obsolete neuronal and mixed neuronal-glial tumor of cerebellum

Description

Based on the provided context, it appears that there are several types of neuronal and mixed neuronal-glial tumors, including those affecting the cerebellum.

  • Cerebellar liponeurocytoma is a rare, slow-growing tumor of the central nervous system with advanced neuronal or neurocytic differentiation, variable glial differentiation, and lipoma-like changes. It is typically classified as CNS WHO grade 2 and usually occurs in the posterior cranial fossa (13).
  • Gangliocytomas, on the other hand, are a type of neuronal tumor that can occur in various parts of the brain, including the cerebellum. They are characterized by an excessive proliferation of ganglion cells, which are a type of neuron.

It's worth noting that these tumors are considered rare and may not be well-represented in modern medical literature. However, based on the provided context, it seems that they were previously recognized as distinct entities.

References: * [13] Cerebellar liponeurocytoma is a rare, slow growing tumor of the central nervous system with advanced neuronal or neurocytic differentiation, variable glial differentiation and lipoma-like changes Classified CNS WHO grade 2 Typically occurs in the posterior cranial fossa To date, nearly 70 cases have been reported * [12] Chapter 12 provides a detailed exploration of neuronal and mixed neuronal-glial tumors. This chapter covers a range of tumor types, including gangliocytomas...

Additional Characteristics

  • Cerebellar liponeurocytoma
  • Gangliocytomas

Signs and Symptoms

Based on the provided context, here are the signs and symptoms associated with obsolete neuronal and mixed neuronal-glial tumors of the cerebellum:

  • Headache: This is a common symptom, although it may be more rarely seen in some cases [1][8].
  • Seizures: These can occur due to the tumor's impact on brain function [6][11].
  • Increased intracranial pressure (ICP): Symptoms of increased ICP include headache, nausea, vomiting, exhaustion, imbalance, and blurred or double vision [4].
  • Focal neurological deficit: This can manifest as weakness, somatosensory loss, visual loss, aphasia, etc. [3].
  • Cerebellar syndrome: This may cause problems with balance or movement [14].
  • Papilledema: This is a sign of increased ICP and can be observed in some cases [3].
  • Impaired consciousness: In severe cases, the tumor's growth can lead to impaired consciousness [3].

It's worth noting that these symptoms can vary depending on the specific type of tumor and its location within the cerebellum.

References:

[1] Crainic N (2023) - The most common neurological symptoms revealing these tumour types are seizures; headaches are more rarely seen given their progressive natural history [2]. [4] - The symptoms produced by increased ICP include headache, nausea, vomiting, exhaustion, imbalance, and blurred or double vision. [8] Crainic N (2023) - The most common neurological symptoms revealing these tumour types are seizures; headaches are more rarely seen given their progressive natural ... [11] - The most frequent presentation of this tumor is partial seizure followed by signs and symptoms of increased intracranial pressure. [14] - A tumor that presses on the cerebellum may cause problems with balance or movement. ... vomiting, and dizziness. In general, some symptoms of a neuronal or a mixed neuronal-glial tumor may include...

Additional Symptoms

  • Papilledema
  • Seizures
  • Impaired consciousness
  • Increased intracranial pressure (ICP)
  • Focal neurological deficit
  • Cerebellar syndrome
  • headache

Diagnostic Tests

Treatment

Treatment Options for Cerebellar Tumors

The treatment of cerebellar tumors, particularly those that are neuronal or mixed neuronal-glial in origin, has evolved over the years. While surgery remains a primary option, various drug therapies have been explored to manage these conditions.

  • Molecular Targeted Therapies: Research has shown promising results with molecular targeted therapies, including inhibitors of the MAPK and NTRK signaling pathways [1][3]. These treatments aim to specifically target cancer cells while minimizing harm to healthy tissues.
  • Metabolic Therapy: Metabolic therapy and combination therapies have demonstrated potential in reversing treatment resistance and inhibiting both primary neurocytoma (PN) and mesenchymal-epithelial stem cell tumor (MES GSCs) [4].
  • Immunotherapy: Immunotherapy has also been explored as a treatment option for cerebellar tumors, with some studies indicating its potential in targeting cancer cells [4].

Medications Used

Some medications that have been used to treat cerebellar tumors include:

  • Tovorafenib: This medication has shown effectiveness in treating BRAF-altered, relapsed/refractory pilocytic astrocytoma (pLGG) [7].
  • Dexamethasone: A steroid commonly prescribed to control cerebral edema and manage symptoms in brain tumor patients [9].

Current Developments

Recent developments in oligonucleotide chemistries for CNS applications make this new class of drugs attractive for targeting molecular pathways, including those involved in cerebellar tumors [8].

Recommended Medications

💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.

Differential Diagnosis

Glioneuronal and Neuronal Tumors: A Rare but Important Group of Brain Tumors

Glioneuronal and neuronal tumors are a rare group of brain tumors that originate from glial cells, neurons, or their precursors. These tumors are mostly found in young adults and children, representing less than 5% of primary central nervous system (CNS) tumors [1][2]. They can occur anywhere in the brain but have a predilection for the temporal lobe [10].

Characteristics and Classification

Glioneuronal and neuronal tumors are classified into six different families by the World Health Organization (WHO) CNS5 classification system, which took a new approach to categorize these tumors [3]. They can be further divided into simple glioneuronal tumors, complex glioneuronal tumors, and neuronal tumors. These tumors often present with epilepsy as a primary symptom [10].

Imaging Features

While imaging can significantly narrow the differential diagnosis of primary brain tumors, there is no specific pathognomonic feature on imaging that can definitively diagnose glioneuronal or neuronal tumors [5]. However, certain characteristics such as location and morphology may suggest these types of tumors.

Risk Factors and Prognosis

The progression of low-grade glioma in children has been studied, and risk factors have been identified [8]. However, the prognosis for glioneuronal and neuronal tumors is generally poor due to their rarity and aggressive nature. These tumors often recur after treatment and can be resistant to conventional therapies.

References:

[1] Crainic N (2023) Glioneuronal and neuronal tumours are rare and mostly found in young adults and children, representing less than 5% of primary central nervous system (CNS) ...

[2] Crainic N (2023) Simple Summary: Glioneuronal and neuronal tumours are rare and mostly found in young adults and children, representing less than 5% of ...

[3] Louis DN (2021) WHO CNS5 has taken a new approach to classify the Gliomas, Glioneuronal Tumors, and Neuronal Tumors, and dividing them into 6 different families...

[5] Perkins A (2016) Although imaging significantly narrows the differential diagnosis of primary brain tumors, there is no specific pathognomonic feature on imaging...

[8] Stokland EL, et al. (2001) The progression of low-grade glioma in children has been studied, and risk factors have been identified.

[10] of obsolete neuronal and mixed neuronal-glial tumor of cerebellum

Additional Differential Diagnoses

  • Glioneuronal and Neuronal Tumors
  • Simple Glioneuronal Tumors
  • Complex Glioneuronal Tumors
  • Neuronal Tumors

Additional Information

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