What is Neuroblastoma?

Neuroblastoma is a type of cancer that develops from immature nerve cells (neuroblasts) found in various body parts. It is most commonly diagnosed in young children, typically before age 5. It can originate in different areas, often in the adrenal glands on top of the kidneys. However, it can also arise in other nerve tissue along the spine, chest, abdomen, or pelvis.

Neuroblastoma is considered a childhood cancer and accounts for many cancers in infants and toddlers. It varies widely in its behaviour, ranging from slow-growing tumours that may resolve independently to aggressive forms requiring intensive treatment.

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Types of Neuroblastoma:

Localized Neuroblastoma : This type of neuroblastoma is confined to where it originated and has not spread to distant sites. It is often easier to treat and has a more favourable prognosis.

Metastatic Neuroblastoma : Metastatic neuroblastoma indicates that cancer has spread to other body parts, such as distant bones, bone marrow, lymph nodes, or other organs. Metastatic neuroblastoma is typically more challenging and may require a more intensive treatment approach.

Stage 4: Neuroblastoma (Special Clinical Stage 4) : This is a unique type of neuroblastoma that primarily occurs in infants younger than one-year-old. It involves localized primary tumours but also has distant metastasis to specific organs, such as the liver, skin, or bone marrow. Despite the widespread disease, stage 4S neuroblastoma often has a relatively good prognosis, and some cases may even regress without extensive treatment.

INSS Staging : Neuroblastoma can also be categorized using the International Neuroblastoma Staging System (INSS), which classifies cancer into different stages based on factors such as tumour size, lymph node involvement, and metastasis. The locations range from Stage 1 (localized tumour) to Stage 4 (widespread metastasis) and include subcategories.

Histopathological Classification : Neuroblastomas can be classified based on how the tumour cells look under a microscope. This classification helps predict the tumour's behaviour and how it might respond to treatment. Subtypes include favourable histology (cells look relatively normal) and unfavourable histology (cells are more abnormal).

Segmental Chromosome Aberration : Genetic characteristics of the tumour cells can also influence the classification and prognosis of neuroblastoma. Segmental chromosome aberrations, such as specific chromosomal deletions or alterations, categorize the cancer into different groups.

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Symptoms of neuroblastoma include:

Abdominal Swelling : A noticeable lump or mass in the abdomen, often causing the stomach to appear enlarged or distended. This is one of the most common symptoms of neuroblastoma.

Pain : Children may experience pain or discomfort in the affected area, which can be localized to the abdomen, chest, bones, or other body parts.

Fatigue : Unexplained tiredness, weakness, or lack of energy.

Fever : Elevated body temperature, which may be persistent or recurrent.

Loss of Appetite and Weight Loss : Decreased interest in eating and unexplained weight loss, often leading to poor growth.

Bulging Eyes : Protruding or bulging eyes (proptosis) due to tumour growth behind the eye socket. This can cause changes in eye appearance and vision problems.

Skin Changes : Bluish discolouration of the skin, visible veins, or other skin changes over the tumour area.

Urinary Symptoms : Changes in urinary habits, such as frequent urination or urinary retention.

Bowel Changes : Changes in bowel habits, constipation, or other gastrointestinal symptoms.

Difficulty Breathing : Tumors in the chest or neck can press on the airways or other structures, leading to breathing difficulties, wheezing, or chronic cough.

Bone Pain : Pain in the bones, joints, or other skeletal areas if the cancer has spread to the bones.

Neurological Symptoms : Depending on the tumour's location, it may affect nearby nerves and lead to weakness, numbness, or difficulty moving.

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 (mutations) in specific genes involved in cell growth, differentiation, and development can lead to the uncontrolled growth of nerve cells and the formation of neuroblastoma tumours. Mutations may occur randomly or be inherited from parents.

Chromosomal Abnormalities : Alterations in specific chromosomes or segments of DNA may play a role in neuroblastoma development. For example, deletions or duplications of genetic material can disrupt normal cellular processes.

MYCN Amplification : An abnormal increase in copies of the MYCN gene is associated with a more aggressive form of neuroblastoma.

Neuroblastoma Predisposition : Inherited genetic mutations that increase the risk of developing neuroblastoma have been identified in some families. These mutations may increase the susceptibility of nerve cells to tumour formation.

Environmental Factors : While not fully established, specific ecological exposures during pregnancy may contribute to the risk of neuroblastoma. Factors such as maternal exposure to certain chemicals, drugs, or infections may play a role in the development of the disease.

Age : Neuroblastoma is most commonly diagnosed in children under 5, suggesting that developmental processes during early childhood may influence its formation.

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Risk Factors for Neuroblastoma

Age : Neuroblastoma is primarily a childhood cancer, with most cases diagnosed in children under 5, The risk is highest in infants.

Genetic Predisposition : Inherited genetic mutations can increase the risk of neuroblastoma. Some families have a higher incidence of neuroblastoma due to genetic changes affecting nerve cell development and growth.

