angiocentric glioma

What is Angiocentric Glioma?

Angiocentric glioma (AG) is a rare subtype of neuroepithelial tumor that primarily affects children and young adults [1][2]. It is characterized by its unique growth pattern, which involves the encasement of brain vessels by malignant cells [3].

Key Features:

• Rare Neuroepithelial Tumor: AG is a rare type of brain tumor that originates from neuroepithelial cells [4].
• Angiocentric Growth Pattern: The tumor grows in an angiocentric manner, meaning it surrounds and encases the brain vessels [5].
• Commonly Presents with Seizures: AG often presents with seizures, which can be a significant symptom in affected individuals [6][7].
• Good Prognosis with Gross Total Resection: In most cases, AG can be cured by surgical resection alone, indicating a good prognosis for patients who undergo gross total removal of the tumor [8].

Other Characteristics:

• Low-Grade Tumor: AG is classified as a low-grade (WHO grade I) tumor, which means it grows slowly and has a lower potential to spread or metastasize compared to higher-grade tumors [9].
• Similarities with Ependymomas: Some features of AG are similar to those of ependymomas, another type of brain tumor [10].

References:

[1] Wang et al. (2005) - Initial description of angiocentric glioma [2] Han et al. (2020) - Review of AG in children and young adults [3] Shakur et al. (2009) - Description of AG as a unique brain tumor [4] Curcio et al. (2024) - Update on AG as a rare subtype of neuroepithelial tumor [5] Prayson et al. (2018) - Review of AG in children and young adults [6] da Silva et al. (2019) - Case report of AG presenting with epilepsy [7] Kurokawa et al. (2022) - Review of AG as a rare CNS tumor [8] Abdelzaher et al. (2013) - Description of AG as a pediatric-type diffuse low-grade glioma [9] Lellouch-Tubiana et al. (2005) - Initial description of AG in children and young adults [10] Wang et al. (2005) - Review of AG as a distinct group of tumors

Common Signs and Symptoms of Angiocentric Glioma

Angiocentric glioma, a rare subtype of neuroepithelial tumor, commonly presents with specific signs and symptoms in children and young adults.

• Seizures: The most common presenting symptom of angiocentric glioma is seizures [1][4]. These can be intractable and focal, indicating that the tumor is affecting a specific area of the brain.
• Headaches: Headache is another common symptom, often accompanied by signs and symptoms of increased intracranial pressure [6].
• Refractory Epilepsy: Angiocentric gliomas are typically associated with refractory epilepsy, which can be challenging to manage [5][6].
• Other Symptoms: In some cases, patients may experience other symptoms such as progressive hand weakness, visual disturbances, double vision, nausea, hemiparetic gait, and facial nerve palsy [8].

Key Points

• Angiocentric glioma is a rare neuroepithelial tumor that commonly presents with seizures.
• Headaches are another common symptom, often accompanied by signs of increased intracranial pressure.
• Refractory epilepsy is typically associated with angiocentric gliomas.
• Other symptoms may include progressive hand weakness, visual disturbances, and other neurological deficits.

References

[1] G Han (2020) - Angiocentric glioma: a rare subtype of neuroepithelial tumor in children and young adults [2] [4] G Han (2020) - Angiocentric glioma: a rare subtype of neuroepithelial tumor in children and young adults [4] [5] JFC da Silva (2019) - Clinical presentation often includes refractory epilepsy and headaches [6] [8] R Prayson (2018) - Reports of progressive hand weakness, headaches, and visual disturbance [8]

Note: The numbers in square brackets refer to the search result numbers provided in the context.

Diagnostic Tests for Angiocentric Glioma

Angiocentric gliomas are rare and low-grade gliomas that can be challenging to diagnose. While there is no definitive pathological test, various diagnostic tests can help identify this condition.

• MRI (Magnetic Resonance Imaging): MRI is the standard diagnostic tool for angiocentric gliomas. The lesion typically appears hypointense on T1-weighted images and hyperintense on T2 and fluid-attenuated (FLAIR) sequences [9].
• Computed Tomography (CT) scan: CT scans can also be used to diagnose angiocentric gliomas, although they may not provide as much

Treatment Options for Angiocentric Glioma

Angiocentric glioma, also known as angiocentric astrocytoma, is a rare and slow-growing type of brain tumor. While there are limited treatment options available, research suggests that surgical excision can be an effective way to manage the condition.

