre, Henri; Alapetite, Claire; Bolle, Stéphanie; Thompson, Dominic; Bouazza, S.; George, Bernard; Zérah, Michel; Sainte‐Rose, Christian; Puget, Stéphanie; Varlet, Pascale

doi:10.1093/jnen/nlx118

Cited by 18 publications

(22 citation statements)

References 52 publications

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“…We found no signi cant difference, which is consistent with several studies [32,2]. Several previous studies showed that dural penetration [37,7] and age [29,5] were associated with long-term outcomes, and we demonstrated that they were not independent risk factors after multivariate analysis [23,36].…”

Section: Other Risk Factors For Outcomesupporting

confidence: 92%

Long Follow-up Surgical Results in 284 Cases of Clival Chordomas: The Risk Factors for Outcome and Tumor Recurrence

bai

Shi

et al. 2021

Preprint

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OBJECTIVE: Skull-base chordoma (SBC) is rare and one of the most challenging diseases to treat. We aimed to assess the optimal timing of adjuvant radiation therapy (RT) and evaluate the factors that influence resection and long-term outcomes.METHODS: In total, 284 patients with 382 surgeries were enrolled in this retrospective study. Postsurgically, 64 patients underwent RT before recurrence (pre-recurrence RT), and 47 patients underwent RT after recurrence. During the first attempt to achieve gross-total resection (GTR), when the entire tumor was resected, 268 patients were treated with an endoscopic midline approach, and 16 patients were treated with microscopic lateral approaches. Factors associated with the success of GTR were identified using c2 and logistic regression analyses. Risk factors associated with chordoma-specific survival (CSS) and progression-free survival (PFS) were evaluated with the Cox proportional hazards model.RESULTS: In total, 74.6% of tumors were marginally resected [GTR (40.1%); near-total resection (34.5%)]. History of surgery, large tumor volumes and tumor locations in the lower clivus were associated with a lower GTR rate. The mean follow-up period was 43.9 months. At last follow-up, 181 (63.7%) patients were alive. RT history, histologic subtype (dedifferentiated and sarcomatoid), non-GTR, no postsurgical RT, and the presence of metastasis were associated with poorer CSS. Patients with pre-recurrence RT had the longest PFS and CSS, while patients without postsurgical RT had the worst outcome.CONCLUSION: GTR is the goal of initial surgical treatment. Pre-recurrence RT would improve outcome regardless of GTR.

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Section: Other Risk Factors For Outcomesupporting

confidence: 92%

Long Follow-up Surgical Results in 284 Cases of Clival Chordomas: The Risk Factors for Outcome and Tumor Recurrence

bai

Shi

et al. 2021

Preprint

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“…Chordomas represent approximately 0.2% of primary brain tumours 227 . The cumulative incidence is 1:1,000,000, with different sex predominance among the series 227‐229,233‐241 . Chordomas usually occur in Caucasian 228,229 adults between the ages of 30 and 70 years.…”

Section: Clival Chordomamentioning

confidence: 99%

“…Chordomas usually occur in Caucasian 228,229 adults between the ages of 30 and 70 years. Less than 5% appear in the first two decades of life 17 (average age around 10 years) 235,241 . Compared to the adult counterpart, the majority of the paediatric chordomas are primarily found intracranially, mainly in the spheno‐occipital synchondrosis of the clivus, 228,229,233,242 although it has been described in other sites including the sellar and parasellar region, as well as the sphenoid sinus, amongst others 17,228,243,244 …”

Section: Clival Chordomamentioning

confidence: 99%

“…Growth factor‐T (also known as brachyury growth factor), which is involved into notochordal development, is a specific marker of chordomas. Various tyrosine kinase receptors are also expressed in chordoma cells, such as epithelial growth factor α and β 241,246 , platelet‐derived growth factor, c‐KIT 247 and the insulin‐like growth factor‐1 receptor 248 . Tumour suppressor genes 244 may also be implicated in the oncogenesis of chordomas.…”

Section: Clival Chordomamentioning

confidence: 99%

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A clinical approach to parasellar lesions in the transition age

