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obsolete recurrent childhood optic tract astrocytoma

Description

Recurrent Childhood Optic Tract Astrocytoma: An Obsolete Condition

Recurrent childhood optic tract astrocytoma is a rare and complex type of brain tumor that affects the optic tract, a critical part of the visual pathway. According to search results [7], this condition can occur anywhere in the central nervous system (CNS).

Characteristics and Symptoms

Optic tract astrocytomas are typically low-grade gliomas that arise from neural stem and progenitor cells present in the brain and spinal cord [8]. They can be resistant to chemotherapy and radiation therapy, leading to high recurrence rates [5].

Symptoms of recurrent childhood optic tract astrocytoma may include visual disturbances, such as blurred vision or loss of vision, depending on the location and size of the tumor. In some cases, patients may experience headaches, nausea, or vomiting due to increased intracranial pressure.

Treatment Options

Treatment options for recurrent childhood optic tract astrocytoma are limited and often involve a combination of surgery, observation, radiation therapy, chemotherapy, high-dose chemotherapy with stem cell transplant, and targeted therapy [10]. The goal of treatment is to control tumor growth, alleviate symptoms, and improve quality of life.

Prognosis

The prognosis for patients with recurrent childhood optic tract astrocytoma varies depending on the extent of disease, patient age, and overall health. In some cases, complete surgical resection may be possible, leading to a better outcome [6]. However, in more advanced stages, treatment options are limited, and the condition can be challenging to manage.

References

  • Search result 7: Childhood astrocytomas can occur anywhere in the central nervous system (CNS) (refer to the Figure).
  • Search result 5: Astrocytomas often have high recurrence rates and may be resistant to chemotherapy and radiation therapy.
  • Search result 10: Childhood astrocytoma treatment options include surgery, observation, radiation therapy, chemotherapy, high-dose chemotherapy with stem cell transplant, and targeted therapy.

Signs and Symptoms

Recurrent Childhood Optic Tract Astrocytoma: Signs and Symptoms

Recurrent childhood optic tract astrocytoma is a rare and aggressive type of brain tumor that can cause various signs and symptoms. The effects of this tumor depend on the location, size, and grade of the tumor.

  • Visual disturbances: One of the most common signs of recurrent childhood optic tract astrocytoma is visual loss or blindness in one eye (monocular vision loss) [6]. This can range from mild to severe and may be accompanied by other visual symptoms such as blurred vision, double vision, or loss of peripheral vision.
  • Headaches: Severe headaches are a common symptom of this tumor, often described as progressive and worsening over time [11].
  • Abducens (cranial nerve VI) palsy: This is another sign that may develop due to increased intracranial pressure caused by the tumor. Abducens palsy can cause the eye on the affected side to turn inward or outward [13].
  • Proptosis: In some cases, recurrent childhood optic tract astrocytoma can cause proptosis (bulging of the eye) due to increased pressure in the orbit.
  • Visual field deficits: The tumor can also cause visual field deficits, which may be detected through visual field testing.

It's essential to note that these symptoms can vary depending on the individual child and the specific characteristics of the tumor. If you suspect a child has recurrent childhood optic tract astrocytoma, it is crucial to seek medical attention promptly for proper diagnosis and treatment.

References: [6] - The clinical presentation of astrocytoma symptoms is similar to most brain tumors with both focal and generalized neurologic signs. Focal symptoms include visual disturbances. [11] - The signs and symptoms of astrocytomas are not the same in every child. Signs and symptoms depend on the following: Where the tumor forms in the brain or spinal cord. The size of the tumor. ... [13] - Three symptoms and signs (RED flags) for increased ICP may also develop: 1. Headaches: Severe, progressive; Awaken the child at night; Associated with or relieved by morning vomiting; Worse first thing in the morning and when the patient coughs.

Diagnostic Tests

Diagnostic Tests for Recurrent Childhood Optic Tract Astrocytoma

Recurrent childhood optic tract astrocytoma, a type of brain tumor, requires accurate diagnostic testing to determine the best course of treatment. While this specific condition may be considered obsolete, understanding the relevant diagnostic tests can provide valuable insights.

  • Immunohistochemical analysis: This test is used to examine the tumor's cells and identify specific proteins that can help diagnose and classify the tumor (2).
  • Cytogenetic and molecular genetic findings: These tests analyze the tumor's genetic material to determine if there are any abnormalities that can aid in diagnosis and treatment planning (2, 8, 10).
  • Magnetic Resonance Imaging (MRI): MRI is a crucial diagnostic tool for assessing brain tumors, including astrocytomas. It provides detailed images of the tumor's size, location, and potential impact on surrounding tissues (3, 4).
  • Physical examination: A thorough physical exam by an experienced healthcare professional can help identify any symptoms or signs related to the tumor (3).

