myxoid glioneuronal tumor

Myxoid glioneuronal tumors (MGNTs) are a type of low-grade glioneuronal neoplasm that has been recently introduced in the World Health Organization (WHO) classification of central nervous system (CNS) tumors.

Characteristics:

• MGNTs are composed of oligodendrocyte-like cells in a mucin-rich stroma [8].
• They have a stereotypic location in the septal region, periventricular white matter, and other specific areas of the brain [7].
• The tumors are typically non-cancerous (benign) and have a distinct isolated genetic mutation [6].

Differential Diagnosis:

• MGNTs should be differentiated from other low-grade gliomas and glioneuronal tumors such as pilocytic astrocytoma, ganglioglioma, and rosette-forming glioneuronal tumor [1].
• They can also be confused with dysembryoplastic neuroepithelial tumors (DNTs), but MGNTs have a distinct PDGFRA p.K385 mutation and DNT-like methylation profile [5].

Recent Classification:

• MGNT was classified as a new tumor type in the WHO classification of CNS tumors, reflecting its unique characteristics and genetic features [9].
• The recent addition of MGNT to the WHO classification highlights the importance of accurate diagnosis and differential diagnosis in clinical practice.

References:

[1] C. Caporalini (2023) - Differential diagnosis, beyond DNT, should include low-grade gliomas and low grade glioneuronal tumors such as pilocytic astrocytoma, ganglioglioma, and rosette ...

[5] DA Solomon (2018) - Myxoid glioneuronal tumor of the septum pellucidum and lateral ventricle is defined by a recurrent PDGFRA p.K385 mutation and DNT-like methylation profile.

[6] EO Narvaez (2021) - Myxoid glioneuronal tumour is a new entity with a distinct isolated genetic mutation recently described.

[7] M Silverman (2021) - Myxoid glioneuronal tumors are a newly defined CNS tumor that has stereotypic location in the septal region, periventricular white matter, ...

[8] L de Villenfagne (2024) - Myxoid glioneuronal tumors (MGNT) are low-grade glioneuronal neoplasms composed of oligodendrocyte-like cells in a mucin-rich stroma.

[9] J Chu (2024) - Myxoid glioneuronal tumour (MGNT), previously described as dysembryoplastic neuroepithelial tumour of the septum pellucidum, was classified as a new tumour type ...

Common symptoms associated with myxoid glioneuronal tumors (MGTs) include:

• Headache [6][7]
• Seizures, which are the most common symptom experienced by patients with glioneuronal and neuronal tumors [2]
• Symptoms related to blockage of cerebrospinal fluid, such as hydrocephalus [2]
• Cognitive deficits, behavioral disorders, visual disturbances, altered mental status, emesis (vomiting), facial drop, and other symptoms [4]

Imaging characteristics:

• MGTs show well-circumscribed margins, cystic features, and a discrete size (mostly 1 to 3 cm) on neuroimaging [1]
• They may demonstrate a solid-cystic appearance, with a bright rim sign on FLAIR imaging, similar to that in DNETs [5][9]

Diagnosis:

• Accurate diagnosis of MGTs can be challenging and often requires a significant body of evidence [14]

Based on the search results, it appears that diagnostic tests for myxoid glioneuronal tumors (MGTs) are crucial in determining the correct diagnosis and treatment plan.

• Imaging studies: According to [4], all brain imaging studies for each patient were reviewed by an expert neuroradiologist. Tumors were examined for T1 and T2 signal intensity, T2 FLAIR, and other characteristics.
• Spectroscopy: As mentioned in [8], spectroscopy demonstrates minor elevation of choline and no elevation of CBV. This information can be useful in differentiating MGTs from other types of tumors.
• Molecular markers: According to [9], there are various molecular markers found among different types of glioneuronal and neuronal tumors that can help determine diagnosis. These markers may be used to identify specific genetic mutations associated with MGTs.

It's worth noting that the differential diagnosis for MGTs includes other types of brain tumors, such as pilocytic astrocytoma, ganglioglioma, and rosette-forming glioneuronal tumor [2]. Therefore, a comprehensive diagnostic workup is essential to ensure accurate diagnosis and treatment.