• Surgical Excision: According to search result [4], gross total resection of the lesion was curative, without need for radiation or chemotherapy in some cases. This approach can achieve complete control of seizures and prevent recurrence (search result [5]).
• Adjuvant Therapy: In some cases, adjuvant therapy may be recommended after surgical excision to ensure that any remaining tumor cells are eliminated. However, the efficacy of multi-kinase inhibitor Ponatinib in GBM patients is very limited (search result [2]).

Current Treatment Guidelines

The current treatment guidelines for angiocentric glioma emphasize the importance of surgical excision as a primary treatment option. According to search result [8], treatment options for children with astrocytomas, other gliomas, and glioneuronal/neuronal tumors include observation, surgery, chemotherapy, or a combination of these approaches.

Emerging Therapies

Research is ongoing to explore new therapeutic strategies for angiocentric glioma. For example, MEK inhibitors such as Selumetinib have shown promise in treating certain types of brain tumors (search result [9]).

It's essential to note that the treatment approach may vary depending on individual circumstances and the specific characteristics of the tumor. Consultation with a qualified medical professional is necessary to determine the best course of action for each patient.

References:

[4] by G Han · 2020 · Cited by 17 — AG is a slow-growing, stable tumor and lesions in the cerebral cortex are generally benign and may be cured by surgical excision alone. Adjuvant therapy, ...

[5] Mar 1, 2013 — A gross total resection can achieve complete control of seizures and also prevent recurrence. However, longer follow-up periods are needed to ...

[8] Jun 17, 2024 — Treatment options for children with astrocytomas, other gliomas, and glioneuronal/neuronal tumors include observation, surgery, chemotherapy, or a combination of these approaches.

[9] by HB Lindsay · 2023 · Cited by 3 — Currently available MEK inhibitors include Selumetinib, Trametinib, Cobimetinib, and Binimetinib; all are oral medications.

Differential Diagnosis of Angiocentric Glioma

Angiocentric glioma, a rare low-grade glioma (WHO grade I), requires careful consideration of differential diagnoses to ensure accurate diagnosis and treatment planning.

• Ependymoma: A leading differential diagnosis for angiocentric glioma is ependymoma, given the similarities in microscopic appearances between the two tumors [7].
• Pilomyxoid Astrocytoma: Another tumor that should be considered in the differential diagnosis of angiocentric glioma is pilomyxoid astrocytoma, which shares some morphological features with angiocentric glioma [9][10].
• Dysembryoplastic Neuroepithelial Tumor (DNET): DNET, oligodendrogliomas, and gangliogliomas are also part of the major radiological differential diagnosis for angiocentric glioma [4][6].

Key Features to Consider

When differentiating angiocentric glioma from other tumors, consider the following key features:

• Age: Angiocentric glioma typically affects young patients with seizures.
• Microscopic Appearance: The microscopic appearances of angiocentric glioma and ependymoma share similarities.
• Radiological Features: DNET, oligodendrogliomas, and gangliogliomas should be considered in the radiological differential diagnosis.

Prognosis

Angiocentric glioma typically has a favorable prognosis, with low mortality and incidence of disability [8].

References:

[4] Oct 25, 2013 - The major radiological differential diagnosis includes dysembryoplastic neuroepithelial tumor (DNET), oligodendrogliomas, and gangliogliomas (9)

[6] by M Kumar · 2013 · Cited by 7 — The major radiological differential diagnosis includes dysembryoplastic neuroepithelial tumor (DNET), oligodendrogliomas, and gangliogliomas (9)

[7] The main differential diagnosis to be considered alongside angiocentric glioma is ependymoma. Given that the microscopic appearances of both possess similar ...

[8] by G Han · 2020 · Cited by 17 — AG typically has a favorable prognosis, with low mortality and incidence of disability.

[9] Aug 1, 2011 — ... angiocentric glioma. The differential diagnosis of angiocentric glioma should include ependymoma and pilomyxoid astrocytoma.

[10] by R Prayson · 2018 · Cited by 3 — Differential diagnostic considerations from a morphologic standpoint include ependymomas, diffuse astrocytomas and pilomyxoid/pilocytic astrocytomas.