What is Neuroblastoma?

Types of Neuroblastoma

• Localized Neuroblastoma : Confined to its origin without spreading, often easier to treat with a better prognosis.
• Metastatic Neuroblastoma : Cancer spread to distant body parts like bones, bone marrow, or organs, requiring intensive treatment.
• Stage 4 Neuroblastoma (Special Clinical Stage 4 or 4S) : Occurs in infants ≤1 year old with localized primary tumors and distant metastasis, often with a good prognosis; some cases may regress without extensive treatment.
• INSS Staging : Categorizes neuroblastoma from Stage 1 (localized) to Stage 4 (widespread metastasis), with specific criteria like tumor size and lymph node involvement.
• Histopathological Classification : Based on tumor cell appearance under a microscope, predicting behavior and treatment response; includes favorable and unfavorable histology subtypes.
• Segmental Chromosome Aberration : Genetic features influencing neuroblastoma classification and prognosis, including specific chromosomal deletions or alterations.

Symptoms of Neuroblastoma

• Abdominal Swelling : A noticeable lump in the belly that makes it look larger.
• Pain : Children may feel pain in their belly, chest, bones, or other parts of their body.
• Fatigue : Feeling very tired or weak without a clear reason.
• Fever : Having a high body temperature that may come and go.
• Loss of Appetite and Weight Loss : Not feeling hungry and losing weight without trying.
• Bulging Eyes: Eyes that stick out more than usual because of a tumor behind them.
• Skin Changes : Skin turning bluish or showing veins near where the tumor is.
• Urinary Symptoms : Changes in how often a child pees or trouble peeing.
• Bowel Changes : Changes in how often a child poops or stomach problems like constipation.
• Difficulty Breathing : Trouble breathing, wheezing, or coughing caused by a tumor pressing on the airways.
• Bone Pain : Pain in bones or joints if the cancer has spread there.
• Neurological Symptoms : Weakness, numbness, or trouble moving due to the tumor affecting nearby nerves.

When to See a Doctor?

Take a doctor's appointment if you are getting tired quickly and need to know why. Your haemoglobin count has fallen if you observe the above Neuroblastoma symptoms or during a blood test.

Causes of Neuroblastoma

• Genetic Mutations : Changes in genes that control cell growth can cause neuroblastoma by making nerve cells grow uncontrollably. These mutations can happen randomly or be inherited.
• Chromosomal Abnormalities : Alterations in DNA segments, like deletions or extra copies, can disrupt normal cell functions and contribute to neuroblastoma.
• MYCN Amplification : Abnormal increases in the MYCN gene are linked to more aggressive forms of neuroblastoma.
• Neuroblastoma Predisposition : Inherited genetic mutations increase the risk of neuroblastoma in some families, making nerve cells more prone to tumor formation.
• Environmental Factors : Factors during pregnancy, like exposure to chemicals, drugs, or infections, may influence neuroblastoma risk, although this link is not fully understood.
• Age : Neuroblastoma is most common in children under 5 years old, suggesting early childhood development factors may play a role in its development.

Risk Factors for Neuroblastoma

• Age : Neuroblastoma is most often found in young children, especially those under 5 years old. Babies are at the highest risk.
• Genetic Predisposition : Some children inherit genetic changes that make them more likely to get neuroblastoma. Families with a history of this cancer, or related cancers like MEN2, are at higher risk due to these genetic mutations affecting nerve cell development.
• Family History : If close family members like siblings, parents, or grandparents have had neuroblastoma or similar cancers, the risk increases. Certain genetic conditions, such as familial neuroblastoma or MEN2, also raise the likelihood.
• Chromosomal Abnormalities : Changes in chromosomes or genetic mutations that affect how nerve cells grow and mature can make someone more susceptible to neuroblastoma.
• MYCN Amplification : In some cases of neuroblastoma, there is an abnormal increase in a gene called MYCN, which can contribute to the development and progression of the cancer.

Complications of Neuroblastoma

• Metastasis : Neuroblastoma can spread to bones, bone marrow, lymph nodes, liver, and lungs, causing additional symptoms and affecting treatment options.
• Tumour Compression : Large or fast-growing tumours can press on nearby structures, causing pain or dysfunction. For example, abdominal tumours can affect digestion.
• Obstruction : Tumours in the abdomen or pelvis can block the gastrointestinal or urinary tracts, causing difficulties in passing stool or urine.
• Respiratory Issues : Chest or airway tumors can lead to breathing difficulties, wheezing, and coughing.
• Bone Pain : If neuroblastoma spreads to bones, it can cause pain and increase fracture risk.
• Anaemia : Tumours can affect bone marrow's ability to produce red blood cells, leading to anaemia and fatigue.
• Malnutrition and Weight Loss : Cancer-related changes like loss of appetite and metabolic effects can cause malnutrition and weight loss.
• Impact on Growth and Development : Neuroblastoma and its treatments can affect a child's growth, development, and overall well-being.

Diagnosis of Neuroblastoma

• Medical History and Examination : The doctor will ask about symptoms, family history, and examine the child for signs like a belly lump.
• Ultrasound : Uses sound waves to create images of organs, helping to visualize and assess the tumor.
• CT Scan : Provides detailed cross-sectional images of the body to detect and stage the tumor.
• MRI : Produces detailed images using magnets and radio waves to locate and evaluate the tumor.
• MIBG Scan : Uses a radioactive substance to detect neuroblastoma cells in the body.
• Biopsy : Involves taking a small tumor sample to confirm the diagnosis and determine its type and aggressiveness through microscopic examination by pathologists.

Bone Marrow Aspiration and Biopsy

• Bone Marrow Aspiration : Checks if cancer has spread by taking a small sample from the hip bone for examination.
• Laboratory Tests : Urine and blood samples are analyzed for substances like catecholamines and other markers that can show neuroblastoma.
• Genetic and Molecular Testing : Identifies specific genetic mutations linked to neuroblastoma.

Normal Hemoglobin Levels in Children

• Newborns : 14 to 24 g/dL
• Infants : 9.5 to 13.5 g/dL
• Children (1-6 years) : 9.5 to 14 g/dL
• Children (7-12 years) : 11.5 to 15.5 g/dL
• Adolescents (13-18 years) : 12 to 16 g/dL

Treatment of Neuroblastoma

• Observation (Watchful Waiting) : Some neuroblastomas may be monitored without immediate treatment to assess their behavior, typically for small, low-risk tumors.
• Surgery : Common for localized neuroblastomas, surgical removal varies based on tumor size and location.
• Chemotherapy : Powerful medications used to destroy cancer cells or shrink tumors. Administered before or after surgery.
• Radiation Therapy : High-energy beams target and destroy cancer cells, often used post-surgery or in select cases.
• Stem Cell Transplantation : May be necessary after high-dose chemotherapy or radiation to replace damaged marrow with healthy stem cells.
• Immunotherapy : Enhances the immune system to target and destroy cancer cells, utilizing treatments like monoclonal antibodies and cytokines.
• Targeted Therapy : Medications targeting specific genetic mutations or tumor characteristics to disrupt cancer cell growth and survival.
• Retinoid Therapy : Derivatives of vitamin A used to mature neuroblastoma cells.
• Supportive Care : Manages side effects, alleviates pain, and maintains overall well-being during treatment.

Neuroblastoma Dos and Don'ts