In terms of specific diagnostic tests, it's not clear from the search results what the most common or recommended tests are for MGTs. However, based on the information provided, it appears that imaging studies, spectroscopy, and molecular marker analysis may be useful in diagnosing and characterizing these tumors.

References: [2] - Differential diagnosis of MGTs includes pilocytic astrocytoma, ganglioglioma, and rosette-forming glioneuronal tumor. [4] - Imaging studies are reviewed by an expert neuroradiologist to examine T1 and T2 signal intensity, T2 FLAIR, and other characteristics. [8] - Spectroscopy demonstrates minor elevation of choline and no elevation of CBV. [9] - Molecular markers are found among different types of glioneuronal and neuronal tumors that can help determine diagnosis.

Based on the available information, it appears that surgery is the primary treatment for myxoid glioneuronal tumors. However, there is limited information available on the use of drug treatments for this specific type of tumor.

According to search result [3], Imatinib is an FDA-approved molecule used for the treatment of various cancers, but its use in myxoid glioneuronal tumors is not specifically mentioned.

Search result [4] mentions that the mainstay of treatment for these kinds of tumors is gross total resection, and subtotal resection may be preferred to preserve key neural structures. However, it does not mention any specific drug treatments.

Search result [9] states that targeted therapy is one of the most common treatments for myxoid glioneuronal tumors, but it does not specify which drugs are used.

It's worth noting that the available information on drug treatment for myxoid glioneuronal tumors is limited and inconclusive. Further research would be needed to determine the effectiveness of specific drug treatments for this type of tumor.

Possible Treatment Options:

• Surgery (gross total resection or subtotal resection)
• Targeted therapy (specific drugs not specified)

Limitations: The available information on drug treatment for myxoid glioneuronal tumors is limited and inconclusive. Further research would be needed to determine the effectiveness of specific drug treatments for this type of tumor.

References: [3] Imatinib is an FDA-approved molecule used for the treatment of various cancers, but its use in myxoid glioneuronal tumors is not specifically mentioned. [4] The mainstay of treatment for these kinds of tumors is gross total resection, and subtotal resection may be preferred to preserve key neural structures. However, it does not mention any specific drug treatments. [9] Targeted therapy is one of the most common treatments for myxoid glioneuronal tumors, but it does not specify which drugs are used.

Differential Diagnosis of Myxoid Glioneuronal Tumor (MGT)

Myxoid Glioneuronal Tumor (MGT) is a rare benign brain tumor, and its differential diagnosis includes several other types of tumors. According to the search results, the following are some of the key differentials:

• Low-grade gliomas: These include pilocytic astrocytoma, ganglioglioma, and others [1][3].
• Central neurocytoma, subependymoma, dysembryoplastic neuroepithelial tumor (DNET), rosette-forming glioneuronal tumor, and colloid cyst are also part of the differential diagnosis [4][5].
• Subependymoma, ependymoma, subependimal giant cell astrocytoma, meningioma, and intraventricular oligodendroglioma should be considered in the differential diagnosis [7].

It's essential to note that the differential diagnosis of MGT is not exhaustive, and other tumors may also need to be considered on a case-by-case basis.

References:

[1] C. Caporalini (2023) - Differential diagnosis beyond DNT includes low-grade gliomas and low-grade glioneuronal tumors [1] [3] C. Caporalini (2023) - Differential diagnosis, beyond DNT, should include low-grade gliomas and low grade glioneuronal tumors such as pilocytic astrocytoma, ganglioglioma, and others [3] [4] A. Vaz (2022) - The differential diagnosis is central neurocytoma, subependymoma, dysembryoplastic neuroepithelial tumor, rosette-forming glioneuronal tumor, and colloid cyst [4] [5] C. Zamora (2021) - The differential diagnosis includes subependymoma, central neurocytoma, and colloid cyst [5] [7] A. Vaz (2022) - The differential diagnosis is subependymoma, ependymoma, subependimal giant cell astrocytoma, meningioma, and intraventricular oligodendroglioma [7]

Note: The numbers in square brackets refer to the corresponding search result number.