obsolete recurrent pediatric optic nerve astrocytoma

Obsolete Recurrent Pediatric Optic Nerve Astrocytoma

Obsolete recurrent pediatric optic nerve astrocytoma, also known as pilocytic astrocytoma (PCA), is a rare and aggressive type of brain tumor that primarily affects children and young adults. This condition can occur anywhere in the central nervous system, but it most commonly affects the optic nerve.

Key Characteristics:

• Age Group: Primarily affects children and young adults
• Location: Can occur anywhere in the central nervous system, but most commonly affects the optic nerve
• Grade: Low-grade glioma (WHO Grade I)
• Recurrent: Tendency to recur after treatment

Symptoms:

The clinical presentation of astrocytoma symptoms is similar to most brain tumors with both focal and generalized neurologic signs. Focal symptoms include:

• Visual disturbances, such as blurred vision or loss of vision
• Headaches
• Seizures
• Weakness or numbness in the face or limbs

Treatment Options:

Treatment options for children with astrocytomas, other gliomas, and glioneuronal/neuronal tumors include observation, surgery, chemotherapy, and radiation therapy. The choice of treatment depends on various factors, including the tumor's location, size, and grade.

References:

• [1] SG Menon (2017) - Pilocytic astrocytoma is a low-grade glioma that affects mostly children and young adults and can occur anywhere in the central nervous system.
• [3] Obsolete recurrent pediatric optic nerve astrocytoma. Term ID: DOID:6728; Synonyms. Definition: References: Obsolete: true; Ontology: Human Disease ...
• [4] Obsolete recurrent pediatric optic nerve astrocytoma. Term ID: DOID:6728; Synonyms. Definition: References: Obsolete: true; Ontology: Human Disease ...
• [5] J Fangusaro (2021) - Sporadic (without neurofibromatosis type 1) optic pathway and hypothalamic gliomas (OPHGs) are often multiply recurrent and cause significant visual deficits.
• [8] M Willman (2023) - The clinical presentation of astrocytoma symptoms is similar to most brain tumors with both focal and generalized neurologic signs. Focal symptoms include ...
• [9] Mar 2, 2024 - Rationale: With an incidence of 0.8 per 100,000 people, pilocytic astrocytoma (PCA) is the most common childhood brain tumor.

Based on the provided context, here are the signs and symptoms of recurrent pediatric optic nerve astrocytoma:

Common Symptoms

• Decreased visual acuity [2]
• Visual field deficits [2]
• Optic disc swelling and/or disc pallor [2]
• Proptosis (bulging eyes) [3]
• Visual evoked potentials (VEP) abnormalities [2]

Clinical Presentation

The clinical presentation of astrocytoma symptoms is similar to most brain tumors, with both focal and generalized neurologic signs. Focal symptoms include:

• Blurred vision [5]
• Other visual disturbances
• Seizures or other neurological deficits

Severe Symptoms

In severe cases, the tumor can cause more significant symptoms, including:

• Deteriorating visual behavior [7]
• Raised intracranial pressure [10]

It's essential to note that these symptoms may vary in severity and presentation depending on the individual child. If you suspect a child has a recurrent pediatric optic nerve astrocytoma, it is crucial to consult with a medical professional for proper diagnosis and treatment.

References: [1] Not applicable (since there are no relevant results) [2] Context result 2 [3] Context result 3 [4] Not applicable (since there are no relevant results) [5] Context result 5 [6] Not applicable (since there are no relevant results) [7] Context result 7 [8] Context result 8 [9] Context result 9 [10] Context result 10

Recurrent Pediatric Optic Nerve Astrocytoma Diagnostic Tests

The diagnostic tests for recurrent pediatric optic nerve astrocytoma have evolved over time to include various imaging and molecular techniques.

• Imaging Studies: Magnetic Resonance Imaging (MRI) is a crucial diagnostic tool for assessing the extent of tumor recurrence. A second review by a pediatric neuroradiologist can confirm the diagnosis of isolated optic nerve glioma [7].
• Ophthalmological Examination: A thorough ophthalmological examination, including fundus photography and ocular coherence tomography, can help identify changes in the optic nerve and surrounding tissues.
• Visual Function Assessment: Standardized methods to assess visual function in children with OPG have been agreed upon. An ophthalmological examination by an experienced professional is essential [5].
• Genetic Testing: Genetic testing may be recommended for children with a family history of neurofibromatosis type 1 (NF1), as they are at higher risk of developing optic nerve tumors.
• Molecular Analysis: Molecular analysis, including immunohistochemical analysis and cytogenetic studies, can provide valuable information on the tumor's molecular features and help guide treatment decisions [2].

Key Takeaways

• MRI is a critical diagnostic tool for assessing tumor recurrence.
• Ophthalmological examination and visual function assessment are essential for monitoring changes in the optic nerve and surrounding tissues.
• Genetic testing may be recommended for children with a family history of NF1.
• Molecular analysis can provide valuable information on the tumor's molecular features.

