extracranial neuroblastoma

Diagnostic Tests for Extracranial Neuroblastoma

Extracranial neuroblastoma, a type of cancer that affects the nervous system, requires a combination of diagnostic tests to confirm its presence and determine its stage. Here are some of the common diagnostic tests used:

• Imaging tests: These include CT scans (Computed Tomography), MRI scans (Magnetic Resonance Imaging), and ultrasound. CT scanning is the modality most commonly used to diagnose and stage neuroblastomas, with about 80-90% of neuroblastomas showing stippled calcifications [4].
• Bone scan: This test uses a small amount of radioactive material to detect any abnormalities in the bones.
• Metaiodobenzylguanidine (MIBG) scanning: MIBG is a critical part of the standard diagnostic evaluation of neuroblastoma, for both the primary tumor and metastatic disease [6].
• Blood tests: These include complete blood count, blood chemistries, kidney, and liver function tests.
• Urine tests: These may indicate the cause of any signs and symptoms your child is experiencing. Initial laboratory testing includes vanillylmandelic acid (VMA) and homovanillic acid (HVA) urine tests [1].
• Bone marrow aspiration and biopsy: This test can help determine if the cancer has spread to the bone marrow.
• Physical exam: A physical exam by a doctor is also an important part of diagnosing neuroblastoma.

Additional Tests

Other tests that may be used to diagnose and stage neuroblastoma include:

• Lymph node biopsy or MRI
• Abdomen and pelvis MRI
• Computerized tomography (CT) scan
• Bone marrow aspiration and biopsy

These diagnostic tests help doctors confirm the presence of extracranial neuroblastoma, determine its stage, and develop a treatment plan.

References: [1] - Initial laboratory testing includes vanillylmandelic acid (VMA) and homovanillic acid (HVA) urine tests. [4] - CT scanning is the modality most commonly used to diagnose and stage neuroblastomas, with about 80-90% of neuroblastomas showing stippled calcifications. [6] - MIBG is a critical part of the standard diagnostic evaluation of neuroblastoma, for both the primary tumor and metastatic disease.

Treatment Options for Extracranial Neuroblastoma

Extracranial neuroblastoma, a type of cancer that affects the sympathetic nervous system, can be treated with various drug therapies. The treatment approach often depends on the stage and risk level of the disease.

• Chemotherapy: Multiagent chemotherapy consisting of doxorubicin, cyclophosphamide, a platinum drug, and etoposide is commonly used to treat neuroblastoma [2]. This combination has shown promising results in clinical trials.
• Targeted Therapy: Small molecule inhibitors such as ALK inhibitors have been explored for their potential in treating neuroblastoma. A recently approved inhibitor has demonstrated pre-clinical activity and is currently being tested in phase I and II trials [3].
• Combination Therapies: Researchers are investigating the use of combination therapies, including chemotherapy and targeted therapy, to improve treatment outcomes. For example, a study found that combining DFMO with AMXT starved neuroblastoma cells, leading to promising results [5].

Current Treatment Guidelines

The National Comprehensive Cancer Network (NCCN) provides guidelines for the diagnosis, risk classification, and treatment of neuroblastoma. These guidelines recommend multiple-agent chemotherapy as a standard treatment approach [10]. Additionally, the NCCN suggests considering targeted therapy options, such as ALK inhibitors, in certain cases.

Key Statistics

• Neuroblastoma accounts for approximately 15% of all cancer-related deaths in children [11].
• The overall survival rate for high-risk neuroblastoma patients is around 40-50%, despite aggressive treatment protocols [14].

References:

[1] VR Ganeshan (2011) - High-risk patients are typically treated with multimodal therapy, including intensive induction chemotherapy...

[2] Nov 12, 2024 - Multiagent chemotherapy consisting of doxorubicin, cyclophosphamide, a platinum drug, and etoposide is used.

[3] by KK Matthay (2012) - A recently approved small molecule inhibitor of ALK has promising pre-clinical activity for neuroblastoma...

[5] Jul 11, 2024 - We're expanding our work on DFMO in neuroblastoma to include a drug called AMXT.

[10] Neuroblastoma is the most common extracranial solid tumor diagnosed in children. This inaugural version of the NCCN Guidelines for Neuroblastoma provides recommendations...

[14] by V Smith (2018) - Treatment includes chemotherapy, surgical resection, high-dose chemotherapy with autologous stem cell rescue...