obsolete recurrent glioma of childhood visual pathway

Recurrent Glioma of Childhood Visual Pathway: An Obsolete Concept?

The concept of "recurrent glioma" in the context of childhood visual pathway tumors has evolved over time. In the past, these tumors were often referred to as "recurrent optic pathway gliomas." However, with advancements in medical research and treatment options, this terminology is no longer commonly used.

Current Understanding

Today, pediatric low-grade gliomas (pLGGs) are recognized as a distinct entity, accounting for 40-50% of all pediatric central nervous system (CNS) tumors [1]. These tumors can arise in various locations within the CNS, including the optic pathway and hypothalamic region. The term "optic pathway glioma" is now reserved for those tumors confined to the precortical visual pathway, sometimes with involvement of the hypothalamus [5].

Treatment Implications

The management of pLGGs has also undergone significant changes. While surgery was once considered a primary treatment option, it is now often used in conjunction with other modalities, such as chemotherapy and radiation therapy, to achieve optimal outcomes [6]. The focus has shifted towards personalized treatment approaches, taking into account the unique characteristics of each tumor and the individual child's needs.

Visual Outcomes

Research has shown that visual outcomes for children with optic pathway gliomas can be improved with conformal radiation therapy [13]. However, the risk of visual loss remains a concern, particularly in infants, who are at high risk due to often large Optic Pathway Hypothalamic Gliomas (OPHGs) frequently presenting with intracranial extension [7].

Conclusion

In summary, the concept of "recurrent glioma" in childhood visual pathway tumors is no longer a relevant term. Instead, pediatric low-grade gliomas are recognized as a distinct entity, requiring personalized treatment approaches that take into account their unique characteristics and the individual child's needs.

References:

[1] Pediatric low-grade gliomas (pLGG) represent 40%–50% of all pediatric CNS tumours and are the most common central nervous system (CNS) tumours in children [1].

[2] pLGG most frequently arises in the cerebellum (35%), followed by the cerebral hemispheres (17%), the optic pathway and hypothalamic region (15%) and brainstem (9%) [2, 3].

[5] The term “optic pathway glioma” is reserved for those tumors confined to the precortical visual pathway, sometimes with the involvement of the hypothalamus.

[6] Surgery was once considered a primary treatment option but is now often used in conjunction with other modalities, such as chemotherapy and radiation therapy, to achieve optimal outcomes [6].

[7] Infants are at high risk due to often large Optic Pathway Hypothalamic Gliomas (OPHGs) frequently presenting with intracranial extension [7].

Based on the provided context, here are the signs and symptoms associated with optic pathway gliomas (OPGs) in children:

Visual Impairment: Visual impairment is the most prevalent manifestation of OPGs, occurring in approximately 94.4% of cases [12]. This can range from mild visual disturbances to severe visual loss.

Other Symptoms:

• Nystagmus: Nystagmus, or involuntary eye movements, may occur at different stages of the disease.
• Proptosis: Proptosis, or bulging eyes, can be a symptom of OPGs.
• Visual Loss: Visual loss is another common symptom of OPGs.
• Hydrocephalus: Hydrocephalus, or fluid accumulation in the brain, may occur in some cases.
• Diencephalic Syndrome: Diencephalic syndrome, characterized by growth failure and weight gain, can be a manifestation of OPGs.
• Neurological Deficits: Neurological deficits, such as weakness or paralysis, may occur in some cases.
• Growth and Developmental Delays: Growth and developmental delays can also be associated with OPGs.

Clinical Features:

• The presenting symptoms of childhood gliomas depend on the anatomical location of the tumor [2].
• The size of the tumor is another factor that influences the clinical features of OPGs.

It's essential to note that these symptoms can vary in severity and may not be present in all cases. A proper diagnosis by a medical professional is necessary for an accurate assessment and treatment plan.

References:

[1] [2] [3] [4] [5] [12]

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

Based on the provided context, it appears that there are various diagnostic tests and recommendations for children with recurrent glioma of the visual pathway.

Recommended Diagnostic Tests

• Examination of the diagnostic tissue by an experienced neuropathologist is strongly recommended, along with molecular testing, if available [3].
• An ophthalmological examination by an experienced ophthalmologist should be included in the assessment of any child presenting with a visual pathway tumour [4].

Laboratory Assessments

• Laboratory assessments, including complete blood count, full chemistry panel, liver function tests, and CPK, were done every 4 weeks [2].

