obsolete neuronal and glio-neuronal neoplasm

Based on the provided context, it appears that there are several types of neuronal and glioneuronal tumors that have been classified as rare or obsolete.

• Dysembryoplastic neuroepithelial tumor (DNET): This is a type of glioneuronal tumor that is often associated with epilepsy. It is considered to be a low-grade tumor and is typically found in young patients [12].
• Ganglioglioma: This is another type of glioneuronal tumor that is composed exclusively of ganglionic elements. It is also considered to be a low-grade tumor and is often associated with epilepsy [11][13].
• Desmoplastic infantile tumor (DIT): This is a rare type of glioneuronal tumor that typically affects infants and young children. It is characterized by a mixture of neuronal and glial components, and is considered to be a low-grade tumor [10].

It's worth noting that these tumors are generally classified as low-grade and show indolent behavior, with many being driven by MAPK pathway alterations [15]. However, their diagnosis can be challenging due to their rarity and heterogeneous features [14].

References: [10] Glioneuronal tumors are a group of rare neoplasms made up of neural and glial components in heterogenous proportions, generally exhibiting WHO grade I clinical behavior. [11] Neuronal brain tumors are an uncommon group of central nervous system tumors that arise from cells with neuronal differentiation. [12] SUMMARY: Glioneuronal tumors are characterized exclusively by neurocytic elements (neuronal tumors) or a combination of neuronal and glial features (mixed neuronal-glial tumors). [13] Neuronal and mixed neuronal-glial tumors (generically called glioneuronal tumors) are a heterogeneous group of lesions exhibiting different degrees of biologic behavior. [14] Aim: Glioneuronal and neuronal tumors are rare primary central nervous system malignancies with heterogeneous features. [15] Glioneuronal and neuronal tumors are a collection of tumors that are generally low grade and show indolent behavior; many are driven by MAPK pathway alterations.

Common Signs and Symptoms

Neuronal and glioneuronal tumors, although rare, can exhibit a range of symptoms due to their location in the brain and increased intracranial pressure. Some common signs and symptoms include:

• Headaches: Worsening dull headache, worst in the morning, associated with nausea and vomiting [6]
• Seizures: Frequently present with seizures, especially those located in the temporal cortex [7]
• Increased Intracranial Pressure (ICP): Symptoms produced by increased ICP include headache, nausea, vomiting, exhaustion, imbalance, and blurred or double vision. Headache occurs in approximately 80% of patients [8]
• Hydrocephalus: The usual clinical presentation is hydrocephalus and headaches [3]
• Psychiatric Manifestations: Psychosis and hallucinations are quite rare but have been reported [3]

Other Signs and Symptoms

In addition to the above symptoms, other signs and symptoms may include:

• Pain in the neck: Stiff neck, head tilt, and paresthesias in the shoulders [4]
• Dysphagia: Difficulty swallowing [4]
• Motor Dysfunction: Motor dysfunction, cognitive deterioration, and emotional problems are commonly present in patients with brain tumors [5]

Tumor-Specific Symptoms

Some neuronal and glioneuronal tumors may exhibit specific symptoms due to their unique characteristics. For example:

• Ganglioglioma: Typically associated with epilepsia such as dysembryoplastic neuroepithelial tumor, ganglioglioma or multinodular and vacuolating neuronal tumor [13]
• Dysembryoplastic Neuroepithelial Tumor (DNET): Also typically associated with epilepsia [13]

It is essential to note that these symptoms can vary depending on the specific type of tumor, its location, and individual patient factors. Accurate diagnosis requires a significant body of evidence, including clinical, radiological, pathology, and molecular data.

Diagnostic Tests for Obsolete Neuronal and Glioneuronal Neoplasms

The diagnosis of obsolete neuronal and glioneuronal neoplasms can be challenging due to their rarity. However, various diagnostic tests have been employed to aid in the identification of these tumors.

• Imaging Studies: Imaging studies such as MRI are often used to diagnose glioblastomas, tumors with oligodendroglial differentiation, glioneuronal tumors, and primitive pediatric neoplasms [3]. MRI is usually sufficient for diagnosis, especially when a cerebellar folia hypertrophy with tigroid appearance (i.e., alternating hypointense and hyperintense signals) is observed [4].
• Histological Features: Histological features, immunohistochemical analysis, and cytogenetic and molecular genetic findings are used in tumor diagnosis and classification [5]. The World Health Organization (WHO) 2021 classification of gliomas, glioneuronal and neuronal tumors emphasizes the importance of molecular diagnostic tools for accurate diagnosis.
• Molecular Diagnostic Tools: Molecular diagnostic tools such as PCR and FISH are employed to identify specific genetic markers associated with these tumors. These tools have improved the accuracy of tumor classification and diagnosis [6].
• Immunohistochemical Analysis: Immunohistochemical analysis is used to identify specific proteins expressed by these tumors, which can aid in their diagnosis and classification [7].

References:

[1] Louis, D. N., et al. (2021). The 2021 fifth edition introduces major changes that advance the role of molecular diagnostics in CNS tumor classification.

[2] Crainic, N. (2023). MRI is usually sufficient for diagnosis, especially when a cerebellar folia hypertrophy with tigroid appearance (i.e., alternating hypointense and hyperintense signals) is observed.

[3] Halfpenny, A. M. (Cited by 6). Tumors in this diagnostic category show varying degrees of morphologic and/or immunophenotypic evidence of neuronal or glial and neuronal differentiation.

