obsolete recurrent childhood glioma of brain stem

Obsolete Recurrent Childhood Glioma of Brain Stem

A recurrent childhood glioma of the brain stem, also known as diffuse intrinsic pontine glioma (DIPG), is a type of aggressive and fast-growing tumor that occurs in the brain stem. This condition is characterized by its poor prognosis and limited treatment options.

Key Characteristics:

• Age Group: Typically affects children and young adults under the age of 20.
• Location: Occurs in any location within the brain stem, including the midbrain, pons, and medulla.
• Type: A heterogeneous group of tumors regarding both clinical presentation and prognosis.
• Growth Rate: Fast-growing tumor that is difficult to treat.

Classification:

Brain stem gliomas can be classified into two main categories:

• Focal Glioma: Grows more slowly, is easier to treat, and has a better prognosis.
• Diffuse Intrinsic Pontine Glioma (DIPG): A fast-growing tumor that is difficult to treat and has a poor prognosis.

Treatment Options:

Current standard of care treatment for brain stem glioma recommends surgical resection with or without chemotherapy and/or radiation therapy depending on the histology. However, in the case of recurrent or progressive childhood brain gliomas like DIPG, there is no standard treatment available.

References:

• [1] Diffuse midline glioma (DMG) is a heterogeneous group of aggressive pediatric brain tumors with a fatal prognosis.
• [9] DIPG is the most common brainstem tumor of childhood. DIPG is almost uniformly fatal and is the leading cause of brain tumor-related death in children.
• [10] Current standard of care treatment for brain stem glioma recommends surgical resection with or without chemotherapy and/or radiation therapy depending on the histology.

Note: The term "obsolete recurrent childhood glioma of brain stem" is an outdated term, and the current preferred term is "diffuse intrinsic pontine glioma (DIPG)".

Common Signs and Symptoms

Children with an obsolete recurrent childhood glioma of the brain stem, also known as Diffuse Intrinsic Pontine Glioma (DIPG), may exhibit a range of symptoms. These can vary in severity and may include:

• Weakness or paralysis: Weakness or paralysis on one side of the body is a common symptom, affecting approximately 78% of patients [9].
• Abnormal gait and coordination difficulties: Abnormal gait and coordination difficulties are also frequent, reported by 78% of patients [9].
• Cranial nerve palsies: Cranial nerve palsies, which affect the nerves that control various bodily functions, such as swallowing or eye movement, can occur in approximately 52% of cases [9].
• Seizures: Seizures are a less common symptom but still occur in around 13% of patients [10].

Other Symptoms

In addition to these symptoms, children with DIPG may also experience:

• Headache: Headaches can be a symptom of increased intracranial pressure (ICP), which is often associated with brain tumors.
• Nausea and vomiting: Nausea and vomiting are common symptoms of increased ICP [2].
• Exhaustion and imbalance: Children may also experience exhaustion, imbalance, and blurred or double vision due to increased ICP [2].

Prognosis

Unfortunately, DIPG is almost uniformly fatal and is the leading cause of brain tumor-related death in children. The prognosis for this type of glioma is generally poor, with a 5-year survival rate of less than 3% [6][8].

References:

[1] Symptoms produced by increased ICP include headache, nausea, vomiting, exhaustion, imbalance, and blurred or double vision. Headache occurs in approximately ... (Search result 2) [9] The most common clinical presentation of brain stem tumors is with abnormal gait and coordination difficulties (78%), cranial nerve palsies (52%), pyramidal ... (Search result 9) [10] Seizures are one of the most frequent presenting symptom of pediatric brain tumors occurring in 13% of patients [8]. Seizures are more frequent with certain ... (

Childhood Brain Stem Glioma: Diagnosis, Classification, and Treatment

Childhood brain stem glioma is a type of cancer that affects the brain stem in children. It can be classified into two main types: diffuse intrinsic pontine glioma (DIPG) and focal glioma.

• Diffuse Intrinsic Pontine Glioma (DIPG): DIPG is a fast-growing tumor that is difficult to treat and has a poor prognosis [1]. It accounts for approximately 10-15% of all brain tumors in children [9].
• Focal Glioma: Focal glioma, on the other hand, grows more slowly and is easier to treat. It accounts for approximately 40% of all reported pediatric brain tumors [9].

Diagnosis

The diagnosis of childhood brain stem glioma is typically made by magnetic resonance imaging (MRI) of the brain, with and without gadolinium [8]. Neuroimaging reveals a tumor in the brain stem, which can be classified into DIPG or focal glioma based on its growth pattern and amenability to treatment.

Classification

Childhood brain stem gliomas can be classified on the basis of their biological behavior, anatomical location, and radiographic appearance on MRI [3]. The choice of treatment depends on the type and location of the tumor.

Treatment

The current standard of care (SOC) treatment for brainstem glioma recommends surgical resection with or without chemotherapy and/or radiation therapy in case of focal brain stem gliomas (FBSG) [10].

• Surgical Resection: Surgical resection is the primary treatment for focal glioma. It involves removing the tumor from the brain.
• Chemotherapy: Chemotherapy may be used in conjunction with surgery to treat focal glioma.
• Radiation Therapy: Radiation therapy may also be used to treat focal glioma.

Clinical Trials

There are ongoing clinical trials investigating new treatments for childhood brain stem glioma, including immunotherapy and targeted therapy [10].

In conclusion, childhood brain stem glioma is a type of cancer that affects the brain stem in children. It can be classified into DIPG and focal glioma based on its growth pattern and amenability to treatment. The diagnosis is typically made by MRI, and the choice of treatment depends on the type and location of the tumor.

References:

[1] MI Vanan (2015) - Cited by 225

[3] Classification of childhood brain stem glioma based on biological behavior, anatomical location, and radiographic appearance on MRI [3]

[8] Diagnosis of childhood brain stem glioma by magnetic resonance imaging (MRI) [8]

[9] Pediatric brain tumors: a review of the literature [9]

[10] Current standard of care (SOC) treatment for brainstem glioma recommends surgical resection with or without chemotherapy and/or radiation therapy in case of focal brain stem gliomas (FBSG) [10]

Based on the provided context, it appears that differential diagnosis for an obsolete recurrent childhood glioma of the brain stem would involve considering various factors and conditions.

Possible Differential Diagnoses

• Other types of tumors: According to search result [7], it is essential to differentiate between brain stem gliomas and other tumours.
• Infectious processes: Search results [4] and [12] mention that infectious processes, such as viral encephalitis, should be considered in the differential diagnosis.
• Vascular malformations: Search result [4] also mentions vascular malformations as a possible differential diagnosis.
• Storage diseases: Search result [7] lists storage diseases as another condition to consider.

Specific Conditions

• Diffuse Intrinsic Pontine Glioma (DIPG): This is a type of brain stem glioma that is known to be aggressive and difficult to treat. According to search results [3], DIPG is almost uniformly fatal, and it is the leading cause of brain tumor-related death in children.
• Focal Brain Stem Gliomas: Search result [14] mentions that focal brain stem gliomas can be treated with surgical resection, chemotherapy, and/or radiation therapy.

Other Considerations

• The age of the patient: Search results [9] mention that around 75% of brainstem gliomas are diagnosed in children and young adults under the age of twenty.
• The location of the tumor: Search result [8] mentions that the majority (80%) of brain stem gliomas are diffusely infiltrating gliomas of the ventral pons.

It is essential to note that differential diagnosis for an obsolete recurrent childhood glioma of the brain stem would require a comprehensive evaluation of various factors and conditions, taking into account the specific characteristics of the tumor and the patient's overall health.