References

[2] PDQPTE Board · 2009 — Immunohistochemical analysis, cytogenetic and molecular genetic findings, and measures of mitotic activity are used in tumor diagnosis and classification. [5] by S Picton — Therefore, standardized methods to assess visual function in children with OPG have now been agreed. An ophthalmological examination by an experienced professional is essential. [7] Mar 5, 2018 — All MRI studies received a second review by a pediatric neuroradiologist (J.H.H.) to confirm the diagnosis of isolated optic nerve glioma.

Based on the provided context, it appears that there are several treatment options available for recurrent pediatric optic nerve astrocytomas.

• Chemotherapy: Most often, chemotherapy is used to treat optic nerve gliomas. Chemotherapy can stop the progression of these tumors, but if they become resistant, radiation therapy may also be an option [5][7].
• MEK/BRAF inhibitors: A combination of chemotherapy, MEK/BRAF inhibitors, and bevacizumab has been found to be useful in treating optic nerve gliomas. This treatment approach has shown promise in some patients [8].
• Bevacizumab and irinotecan: Combination therapy with bevacizumab and irinotecan has achieved a 2-year survival rate of 47.8% in patients with recurrent low-grade gliomas (LGGs) [9].

It's worth noting that the prognosis for children with isolated optic nerve tumors is generally better than those with lesions that involve the chiasm or extend along the optic pathway [12]. Additionally, children with neurofibromatosis type 1 (NF1) have a better prognosis, especially when the tumor is found in asymptomatic patients at the time of screening [12].

In terms of specific drug treatments, lenalidomide has been studied as a potential treatment for recurrent pediatric optic nerve astrocytomas. A randomized phase II trial compared low-dose lenalidomide with high-dose lenalidomide in treating children with juvenile pilocytic astrocytomas or optic nerve pathway gliomas that have come back (recurrent), have not responded to treatment (refractory), or are growing, spreading, or getting worse (progressive) [15].

References: [5] - Children with isolated optic nerve tumors have a better prognosis than those with lesions that involve the chiasm or that extend along the optic pathway.[61-64]; [Level of evidence: 3iiC] [7] - Chemotherapy can stop the progression of these tumors, but if they become resistant, radiation therapy may also be an option. [8] - A combination of chemotherapy, MEK/BRAF inhibitors, and bevacizumab has been found to be useful in treating optic nerve gliomas. This treatment approach has shown promise in some patients. [9] - Combination therapy with bevacizumab and irinotecan has achieved a 2-year survival rate of 47.8% in patients with recurrent low-grade gliomas (LGGs). [12] - Children with isolated optic nerve tumors have a better prognosis than those with lesions that involve the chiasm or extend along the optic pathway. [15] - A randomized phase II trial compared low-dose lenalidomide with high-dose lenalidomide in treating children with juvenile pilocytic astrocytomas or optic nerve pathway gliomas that have come back (recurrent), have not responded to treatment (refractory), or are growing, spreading, or getting worse (progressive).

Based on the provided context, it appears that differential diagnosis for recurrent pediatric optic nerve astrocytoma may include other conditions such as:

• Inflammatory orbital pseudotumour [4]
• Thyroid orbitopathy [4]
• Infective orbital inflammations [4]

Additionally, the following conditions may also be considered in the differential diagnosis of pediatric optic nerve tumors:

• Isolated optic nerve glioma [5]
• Other tests such as positron emission tomography, magnetic resonance spectroscopy, and imaging studies may be used to confirm or rule out these conditions [6]

It's worth noting that the most common type of glioma in children is astrocytoma, which can present a challenge for clinicians due to its diffuse nature and unclear borders [8][9]. Primary brain tumors, including gliomas, are a diverse group of diseases that together constitute the most common solid tumors of childhood [10].

References: [4] - Inflammatory orbital pseudotumour, thyroid orbitopathy, infective orbital inflammations were considered as differential diagnosis keeping painful axial ... [5] - Mar 5, 2018 — All MRI studies received a second review by a pediatric neuroradiologist (J.H.H.) to confirm the diagnosis of isolated optic nerve glioma. [6] - by T Care — ... recurrence, is in the differential diagnosis. Other tests, including positron emission tomography, magnetic resonance spectroscopy, and ... [8] - Apr 19, 2022 — Astrocytoma is the most commonly diagnosed type of glioma in children. According to the World Health Organization (WHO) classification of brain ... [9] - by M Willman · 2023 · Cited by 5 — Diffuse grade 2 astrocytomas present a much greater challenge for clinicians as tumor borders are no longer well-delineated, complicating resection and ... [10] - by M PeaceHealth — Primary brain tumors, including gliomas, are a diverse group of diseases that together constitute the most common solid tumors of childhood.