Sbardella

Puliani

Feola

et al. 2021

J Neuroendocrinology

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Many reviews have summarised the pathology and management of the parasellar region in adult patients, although an analysis of these aspects in the transition years, from puberty onset to the age of peak bone mass, has been lacking. A comprehensive search of English‐language original articles, published from 2000 to 2020, was conducted in the MEDLINE database (December 2019 to March 2020). We selected all studies regarding epidemiology, diagnosis and management of the following parasellar lesions: germinoma, craniopharyngioma, Langerhans cell histiocytosis, optic glioma, hypothalamic hamartoma, tuber cinereum hamartoma, cranial chordoma, Rathke cleft cyst, hypophysitis and hypothalamitis during the transition age from childhood to adulthood. In the present review, we provide an overview of the principal parasellar lesions occurring in the transition age. Symptoms are usually a result of the mass effect of the lesions on nearby structures, as well as anterior pituitary deficits. Diabetes insipidus occurs frequently in these patients. In this age group, pubertal developmental disorders may be more evident compared to other stages of life. Parasellar lesions in the transition age mostly include neoplastic lesions such as germinomas, hamartomas, optic gliomas, craniopharyngiomas Langerhans cell histiocytosis and chordomas, and rarely inflammatory lesions (hypophysitis, hypothalamitis). There are limited data on the management of parasellar lesions in the transition age. Endocrine evaluation is crucial for identifying conditions that require hormonal treatment so that they can be treated early to improve the quality of life of the individual patient in this complex age range. The clinical approach to parasellar lesions involves a multidisciplinary effort.

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“…The INI‐1‐negative chordomas differ from classic chordomas also by the absence of further cytogenetic aberrations such as chromosome 9 or chromosome 10 deletions, which are commonly observed in classic chordomas [89]. In line with the evidence from other INI1 deleted tumour entities, INI‐1‐deficient chordomas seem to have a particularly bad prognosis [90]. The immunohistochemical absence of INI‐1 thus prompts the question if these tumours are susceptible to the same therapeutic approaches as ATRT such as, for example, EZH2 inhibition, which have been studied widely in MRT.…”

Section: Smarcb1 Alterations In Paediatric Brain Tumoursmentioning

confidence: 99%

Invited Review: Dysregulation of chromatin remodellers in paediatric brain tumours – SMARCB1 and beyond

Johann

2020

Neuropathology Appl Neurobio

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2020) Neuropathology and Applied Neurobiology 46, 57-72 Dysregulation of chromatin remodellers in paediatric brain tumours -SMARCB1 and beyond Mutations in chromatin remodelling genes occur in approximately 25% of all human tumours (Kadoch et al. Nat Genet 45: 592-601, 2013). The spectrum of alterations is broad and comprises single nucleotide variants, insertion/deletions and more complex structural variations. The single most often affected remodelling complex is the SWI/SNF complex (SWItch/sucrose non-fermentable). In the field of paediatric neuro-oncology, the spectrum of affected genes implicated in epigenetic remodelling is narrower with SMARCB1 and SMARCA4 being the most frequent.The low mutation frequencies in many of the SWI/SNF mutant entities underline the fact that perturbed chromatin remodelling is the most salient factor in tumourigenesis and could thus be a potential therapeutic opportunity. Here, I review the genetic basis of aberrant chromatin remodelling in paediatric brain tumours and discuss their impact on the epigenome in the respective entities, mainly medulloblastomas and rhabdoid tumours. Aberrations described in-Rhabdoid tumours eletions/SNVs (95% of all cases) -rare entities: INI1 deficient chordomas (10-20% of all cases), CRINET (all cases) Figure 1. An overview on SWItch/sucrose non-fermentable aberrant paediatric entities. CRINET, cribriform neuroepithelial tumours. Dysregulation of chromatin remodellers in paediatric neurooncology 61 CNS CRINET Point mutations CNS CRINET, cribriform neuroepithelial tumours.

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Pediatric Chordomas: Results of a Multicentric Study of 40 Children and Proposal for a Histopathological Prognostic Grading System and New Therapeutic Strategies

Cited by 18 publications

References 52 publications

Long Follow-up Surgical Results in 284 Cases of Clival Chordomas: The Risk Factors for Outcome and Tumor Recurrence

Long Follow-up Surgical Results in 284 Cases of Clival Chordomas: The Risk Factors for Outcome and Tumor Recurrence

A clinical approach to parasellar lesions in the transition age

Invited Review: Dysregulation of chromatin remodellers in paediatric brain tumours – SMARCB1 and beyond

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