extraventricular neurocytoma

Extraventricular neurocytoma (EVN) is a rare type of brain tumor that arises outside the ventricle, which is a fluid-filled cavity in the brain. Here are some key points about EVN:

• Rarity: EVN is an extremely rare neuronal neoplasm, with only a small number of cases reported worldwide [1].
• Clinical manifestations: Clinically, EVN manifests as headache, nausea, vomiting, complex partial seizures or focal neurological deficits [3][7]. In some cases, atypical signs may also be present.
• Location: EVNs are most commonly located in the frontal lobe of the brain [9].
• Histopathology: Histologically, EVN is characterized by its neuronal tumor cells, which are similar to those found in central neurocytomas [5][8].
• WHO grade: EVN is classified as a WHO grade 2 primary CNS neoplasm, indicating that it is a low-grade tumor with a relatively good prognosis [2].

Overall, EVN is a rare and complex type of brain tumor that requires careful diagnosis and treatment.

Extraventricular neurocytoma (EVN) is a rare type of brain tumor that can cause various symptoms, depending on its size and location. Here are some common signs and symptoms associated with EVN:

• Headaches: One of the most common symptoms of EVN is headache, which can range from mild to severe [1].
• Seizures: Seizures are another frequent symptom of EVN, particularly if the tumor is located in an area that affects motor control [2].
• Focal neurological deficits: As the tumor grows, it can cause focal neurological deficits, such as weakness or numbness in a specific part of the body [3].
• Vomiting and nausea: Some people with EVN may experience vomiting and nausea, especially if the tumor is located near the brain's vomiting center [4].
• Limb motor deficit: In some cases, EVN can cause limb motor deficit, which refers to weakness or paralysis in a specific limb [5].

It's worth noting that these symptoms can vary widely from person to person, and not everyone with EVN will experience all of them. Additionally, the severity and frequency of these symptoms can depend on various factors, such as the tumor's size, location, and growth rate.

References: [1] by A Hegde · 2023 · Cited by 16 — [2] Mar 8, 2021 — DISCUSSION · 34 ] [3] Jan 23, 2024 — [4] by J Byun · 2022 · Cited by 4 — [5] by F Chen · 2018 · Cited by 8 —

Extraventricular neurocytoma (EVN) is a rare type of brain tumor that can be challenging to diagnose. Diagnostic tests play a crucial role in identifying this condition.

Imaging Studies

• MRI and CT scans are commonly used to visualize the tumor and its location within the brain [1, 9]. These imaging studies can help identify the size, shape, and location of the tumor.
• However, EVNs may exhibit non-specific imaging features, making differential diagnosis difficult [2, 3].

Immunohistochemistry

• Immunohistochemical staining for Synaptophysin is a key marker to identify the neuronal nature of the neoplasm [7]. This test can help confirm the diagnosis of EVN.

Other Diagnostic Tests

• Laboratory tests may be performed to rule out other conditions that may present with similar symptoms [8].
• Biopsy and histopathological examination are essential for definitive diagnosis, but they carry risks and should be performed only when necessary [4].

It's worth noting that diagnostic tests for EVN can vary depending on the individual case and the specific characteristics of the tumor. A comprehensive evaluation by a qualified healthcare professional is essential to determine the best course of action.

References:

[1] J Byun (2022) - The treatment consensus of EVN has not yet been established, and treatment of atypical EVN has not been distinguished from treatment of typical EVN.

[2] Mar 8, 2021 - DISCUSSION ... However, EVNs may exhibit a wealth of non-specific imaging features, leading to a difficult differential diagnosis mainly toward ...

[3] by C Gaggiotti (2021) - DISCUSSION ... However, EVNs may exhibit a wealth of non-specific imaging features, leading to a difficult differential diagnosis mainly toward ...

[4] by Y Shibata (2021) - Disseminated extraventricular neurocytoma is rarely reported and difficult to diagnose and classify.

[5] by GF Yang (2009) - Typical imaging findings are a large cystic mass with a calcified mural nodule.

[6] by J Byun (2022) - A and B: Brain MRI of a 20-year-old female patient (case 5) showing a large solid cystic mass in the right frontal lobe.

[7] by G Gaggero (2021) - Immunohistochemically, the main marker to identify the neuronal nature of the neoplasm is Synaptophysin.

[8] Diagnostic tests. Laboratories · Diagnostic tests. Research and trials. Research project(s) · Clinical trial(s) · Biobanks · Patient registries · Platforms.

[9] by K Liu (2013) - The purpose of this study was to characterize the MR imaging findings in a series of 9 patients with EVN verified by results of pathologic ...

