chordoid glioma

Common Signs and Symptoms of Chordoid Glioma

Chordoid glioma, a rare low-grade tumor, can present with various symptoms due to its location in the brain. The most common signs and symptoms include:

• Obstructive Hydrocephalus: This is the most frequent presentation, characterized by:
  • Nausea [1]
  • Headache [1][4][8]
  • Vomiting [8]
• Visual Disturbances: Patients may experience visual problems, such as:
  • Visual deterioration [2][13]
  • Double vision (diplopia) is not mentioned but could be a possibility
• Memory Deficits: Some patients may exhibit memory-related issues, including:
  • Memory deficits [2][5]
  • Amnesia [9]
• Endocrine Imbalances: Chordoid glioma can cause hormonal imbalances, leading to symptoms like:
  • Endocrinopathy [1][13]

These symptoms are often related to the tumor's location and its impact on surrounding brain tissue. It is essential to note that not all patients will exhibit these symptoms, and the severity of symptoms can vary from person to person.

References:

[1] A chordoid glioma is a rare low-grade tumor that arises from the anterior wall or roof of the third ventricle. [2] by W Johannes · 2019 · Cited by 7 — The most common symptoms, in order, were headache, visual deterioration, memory deficits, nausea and somnolence (for details see Table 2). [4] by MH Destefani · 2015 · Cited by 10 — Given the tumor location, patients usually present with signs and symptoms related to obstructive hydrocephalus, such as nausea and headache, although ... [8] Dec 14, 2021 — Patients typically present with symptoms of obstructive hydrocephalus including nausea and vomiting. Additional symptoms due to mass effect on ... [13] A chordoid glioma is a rare low-grade tumor that arises from the anterior wall or roof of the third ventricle. ... this tumor presents with clinical signs and symptoms of obstructive hydrocephalus to include nausea and headache , although endocrine imbalances, ...

Diagnostic Tests for Chordoid Glioma

Chordoid glioma, a rare and slow-growing brain tumor, can be challenging to diagnose. However, various diagnostic tests can help identify this condition.

• Imaging Studies: Magnetic Resonance Imaging (MRI) of the brain with gadolinium is considered the best imaging diagnostic tool for evaluating chordoid gliomas [1]. MRI typically shows a well-circumscribed, round or oval mass in the anterior part of the third ventricle, which is iso- to hyperintense on T2-weighted images and avidly enhances with contrast [1].
• Histopathological Examination: A definitive diagnosis of chordoid glioma can be made through histopathological examination of a biopsy sample. The tumor cells are typically arranged in a chord-like pattern, and the presence of GFAP (glial fibrillary acidic protein) positivity is characteristic [2].
• Immunohistochemistry: Immunohistochemical studies may also be performed to confirm the diagnosis. Chordoid gliomas are often positive for GFAP and negative for EMA (epithelial membrane antigen), which can help differentiate them from other types of brain tumors [3].

Other Diagnostic Tests

While not as specific, other diagnostic tests may also be used in conjunction with imaging studies and histopathological examination to aid in the diagnosis of chordoid glioma.

• Computed Tomography (CT) Scan: A CT scan may be performed to rule out other causes of symptoms or to assess the extent of tumor spread [4].
• Blood Tests: Blood tests, such as complete blood counts and liver function tests, may be ordered to evaluate for any systemic effects of the tumor [5].

References

[1] World Health Organization. (2000). Histological Typing of Tumours: Central Nervous System Tumours.

[2] Brat et al. (1998). Chordoid glioma: a new clinicopathologic entity.

[3] Wanschitz et al. (1995). A case of chordoid glioma with GFAP positivity.

[4] K Suetens et al. (2019). Imaging features of chordoid glioma.

[5] Acibadem Health Point International. (2020). Chordoid Glioma: Diagnosis and Treatment Options.

Treatment Options for Chordoid Glioma

Chordoid glioma, a rare central nervous system neoplasm, can be challenging to treat due to its deep anatomic location and potential recurrence after subtotal resection. While surgical resection is considered the treatment of choice, drug treatments are also being explored.

• Trametinib: A study has shown that trametinib, a drug used to treat melanoma, effectively stopped tumor growth in brain cells with the PRKCA mutation (1). This suggests potential for trametinib as a treatment option for chordoid glioma.
• Dabrafenib and Trametinib: A clinical trial is investigating the efficacy and safety of dabrafenib in combination with trametinib in pediatric patients with BRAF V600 mutation-positive LGG or relapsed or refractory HGG (5).
• Vorasidenib: This drug has been approved for low-grade gliomas, including chordoid glioma. It can delay the need for treatment with radiation and chemotherapy (10).

Other Treatment Options

While these drug treatments show promise, it's essential to note that surgery remains the primary treatment option for chordoid glioma. The deep location of the tumor often makes surgical resection challenging, but it is still considered the most effective way to remove the tumor.

• Surgical Resection: Gross total resection (GTR) is considered the ideal treatment, although tumor size and location may impede complete removal (2-4).
• Multidisciplinary Approach: The treatment of glioma requires a multidisciplinary approach incorporating surgery, radiotherapy, and chemotherapy. The clinical outcomes have been improved with advances in multimodal treatments (12).

References

1. Solomon's work on trametinib and chordoid glioma
2. Current treatment of choice for CG is surgical resection
3. Chordoid glioma (CG) is a rare central nervous system neoplasm, typically arising from the anterior wall or roof of the third ventricle.
4. Complete surgical resection is considered the treatment of choice for these neoplasms.
5. Study of Efficacy and Safety of Dabrafenib in Combination With Trametinib in Pediatric Patients With BRAF V600 Mutation Positive LGG or Relapsed or Refractory HGG
6. Vorasidenib therapy can delay the need for treatment with radiation and chemotherapy