Family History : A family history of neuroblastoma or other cancers, especially in siblings, parents, or grandparents, may elevate the risk. Specific genetic syndromes, such as familial neuroblastoma or multiple endocrine neoplasia type 2 (MEN2), are associated with an increased risk.

Chromosomal Abnormalities : Certain chromosomal abnormalities or genetic mutations that affect nerve cell growth and differentiation can predispose individuals to neuroblastoma.

MYCN Amplification : An abnormal increase in neuroblastoma

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complications of neuroblastoma

Metastasis : Neuroblastoma can spread (metastasize) to other body parts, such as bones, bone marrow, lymph nodes, liver, and lungs. Metastasis may lead to additional symptoms and affect treatment options.

Tumour Compression : Large or rapidly growing tumours can press against nearby structures, leading to pain, discomfort, or dysfunction. For example, tumours in the abdomen may compress the digestive organs, causing digestive issues.

Obstruction : Tumors located in the abdomen or pelvis may cause obstructions in the gastrointestinal or urinary tracts, leading to difficulties in passing stool or urine.

Respiratory Distress : Tumors in the chest or near the airways can cause breathing difficulties, wheezing, and coughing.

Bone Pain : If neuroblastoma spreads to the bones, it can cause bone pain and increase the risk of fractures.

Anaemia : Tumors may affect the bone marrow's ability to produce healthy red blood cells, leading to anaemia and fatigue.

Malnutrition and Weight Loss : Appetite loss, difficulty eating, and metabolic changes due to cancer can lead to malnutrition and weight loss.

Growth and Development Issues : Neuroblastoma and its treatments can impact a child's growth, development, and overall well-being.

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Diagnosis of Neuroblastoma:

Medical History and Physical Examination : The healthcare provider will gather a detailed medical history, including the child's symptoms, family history, and any relevant factors.

A thorough physical examination may reveal signs such as an abdominal mass or other abnormalities.

Ultrasound : A non-invasive imaging technique that uses sound waves to create images of internal organs. It can help visualize the tumour and assess its characteristics.

CT Scan (Computed Tomography) : Provides detailed cross-sectional images of the body, aiding in detecting and staging the tumour.

MRI (Magnetic Resonance Imaging) : Produces detailed images using magnetic fields and radio waves, assessing the tumour's location and extent.

MIBG Scan (Metaiodobenzylguanidine) : A specialized nuclear medicine scan that uses a radioactive substance to detect neuroblastoma cells.

Biopsy and Pathology:

A tissue biopsy involves removing a small tumour sample for examination under a microscope. This helps confirm the diagnosis and provides information about the tumour's characteristics and aggressiveness.

Pathologists analyze the biopsy sample to determine the type and grade of neuroblastoma.

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Bone Marrow Aspiration and Biopsy:

These tests assess whether the cancer has spread to the bone marrow. A small sample of bone marrow is typically taken from the hip bone for examination.

Laboratory Tests : Urine and Blood Tests: Urine and blood samples may be analyzed for substances like catecholamines and other markers that can indicate the presence of neuroblastoma.

Genetic and Molecular Testing: Genetic tests may be performed to identify specific genetic mutations associated with neuroblastoma.

For reference, here are the general ranges for normal haemoglobin 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

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Treatment of Neuroblastoma:-

Observation (Watchful Waiting):

Some neuroblastomas may not require immediate treatment and can be closely monitored to assess their behaviour over time. This approach is often used for small, low-risk tumours.

Surgery:

Surgical tumour removal is common, especially for localized neuroblastomas. The extent of surgery depends on factors such as tumour size and location.

Chemotherapy:

Chemotherapy involves using powerful medications to destroy cancer cells or shrink tumours. Chemotherapy is often used to treat neuroblastoma and may be administered before or after surgery.

Radiation Therapy:

Radiation therapy uses high-energy beams to target and destroy cancer cells. It may be used after surgery to eliminate remaining cancer cells or as part of the treatment plan for some instances.

Stem Cell Transplantation:

High-dose chemotherapy and radiation therapy can damage bone marrow. Stem cell transplantation (bone marrow or stem cell transplant) may be necessary to replace damaged marrow with healthy stem cells.

Immunotherapy:

Immunotherapy helps the body's immune system target and destroy cancer cells. Treatments like monoclonal antibodies (such as anti-GD2), and cytokines may be used.

Targeted Therapy:

Certain medications target specific genetic mutations or characteristics of the tumour cells. These therapies are designed to disrupt cancer cell growth and survival.

Retinoid Therapy:

Retinoids, a type of vitamin A derivative, are used to help differentiate and mature neuroblastoma cells.

Supportive Care:

As part of the treatment plan, supportive care focuses on managing side effects, alleviating pain, and maintaining the child's overall well-being.

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Neuroblastoma Dos and Don'ts

Neuroblastoma Care at Medicover Hospitals

At Medicover Hospitals, we take pride in our exceptional team of specialized pediatric oncologists and healthcare professionals dedicated to providing comprehensive care for children diagnosed with neuroblastoma. Our experts are well-equipped to address the unique challenges of neuroblastoma and its treatment. Here's what you can expect from our neuroblastoma.

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