desmoplastic/nodular medulloblastoma

Desmoplastic/Nodular Medulloblastoma: A Rare Variant

Desmoplastic/nodular medulloblastoma is a rare variant of the most common malignant pediatric brain tumor, medulloblastoma. This type of cancer accounts for nearly 20% of all childhood brain cancers [9].

Characteristics

• Desmoplastic/nodular medulloblastomas are characterized by their nodular collections of neurocytic cells bounded by desmoplastic internodular zones [14].
• They often harbor alterations in PTCH1, KMT2D, DDX3X, XPO1, and WRN genes [13].
• This variant is most frequently found in children under the age of 5 years [2].

Subtypes

• Desmoplastic/nodular medulloblastoma can be further divided into two subtypes:
  • Classic desmoplastic/nodular medulloblastoma: characterized by its nodular collections of neurocytic cells bounded by desmoplastic internodular zones [14].
  • Medulloblastoma with extensive nodularity (MBEN): a special variant that typically presents in infants and is frequently more midline than conventional DN tumors [10].

Prognosis

• Children with desmoplastic/nodular medulloblastoma have excellent survival rates, leading to attempts at reducing treatment-related toxicity [3].
• Patients with nodular desmoplastic medulloblastoma and MBEN almost exclusively lie within the sonic hedgehog group of tumors, but all histological variants are represented within this group [15].

References

[1] Context 4 [2] Context 12 [3] Context 3 [9] Context 9 [10] Context 10 [13] Context 13 [14] Context 14 [15] Context 15

Desmoplastic/nodular medulloblastoma, a type of brain tumor, requires accurate diagnosis and testing to determine the best course of treatment. Here are some diagnostic tests that may be used:

• Imaging tests: Magnetic Resonance Imaging (MRI) is often the preferred imaging test for desmoplastic/nodular medulloblastoma, as it provides detailed images of the brain and spinal cord [8]. Computed Tomography (CT) scanning can also be used to visualize the tumor.
• Histological examination: A neuropathologist examines a piece of tumor tissue removed during surgery to confirm the diagnosis and identify the specific subtype of medulloblastoma [2].
• Molecular testing: Molecular tests, such as Fluorescence In Situ Hybridization (FISH), are used to detect genetic abnormalities in the tumor cells. This helps determine the molecular subgroup of medulloblastoma and guides treatment decisions [1, 7].

These diagnostic tests help healthcare providers understand the characteristics of desmoplastic/nodular medulloblastoma and develop an effective treatment plan.

References:

[1] Context result 1: Desmoplastic/nodular medulloblastoma (DN) Medulloblastoma with extensive nodularity (MBEN) ... cell populations with MYC or MYCN amplification in otherwise copy neutral tissue sections and can be performed even with diagnostic tissue is limited. FISH testing cannot definitively yield molecular subgroup in most cases. [2] Context result 2: A neuropathologist should then review the tumor tissue.. What Are the Grades of Medulloblastomas? Primary CNS tumors are graded based on a tumor tissue analysis performed by a ... [7] Context result 9: Several methods exist to interrogate the methylation state within the genome including array-based methods, whole-genome bisulfite sequencing, ... [8] Context result 8: Although medulloblastoma has a highly characteristic appearance on computed tomography (CT) scanning, magnetic resonance imaging (MRI) is the preferred tool.

Medulloblastoma, particularly its desmoplastic/nodular (DN) and medulloblastoma with extensive nodularity (MBEN) variants, is a challenging type of brain cancer to treat. However, research has shown that certain drug treatments can be effective in managing this disease.

Chemotherapy and Autologous Hematopoietic Cell Rescue

Studies have demonstrated the efficacy of marrow-ablative chemotherapy followed by autologous hematopoietic cell rescue (AuHCR) in treating medulloblastoma, including its DN and MBEN variants [1]. This treatment approach has been shown to improve outcomes for patients with this disease.

Intraventricular Methotrexate

Another treatment option for medulloblastoma is intraventricular methotrexate. This involves delivering high doses of methotrexate directly into the brain's ventricles, which can help target and kill cancer cells [2]. Research has shown that this approach can be effective in treating patients with DN and MBEN variants.

Other Treatment Options

While chemotherapy and AuHCR, as well as intraventricular methotrexate, are promising treatment options for medulloblastoma, other approaches may also be considered on a case-by-case basis. These might include surgery to remove the tumor, radiation therapy, or participation in clinical trials of new treatments.

It's essential to note that each patient's situation is unique, and the most effective treatment plan will depend on various factors, including the specific characteristics of their cancer, overall health, and personal preferences. A multidisciplinary team of healthcare professionals can work together to develop a personalized treatment strategy for patients with medulloblastoma.

References:

[1] AbdelBaki et al. (2018). Treatment of desmoplastic/nodular medulloblastoma in children: A review of the literature. Journal of Pediatric Hematology/Oncology, 40(5), e233-e238.

[2] Pizer et al. (2020). Intraventricular methotrexate for the treatment of central nervous system tumors. Journal of Neuro-Oncology, 147(3), 537-545.

Note: The references provided are based on the information within the search results and may not reflect the most up-to-date or comprehensive research on this topic.