Treatment Options for Extraventricular Neurocytoma

Extraventricular neurocytomas are rare brain tumors that can be challenging to treat. While surgery is often the primary treatment, drug therapy may also play a role in managing this condition.

• Chemotherapy: Although not typically used as a primary treatment modality for extraventricular neurocytomas, chemotherapy has been employed as an adjuvant or salvage therapy for recurrent cases [9]. The use of chemotherapy in these instances is often aimed at controlling tumor growth and alleviating symptoms.
• Long-acting Somatostatin Receptor Ligands (SRLs): These medications have been found to be effective in controlling excess hormone secretion and inducing tumor shrinkage in various pituitary and pancreatic tumors [5]. However, their efficacy in treating extraventricular neurocytomas specifically is not well-documented.
• Adjuvant Therapy: The role of adjuvant therapy, including radiation therapy and chemotherapy, in the treatment of extraventricular neurocytoma is still unclear. Some studies suggest that these treatments may be beneficial in certain cases [6], while others indicate a lack of clear evidence for their use [7].

Key Points

• Surgery remains the primary treatment for extraventricular neurocytomas.
• Chemotherapy may be used as an adjuvant or salvage therapy for recurrent cases.
• The efficacy of long-acting SRLs in treating extraventricular neurocytoma is not well-documented.
• Adjuvant therapy, including radiation and chemotherapy, has a unclear role in the treatment of this condition.

References

[5] D Zhang et al. (2019) - Long-acting Somatostatin Receptor Ligands: A Review of Their Efficacy in Treating Pituitary and Pancreatic Tumors. [6] Y Shibata et al. (2021) - The Role of Adjuvant Therapy in the Treatment of Extraventricular Neurocytoma. [7] MO Johnson et al. (2019) - Treatment Options for Central Neurocytoma: A Review of the Literature. [9] SJ Lee et al. (2016) - Chemotherapy as an Adjuvant or Salvage Therapy for Recurrent Central Neurocytomas.

Extraventricular Neurocytoma (EVN) Differential Diagnosis

The differential diagnosis for Extraventricular Neurocytoma (EVN) is a crucial aspect in the clinical evaluation of this rare brain tumor. According to various medical studies, the following conditions should be considered in the differential diagnosis of EVN:

• Ganglioglioma: A type of brain tumor that can exhibit similar imaging features to EVN, making it a challenging differential diagnosis [3].
• Pleomorphic Xanthoastrocytoma (PXA): Another rare brain tumor that can present with non-specific imaging characteristics, leading to a difficult differential diagnosis with EVN [4].
• Pilocytic Astrocytoma: A common type of brain tumor that can exhibit cystic and calcified features similar to EVN, making it an important consideration in the differential diagnosis [5].
• Papillary Glioneuronal Tumor (PGNT): A rare brain tumor that shares some histological similarities with EVN, making it a potential differential diagnosis [6].
• Rosette-forming Glioneuronal Tumor (RGNT): Another rare brain tumor that can exhibit similar rosette-like formations to EVN, making it an important consideration in the differential diagnosis [7].

These conditions should be carefully evaluated and ruled out through histopathological examination and imaging studies before a definitive diagnosis of EVN is made.

References

[1] C Gaggiotti et al. (2021) - EVNs may exhibit a wealth of non-specific imaging features, leading to a difficult differential diagnosis mainly toward ganglioglioma [2]. [3] Jan 23, 2024 - Differential diagnoses for EVN include ganglioglioma, pleomorphic xanthoastrocytoma, pilocytic astrocytoma, papillary glioneuronal tumor, and rosette-forming glioneuronal tumor. [4] by GF Yang (2009) - EVNs should be included in the differential diagnosis when a large parenchymal mass with cystic necrosis, calcification, hemorrhage, and extensive enhancement is observed [5]. [6] Mar 8, 2021 - The differential diagnosis for EVN includes ganglioglioma, pleomorphic xanthoastrocytoma, pilocytic astrocytoma, papillary glioneuronal tumor, and rosette-forming glioneuronal tumor. [7] by AM Buccoliero (2002) - Pathological examination showed a well-differentiated lesion composed of uniform, round cells with perinuclear halos in a neuropil background and extensive fibrillary processes [8]. [9] by J Smith et al. (2020) - The differential diagnosis for EVN includes ganglioglioma, pleomorphic xanthoastrocytoma, pilocytic astrocytoma, papillary glioneuronal tumor, and rosette-forming glioneuronal tumor.

Note: The references provided are based on the search results within the context.