medulloblastoma non-WNT/non-SHH group 4

Medulloblastoma Non-WNT/Non-SHH Group 4: Key Characteristics

Medulloblastoma non-WNT/non-SHH group 4 is a subtype of medulloblastoma, a malignant brain tumor that primarily affects children. This subgroup accounts for approximately 35% of all medulloblastoma cases [9]. The key characteristics of this group are:

• Lack of MYC amplifications: Unlike other subgroups, non-WNT/non-SHH group 4 medulloblastomas do not exhibit MYC amplifications [8].
• Absence of TP53 mutations: This subgroup is also characterized by the absence of TP53 mutations [1].
• Potential chromosome 17 abnormalities: Chromosome 17 abnormalities may be present in some cases, but this is not a defining feature of non-WNT/non-SHH group 4 medulloblastomas [8].

Clinical Significance

Non-WNT/non-SHH group 4 medulloblastomas are the most common subgroup and have a significant impact on global health [5]. Infants with SHH-activated medulloblastoma (SHH-1 and SHH-2) do not receive radiation to the brain, whereas those in this group may require different treatment approaches [7].

References

[1] Context 1 [8] Context 8 [9] Context 9

Common Signs and Symptoms of Medulloblastoma Non-WNT/Non-SHH Group 4

Medulloblastoma is a type of brain tumor that can affect children, and the non-WNT/non-SHH group 4 subtype has its own set of symptoms. Here are some common signs and symptoms associated with this particular group:

• Morning Headaches: These headaches tend to be worse in the morning and can be a result of increased intracranial pressure due to the growing tumor or hydrocephalus [1].
• Nausea and Vomiting: Many children with medulloblastoma experience nausea and vomiting, which can be caused by the tumor's mass effect or obstructive hydrocephalus [6][10].
• Feeling Extremely Tired: As the tumor grows, it can cause lethargy and a general feeling of being extremely tired [6].
• Loss of Balance and Coordination: The growing tumor in the cerebellum can affect balance and coordination, leading to stumbling, falling, and clumsiness [8][9].
• Changes in Mood and Behavior: Some children may exhibit changes in their mood and behavior that don't fit the situation or are out of character for them [5].

It's essential to note that these symptoms can vary depending on the location and size of the tumor. If you suspect that your child is experiencing any of these symptoms, it's crucial to consult with a medical professional for proper evaluation and treatment.

References: [1] - Context result 3 [5] - Context result 5 [6] - Context result 6 [8] - Context result 8 [9] - Context result 9 [10] - Context result 10

Medulloblastoma, specifically the non-WNT/non-SHH group 4 subtype, requires accurate diagnosis and classification to determine the most effective treatment plan.

• Magnetic Resonance Imaging (MRI): This diagnostic test is considered the gold standard for medulloblastoma diagnosis. MRI with gadolinium DTPA administration can provide detailed images of the brain tumor, allowing neuropathologists to assess its characteristics [5].
• Tumor tissue analysis: A thorough examination of the tumor tissue by a neuropathologist is essential for accurate classification and grading of medulloblastomas. This involves analyzing the tumor's molecular features, including the presence or absence of specific genetic mutations [2].

It's worth noting that the non-WNT/non-SHH group 4 subtype represents the largest molecular group of medulloblastoma, and its diagnosis requires molecular testing for an accurate classification [8]. This information is crucial in determining the most effective treatment plan and prognosis for patients with this type of tumor.

References: [2] Aug 20, 2024 — Primary CNS tumors are graded based on a tumor tissue analysis performed by a neuropathologist. Medulloblastomas are all classified as grade 4 (... [5] Sep 27, 2022 — MRI with the administration of gadolinium DTPA is the diagnostic test of choice for medulloblastoma. [8] by BA Orr · 2020 · Cited by 136 — Medulloblastoma, non-WNT/non-SHH represents the largest molecular group of MB. It is comprised of the provisional G3 and G4 tumors, representing...

Current Drug Treatments for Medulloblastoma Non-WNT/Non-SHH Group 4

Medulloblastoma, a type of brain cancer, is classified into several subgroups based on molecular characteristics. The non-WNT/non-SHH subgroup, specifically Group 4, accounts for the most common molecular subgroup and is more prevalent among males.

Standard-of-Care Treatment Options

For average-risk patients in this subgroup, excellent survival rates have been achieved with current standard-of-care treatment options [15]. However, high-risk patients require more aggressive treatments.

Chemotherapy Regimens

High-risk patients in the non-WNT/non-SHH stratum receive a combination of chemotherapy drugs, including gemcitabine and pemetrexed [5]. These regimens aim to target cancer cells while minimizing side effects.

Emerging Treatment Options

Research is ongoing to identify new treatment options for non-WNT/non-SHH medulloblastoma. Vismodegib (GDC-0449), a Food and Drug Administration (FDA)-approved SMO inhibitor, has shown promise in treating SHH-dependent cancer but requires further investigation for MB [7].

Current Clinical Trials

Several clinical trials are underway to evaluate new treatment options for non-WNT/non-SHH medulloblastoma. These studies aim to improve patient outcomes by reducing treatment-related toxicity and side effects [11].

Treatment Approaches

In addition to chemotherapy, treatment decisions for Group 4 patients may consider factors such as age, extent of disease burden, and molecular/pathology classification. Treatment approaches, including the number of rounds of chemotherapy and specific drugs used, may vary depending on individual patient needs.

References: [5] Thompson EM. Patients with WNT or SHH medulloblastoma receive the same three to five cycles induction chemotherapy as the non-WNT/non-SHH but do not undergo randomization... [7] Vismodegib (GDC-0449), a Food and Drug Administration (FDA)-approved SMO inhibitor, has shown promise in treating SHH-dependent cancer but requires further investigation for MB. [11] Non-WNT/non-SHH medulloblastoma is currently treated with surgery, chemotherapy, and radiotherapy; however, new drugs are needed to treat patients who are not yet curable and to reduce treatment-related toxicity and side effects.

Medulloblastoma, particularly the non-WNT/non-SHH group 4 subtype, can be challenging to diagnose differentially due to its complex molecular characteristics. However, based on the available information, here are some key points to consider:

• Ependymoma: This is a primary concern in differential diagnosis for medulloblastomas, especially those arising from the floor of the 4th ventricle. Ependymomas typically squeeze out the foramen of Luschka and do not exhibit the same bimodal age distribution as medulloblastomas [4].
• Other molecular subtypes: While less common, other molecular subtypes of medulloblastoma, such as WNT-activated or SHH-activated, may also need to be considered in differential diagnosis. However, these subtypes have distinct molecular characteristics that can help differentiate them from group 4 medulloblastomas [2].
• TPD52 immunopositivity: TPD52 positivity is an independent predictor of adverse outcome for group 3/4 medulloblastoma, which may be relevant in differential diagnosis [7].

It's essential to note that the prognosis and treatment approach can vary significantly depending on the molecular subtype of medulloblastoma. Therefore, accurate classification and differentiation from other tumors are crucial for determining the best course of action.

References:

[4] Oct 14, 2024 - Differential diagnosis; ependymoma. [7] Jan 5, 2022 - TPD52 immunopositivity is an independent predictor of adverse outcome for group 3/4 medulloblastoma.