Imaging Studies

• Brain MRI is the gold standard neuroimaging examination for the diagnosis of OPG in symptomatic patients with NF1 [9].
• MRI and magnetic resonance angiogram provide valuable information about the relationships of the tumor to surrounding structures [10].

It's worth noting that the specific diagnostic tests recommended may vary depending on the individual child's condition and medical history.

References: [1] Not applicable [2] by J Fangusaro · 2021 · Cited by 117 [3] Jun 17, 2024 [4] by S Picton [5] Not applicable [6] Not applicable [7] Not applicable [8] Not applicable [9] by M Cassina · 2019 · Cited by 44 [10] Apr 10, 2018

Current Treatment Options for Recurrent Gliomas in Childhood Visual Pathway

Recurrent gliomas in the childhood visual pathway can be challenging to treat, and the most effective approach often involves a combination of therapies. While there is no single "cure" for these tumors, various treatment options are available to manage symptoms and slow disease progression.

Chemotherapy: A Common Treatment Approach

Chemotherapy remains a primary treatment option for recurrent gliomas in children. This approach involves administering medications that target rapidly dividing cancer cells, thereby slowing or stopping tumor growth. The typical chemotherapy regimen for childhood visual pathway gliomas includes weekly carboplatin and vincristine, which has been shown to be effective in controlling newly diagnosed, progressive low-grade gliomas in children [3].

Radiation Therapy: An Alternative Option

In cases where chemotherapy is ineffective or the tumor is resistant to treatment, radiation therapy may also be considered. This approach involves using high-energy beams to kill cancer cells and shrink tumors. Radiation therapy can be used alone or in combination with other treatments, such as surgery or chemotherapy.

Targeted Therapies: Promising New Options

Recent studies have explored the use of targeted therapies, including MEK and BRAF inhibitors, for treating childhood visual pathway gliomas [15]. These medications work by targeting specific molecular pathways involved in cancer cell growth and survival. While still relatively new, these treatments show promise in improving outcomes for children with recurrent gliomas.

Surgery: A Consideration for Residual Tumors

In some cases, surgery may be necessary to remove residual tumor tissue or alleviate symptoms caused by the growing tumor. This approach is typically considered when other treatments have failed or are no longer effective.

Current Research and Future Directions

Ongoing research aims to identify more effective treatment strategies for childhood visual pathway gliomas. Studies investigating new targeted therapies, immunotherapies, and combination regimens continue to advance our understanding of these complex tumors. As a result, patients with recurrent gliomas may have access to innovative treatments that improve their quality of life and overall prognosis.

References:

[3] Weekly carboplatin and vincristine for childhood visual pathway gliomas [15] MEK and BRAF inhibitors in pediatric low-grade gliomas

Based on the provided context, it appears that you are looking for information on the differential diagnosis of a specific type of tumor, namely an "obsolete recurrent glioma" affecting the visual pathway in children.

Possible Causes and Considerations

According to the search results, the differential diagnosis for gliomas is quite extensive [15]. In the case of pediatric low-grade gliomas (pLGG), which are the most common central nervous system (CNS) tumors in children, the main diseases to consider for differential diagnosis include:

• CNS infections, such as viral encephalitis
• Primary epilepsy
• Paediatric demyelinating CNS syndromes

Additionally, a list of main diseases to consider for differential diagnosis of paediatric glioma cases is given in Table 2 [15].

Specific Considerations for Visual Pathway Gliomas

Regarding visual pathway gliomas specifically, it's worth noting that these tumors can be challenging to diagnose due to their location and the fact that they often present with non-specific symptoms. However, some possible differential diagnoses to consider include:

• Optic neuritis
• Papilledema
• Retinal detachment

Key Points to Consider

When considering a diagnosis of an obsolete recurrent glioma affecting the visual pathway in children, it's essential to keep the following points in mind:

• The tumor is likely to be a low-grade glioma, such as a pilocytic astrocytoma or an optic pathway glioma
• The patient may experience vision loss or other non-specific symptoms
• A comprehensive review of the literature on visual pathway gliomas in childhood, such as that written by Dutton in 1994 [13], can provide valuable insights into the epidemiology and clinical presentation of these tumors.

References

[1] Table 2 from reference [15] [2] Reference [14] [3] Reference [12] [4] Reference [11] [5] Reference [10]

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