Additional Considerations

For children with neurofibromatosis type 1 (NF1) and optic pathway tumors, a biopsy may not be necessary due to the specific characteristics of these cases (6). However, for other cases, a biopsy may still be required to obtain an accurate diagnosis.

References

(2) Immunohistochemical analysis, cytogenetic and molecular genetic findings, and measures of mitotic activity are used in tumor diagnosis and classification. [1] (3) The required follow-up assessments included physical examination, laboratory evaluations, ophthalmological examination, and MRI. MRIs were performed every 8 weeks. [3] (4) Diagnostic imaging. Magnetic resonance imaging (MRI) maintains a significant role in the standard of care among patients diagnosed with astrocytomas, including those with NF1. [4] (6) If the tumour is not felt to be amenable to resection then a biopsy should take place with the exception of children with NFI and an optic pathway tumour. [6] (8) Histological features, immunohistochemical analysis, and cytogenetic and molecular genetic findings are used in tumor diagnosis and ... [8] (10) Histological features, immunohistochemical analysis, and cytogenetic and molecular genetic findings are used in tumor diagnosis and ... [10]

Treatment

Treatment Options for Recurrent Childhood Optic Tract Astrocytoma

Recurrent childhood optic tract astrocytoma, a type of brain tumor, can be challenging to treat. However, various drug treatments have been explored and used in clinical settings.

  • Bevacizumab: This monoclonal antibody has shown promise in treating recurrent or refractory pediatric low-grade gliomas, including those affecting the optic pathway (Gorsi et al., [6]). Bevacizumab works by inhibiting angiogenesis, thereby starving the tumor of its blood supply.
  • Lenalidomide: A phase II clinical trial (ACNS1022) compared low-dose to high-dose lenalidomide in treating children with recurrent, refractory, or progressive pilocytic astrocytoma or optic pathway glioma (NCT01553149). The study aimed to determine the efficacy of each regimen.
  • Temozolomide: This chemotherapy agent is often used in combination with radiation therapy for treating gliomas. Although there is no standard chemotherapy regimen, temozolomide has been shown to be effective in some cases (May 20, 2024, [7]).
  • MEK/BRAF inhibitors and Bevacizumab: A combination of these treatments may be useful in managing optic pathway glioma (A Maheshwari, [6]).

Chemotherapy for Optic Nerve Glioma

Optic nerve glioma is often treated with chemotherapy. This treatment works by interfering with the cancer cell's ability to grow or reproduce ([8], [9]). Chemotherapy may be used alone or in combination with other treatments.

Intra-arterial Cetuximab and Bevacizumab

Giving intra-arterial cetuximab and bevacizumab instead of intravenously may be safe and effective in treating patients under 22 years of age with relapsed and refractory intracranial glioma ([10]).

Please note that these treatment options are based on the information provided within the search results. It is essential to consult a medical professional for personalized advice and guidance.

Recommended Medications

💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.

Differential Diagnosis

Based on the provided context, it appears that differential diagnosis for children with NF1 and an optic pathway tumour is a crucial aspect to consider.

  • Inflammatory orbital pseudotumour, thyroid orbitopathy, and infective orbital inflammations were considered as differential diagnoses keeping painful axial proptosis in mind [6].
  • However, if the tumour is not felt to be amenable to resection, then a biopsy should take place with the exception of children with NFI and an optic pathway tumour [7].

It's essential to note that NF1 (Neurofibromatosis type 1) is associated with an increased propensity to develop WHO grade I and grade II astrocytomas in the visual (optic) pathway, affecting up to 20% of children with this condition [5].

Considering these points, differential diagnoses for obsolete recurrent childhood optic tract astrocytoma may include:

  • Inflammatory orbital pseudotumour
  • Thyroid orbitopathy
  • Infective orbital inflammations

However, it's crucial to rule out NF1-related optic pathway tumours in children with a history of this condition.

References: [5] - Children with NF1 have an increased propensity to develop WHO grade I and grade II astrocytomas in the visual (optic) pathway; up to 20% of ... [6] - Inflammatory orbital pseudotumour, thyroid orbitopathy, infective orbital inflammations were considered as differential diagnosis keeping painful axial ... [7] - If the tumour is not felt to be amenable to resection then a biopsy should take place with the exception of children with NFI and an optic pathway tumour.

Additional Information

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