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Last update 08 May 2025

Neurocytoma

Last update 08 May 2025

Basic Info

Synonyms

Neurocytoma, Neurocytomas, neurocytoma

+ [5]

Introduction

A benign brain tumor composed of neural elements which most often arise from the SEPTUM PELLUCIDUM and the walls of the lateral ventricles. Immunohistochemistry and electron microscopy evaluations may reveal expression of neuron specific enolase and synaptophysin and cells containing microtubuli, neurosecretory granules, and presynaptic vesicles. (From Acta Med Port 1994 Feb;7(2):113-9)

Drugs associated with Neurocytoma

Trametinib dimethyl sulfoxide

Target

MEK1 x MEK2

Mechanism

MEK1 inhibitors [+1]

Active Org.

National Cancer Institute [+12]

Originator Org.

GSK Plc

Active Indication

BRAF Fusion Glioma [+125]

Inactive Indication

Adenocarcinoma of Lung [+43]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

United States

First Approval Date29 May 2013

Dabrafenib Mesylate

Target

BRAF x CRAF

Mechanism

BRAF inhibitors [+1]

Active Org.

Novartis Pharmaceuticals Corp. [+8]

Originator Org.

GSK Plc

Active Indication

BRAF Fusion Glioma [+40]

Inactive Indication

Ameloblastoma [+9]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

United States

First Approval Date29 May 2013

Clinical Trials associated with Neurocytoma

NCT03975829

/ Active, not recruitingPhase 4

An Open Label, Multi-center Roll-over Study to Assess Long-term Effect in Pediatric Patients Treated With Tafinlar (Dabrafenib) and/or Mekinist (Trametinib)

A roll-over study to assess long-term effect in pediatric patients treated with dabrafenib and/or trametinib.

Start Date04 Nov 2019

Sponsor / Collaborator

Novartis Pharmaceuticals Corp.

NCT02684058

/ CompletedPhase 2

Phase II Open-label Global Study to Evaluate the Effect of Dabrafenib in Combination With Trametinib in Children and Adolescent Patients With BRAF V600 Mutation Positive Low Grade Glioma (LGG) or Relapsed or Refractory High Grade Glioma (HGG)

The purpose of this study was to investigate the activity of dabrafenib in combination with trametinib in children and adolescent patients with BRAF V600 mutation positive low grade glioma (LGG) or relapsed or refractory high grade glioma (HGG)

Start Date28 Dec 2017

Sponsor / Collaborator

Novartis Pharmaceuticals Corp.

100 Clinical Results associated with Neurocytoma

100 Translational Medicine associated with Neurocytoma

0 Patents (Medical) associated with Neurocytoma

871

Literatures (Medical) associated with Neurocytoma

01 Dec 2025·Child's Nervous System

Congenital extraventricular neurocytoma with atypical features in a 3-day-old neonate

Article

Author: Akiyama, Yukinori ; Mikuni, Nobuhiro ; Kimura, Yusuke ; Matsunaga, Sho ; Yamamoto, Masaki

Extraventricular neurocytoma (EVN) is a rare neuronal tumor with a marked tendency towards ganglionic or glial differentiation. Although EVN commonly occurs in young adults, congenital cases are extremely rare, and standardized therapeutic strategies remain undetermined. The presence of atypical features such as increased mitotic activity on histological analysis is correlated with a higher rate of recurrence and poor prognosis. A 3-d-old infant with no abnormalities at birth presented with recurrent apnea and paroxysmal eye deviation on the upper right side. Magnetic resonance imaging revealed a large intracranial tumor located anterior to the brainstem. The patient underwent biopsy via craniotomy, leading to a histological diagnosis of EVN with atypical features. Despite the absence of adjuvant radiation therapy or chemotherapy, the lesion remained stable over 18 months, and the patient's growth was within normal limits. EVN is an important differential diagnosis of congenital brain tumors. Therapeutic strategies for congenital cases should be distinguished from those used for older children, and treatment decisions should be based on individual patient risks.

01 Jun 2025·Pediatric Blood & Cancer

Extraventricular Neurocytoma in a Five‐Month‐Old Infant

Letter

Author: Wang, Han ; Fang, Yuan ; Chen, Lian

01 May 2025·World Neurosurgery

The Possible Role of Wnt/β-Catenin Pathway in Central Neurocytoma

Article

Author: Doğan, Kutsal ; Veli Alkan, Gökhan ; Önder, Evrim

BACKGROUNDCentral neurocytoma (CN) is a rare intraventricular tumor with unclear molecular features. Ki-67 indices more than 4% indicate aggressive behavior, classifying these as atypical central neurocytoma (ACN). Pathway alteration studies revealed limited results. Despite histological similarities to oligodendrogliomas, CNs lack definitive molecular markers, necessitating alternative diagnostic approaches.METHODSIn this study, we aimed to explore the involvement of the Wnt/β-catenin signalling pathway in CN by evaluating the immunohistochemical expressions of β-catenin and lymphoid enhancer-binding factor 1 (LEF-1). We have documented a total number of 15 CNs. Among 15 cases, 4 were in the atypical category.RESULTSPatient ages were between 11 and 47 years. Ki-67 indices in ACN cases were 7%, 7%, 9%, and 15%. β-catenin demonstrated diffuse membranous staining without any nuclear positivity in 12 cases. Three cases had focal nuclear expression in addition to patchy membranous staining. The nuclear β-catenin positivity was between 3% and 5%. Among these 3 cases, 1 was ACN and showed 5% nuclear positivity. These 3 cases also demonstrated scattered nuclear positivity with LEF-1. Nuclear staining with LEF-1 was weak and highest in the ACN case with 3% nuclear positivity. There was no additional case staining with LEF-1.CONCLUSIONSThe rarity of CN is a limitation to discover its nature. There is no evidence for the possibility of targeted therapies. Still, there are few findings suggesting the involvement of the Wnt/β-catenin pathway. Although our findings may suggest some alterations of this pathway, they are limited to make an interpretation. Multicenter studies have great importance in clarifying genetic modifications in CN and the possibility of targeted therapies.

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