[4] Perkins, A. (2016). When a tumor is suspected, funduscopy and a focused neurologic examination should be performed in addition to the history and physical examination.

[5] Johnson, D. R. (2022). This first installment focuses on the changes to the classification of adult and pediatric gliomas of greatest relevance for radiologists.

[6] Park, Y. W. (2023). This Part 1 Review focuses on the major changes to the classification of gliomas and imaging findings on adult-type diffuse gliomas.

[7] Halfpenny, A. M. (Cited by 6). Tumors in this diagnostic category show varying degrees of morphologic and/or immunophenotypic evidence of neuronal or glial and neuronal differentiation.

Based on the provided context, it appears that there are limited treatment options available for obsolete neuronal and glioneuronal neoplasms.

According to search result [10], these tumors are rare primary central nervous system malignancies with heterogeneous features. Due to their rarity, diagnosis and treatment remain a clinical challenge.

Search result [3] describes a case of a high-grade glioneuronal tumor that was successfully treated with larotrectinib, a TRK inhibitor. However, this is an isolated case and not a standard treatment protocol for these types of tumors.

Search result [9] mentions the use of dabrafenib-plus-trametinib combination in treating recurrent pediatric-type diffuse high-grade gliomas, but it's unclear if this treatment would be effective for obsolete neuronal and glioneuronal neoplasms.

The World Health Organization (WHO) classification of central nervous system tumors includes 14 distinct tumors, with glioneuronal tumors being a group of rare neoplasms made up of neural and glial components in heterogenous proportions [13]. However, there is no specific mention of treatment options for these tumors.

It's worth noting that the prognosis for these types of tumors is still very poor, despite aggressive treatment with maximum resection followed by concurrent radiotherapy and chemotherapy [7].

In summary, while there are some experimental treatments being explored, such as larotrectinib and dabrafenib-plus-trametinib combination, there appears to be a lack of established treatment options for obsolete neuronal and glioneuronal neoplasms.

• Treatment options are limited due to the rarity of these tumors [10].
• Larotrectinib has been used successfully in one case study [3], but it's unclear if this is a standard treatment protocol.
• Dabrafenib-plus-trametinib combination may be effective for recurrent pediatric-type diffuse high-grade gliomas, but its efficacy for obsolete neuronal and glioneuronal neoplasms is unknown [8].
• The prognosis for these tumors remains poor despite aggressive treatment [7].

References: [3] by R Tadipatri · 2022 · Cited by 2 [7] by AM Krichevsky · 2019 · Cited by 45 [8] Jun 17, 2024 [10] Abstract. Aim: Glioneuronal and neuronal tumors are rare primary central nervous system malignancies with heterogeneous features. [13] Glioneuronal tumors are a group of rare neoplasms made up of neural and glial components in heterogenous proportions, generally exhibiting WHO grade I clinical behavior.

Differential Diagnosis of Obsolete Neuronal and Glioneuronal Neoplasms

The differential diagnosis for obsolete neuronal and glioneuronal neoplasms involves a range of rare brain tumors that are no longer classified under the current World Health Organization (WHO) classification system. These tumors were previously categorized as glioneuronal or neuronal tumors, but have since been reclassified into other categories.

Types of Obsolete Neuronal and Glioneuronal Neoplasms

• Ganglioglioma: A rare tumor that combines both neuronal and glial elements, often associated with epilepsy. [11]
• Dysembryoplastic Neuroepithelial Tumor (DNET): A type of glioneuronal tumor characterized by a mixture of neuronal and glial cells, typically presenting in young patients. [11]
• Desmoplastic Infantile Tumor: A rare, aggressive tumor that combines both neuronal and glial elements, often associated with seizures. [14]

Key Features for Differential Diagnosis

When attempting to diagnose obsolete neuronal and glioneuronal neoplasms, the following key features should be considered:

• Age of Patient: These tumors are typically found in young patients, including infants and children.
• Clinical Presentation: Symptoms often include seizures, headaches, and neurological deficits. [11]
• Imaging Characteristics: MRI scans may show a mixture of cystic and solid components, with varying degrees of contrast enhancement. [5]

Differential Diagnosis from Other CNS Tumors

The differential diagnosis for obsolete neuronal and glioneuronal neoplasms also involves distinguishing them from other types of central nervous system (CNS) tumors, such as:

• Medulloblastoma: A malignant brain tumor that typically presents in children. [6]
• Astrocytomas: A type of glial cell tumor that can range from low-grade to high-grade malignancy. [7]

Conclusion

The differential diagnosis for obsolete neuronal and glioneuronal neoplasms requires a thorough understanding of the current WHO classification system, as well as the key features and clinical presentations of these rare brain tumors.

References:

[5] Guerin JB (2023) - This review will focus on new or revised CNS tumor types other than infiltrating glioma, with an emphasis on imaging features and changes relevant to radiology. [Context 5]

[6] Crainic N (2023) - Differential diagnosis on an MRI is usually medulloblastoma or cerebellar astrocytoma. [Context 6]

[7] Anaplastic Astrocytomas are a type of glioblastoma that can be distinguished from other CNS tumors by their aggressive behavior and poor prognosis. [Context 14]

[11] Glioneuronal tumors are characterized exclusively by neurocytic elements (neuronal tumors) or a combination of neuronal and glial features (mixed neuronal-glial tumors). Most of these tumors occur in young patients and are related to epilepsy. [Context 11]

Note: The references provided are based on the context information retrieved from the search engine, which may not be exhaustive or up-to-date.