Left Mesial Temporooccipital PLNTY: Supracerebellar Transtentorial Approach in Epilepsy Surgery

Rizzi, Michele; Castelli, Nicolò; Giacomo, Roberta Di; Cojazzi, Vittoria; Innocenti, Niccolò; Levi, Vincenzo; Nazzi, Vittoria; Schiariti, Marco

doi:10.1016/j.wneu.2022.12.100

Cited by 2 publications

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“…8 were excluded due to being off topic. Rizzi et al was excluded due to lack of BRAF V600E status[10]. Zhao et al was excluded due to the lack of available full text English manuscript[11].…”

mentioning

confidence: 99%

Systematic review and cumulative analysis of clinical properties of BRAF V600E mutations in PLNTY histological samples

Baumgartner,

Lang,

Tucker

et al. 2023

Childs Nerv Syst

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Polymorphous low-grade neuroepithelial tumors of the young (PLNTY) represent a rare pediatric-type tumor that most commonly presents as medically refractory epilepsy. PLNTY has only recently been recognized as a distinct clinical entity, having been rst described in 2016 and added to the World Health Organization classi cation of CNS tumors in 2021. Molecular studies have determined that PLNTY is uniformly driven by aberrant MAPK pathway activation, with most tumors carrying either a BRAF V600E mutation or activating FGFR2 or FGFR3 fusion protein. Although it is known that these driver mutations are mutually exclusive, little is known about differences in clinical presentation or treatment outcomes between PLNTY cases driven by these distinct mutations. In this study, we performed a systematic review and cumulative analysis of PLNTY cases to assess whether or not PLNTY tumors carrying the BRAF V600E mutation exhibit different clinical behaviors. By searching the literature for all cases of PLNTY wherein BRAF V600E status was characterized, we compiled a dataset of 62 unique patient instances.Using a logistic regression-based approach, we assessed a primary outcome of what factors of a clinical presentation were associated with BRAF V600E mutations and a secondary outcome of what factors predicted total seizure freedom post-surgical resection. PLNTY cases carrying BRAF V600E mutations in the literature were strongly positively associated with adult patients (p=0.0055, OR=6.556; 95% Conf. Int. = 1.737 -24.742). BRAF V600E status was also positively associated with tumor involvement of the temporal lobe (p=0.0046, OR=11.036; 95% Conf. Int. = 2.100 -58.006). Male sex was also positively associated with BRAF V600E status, but the result did not quite achieve statistical signi cance (p=0.0731). BRAF V600E status was not found to be associated with post-operative seizure freedom.These ndings indicate that BRAF V600E-positive PLNTY exhibit characteristic clinical presentations but are not necessarily different in treatment responsiveness.

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mentioning

confidence: 99%

Systematic review and cumulative analysis of clinical properties of BRAF V600E mutations in PLNTY histological samples

Baumgartner,

Lang,

Tucker

et al. 2023

Childs Nerv Syst

View full text Add to dashboard Cite

show abstract

Systematic review and cumulative analysis of clinical properties of BRAF V600E mutations in PLNTY histological samples

Baumgartner

Lang

Tucker

et al. 2023

Preprint

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Polymorphous low-grade neuroepithelial tumors of the young (PLNTY) represent a rare pediatric-type tumor that most commonly presents as medically refractory epilepsy. PLNTY has only recently been recognized as a distinct clinical entity, having been first described in 2016 and added to the World Health Organization classification of CNS tumors in 2021. Molecular studies have determined that PLNTY is uniformly driven by aberrant MAPK pathway activation, with most tumors carrying either a BRAF V600E mutation or activating FGFR2 or FGFR3 fusion protein. Although it is known that these driver mutations are mutually exclusive, little is known about differences in clinical presentation or treatment outcomes between PLNTY cases driven by these distinct mutations. In this study, we performed a systematic review and cumulative analysis of PLNTY cases to assess whether or not PLNTY tumors carrying the BRAF V600E mutation exhibit different clinical behaviors. By searching the literature for all cases of PLNTY wherein BRAF V600E status was characterized, we compiled a dataset of 62 unique patient instances. Using a logistic regression-based approach, we assessed a primary outcome of what factors of a clinical presentation were associated with BRAF V600E mutations and a secondary outcome of what factors predicted total seizure freedom post-surgical resection. PLNTY cases carrying BRAF V600E mutations in the literature were strongly positively associated with adult patients (p=0.0055, OR=6.556; 95% Conf. Int. = 1.737 – 24.742). BRAF V600E status was also positively associated with tumor involvement of the temporal lobe (p=0.0046, OR=11.036; 95% Conf. Int. = 2.100 – 58.006). Male sex was also positively associated with BRAF V600E status, but the result did not quite achieve statistical significance (p=0.0731). BRAF V600E status was not found to be associated with post-operative seizure freedom. These findings indicate that BRAF V600E-positive PLNTY exhibit characteristic clinical presentations but are not necessarily different in treatment responsiveness.

show abstract

Left Mesial Temporooccipital PLNTY: Supracerebellar Transtentorial Approach in Epilepsy Surgery

Cited by 2 publications

References 0 publications

Systematic review and cumulative analysis of clinical properties of BRAF V600E mutations in PLNTY histological samples

Systematic review and cumulative analysis of clinical properties of BRAF V600E mutations in PLNTY histological samples

Systematic review and cumulative analysis of clinical properties of BRAF V600E mutations in PLNTY histological samples

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