Neuroblastoma is a rare and aggressive type of cancer that develops in immature nerve cells called neuroblasts. It primarily affects infants and young children, usually forming in the adrenal glands, which sit atop the kidneys. However, it can also arise in nerve tissues along the spine, chest, abdomen, or pelvis. This cancer is known for its unpredictable behavior, ranging from spontaneous regression to rapid progression and metastasis.
Neuroblastoma originates from neural crest cells, which play a crucial role in the development of the nervous system and adrenal glands. In some cases, these immature nerve cells fail to mature properly and begin multiplying uncontrollably, forming a tumor.
Primarily affects children – Most cases occur in children under five years old.
Can arise in different locations – Typically starts in the adrenal glands but can also appear along the spinal cord.
Variable progression – Some cases regress spontaneously, while others grow aggressively and spread.
Genetic factors – Certain gene mutations and familial inheritance may play a role in its development.
The exact cause of neuroblastoma is not well understood, but several factors may increase the risk.
Genetic mutations – Changes in the ALK and PHOX2B genes have been linked to neuroblastoma.
Family history – A small percentage of cases are inherited.
Congenital abnormalities – Certain birth defects may indicate a predisposition.
Maternal exposure – Possible links to environmental factors during pregnancy.
The symptoms of neuroblastoma vary depending on the tumor’s location and whether it has spread.
Abdominal swelling – Often due to tumor growth in the adrenal glands.
Pain – Caused by tumors pressing on nerves or organs.
Lumps under the skin – Indicate metastasis to lymph nodes.
Unexplained weight loss – A common sign of advanced cancer.
Fatigue and weakness – Due to the body’s inability to fight the cancer.
Horner’s syndrome – Drooping eyelid, smaller pupil, and reduced sweating on one side of the face if the tumor affects the sympathetic nervous system.
Blue or bruised-looking skin – Occurs when cancer spreads to the skin.
Bone pain – Suggests metastasis to the bone marrow.
Neuroblastoma is classified based on its biological characteristics and how aggressive it is.
Low-Risk Neuroblastoma
Often occurs in infants.
Can regress spontaneously without treatment.
Has a high survival rate.
Intermediate-Risk Neuroblastoma
Requires treatment but has a good prognosis.
May involve localized spread.
High-Risk Neuroblastoma
Highly aggressive and likely to spread.
Requires intensive treatment.
Has a lower survival rate.
Diagnosing neuroblastoma involves multiple tests to confirm the presence and spread of the disease.
Physical exam – Checking for lumps, swelling, and neurological signs.
Ultrasound – Initial imaging test for detecting abdominal masses.
MRI/CT scan – Provides detailed images of the tumor.
MIBG scan – Uses a radioactive tracer that highlights neuroblastoma cells.
Biopsy – Examination of tumor tissue under a microscope.
Bone marrow aspiration – Checks for cancer cells in the bone marrow.
Urine tests – Detects catecholamines, which are produced by neuroblastoma cells.
Treatment depends on the stage and risk level of the cancer.
Tumor removal – Used in localized cases where the tumor can be safely extracted.
Debulking – Removing as much tumor as possible before further treatment.
Used for intermediate and high-risk cases.
Kills cancer cells – Helps shrink tumors before surgery.
Common drugs – Cisplatin, vincristine, and doxorubicin.
High-energy rays target and destroy cancer cells.
Used after surgery to prevent recurrence.
Involves MIBG radiotherapy, which specifically targets neuroblastoma cells.
Boosts the immune system to recognize and fight cancer cells.
Antibody therapy (GD2 inhibitors) – Helps the immune system attack neuroblastoma cells.
For high-risk cases after chemotherapy.
Replaces damaged bone marrow with healthy stem cells.
Improves recovery and long-term survival.
Focuses on specific molecules involved in cancer growth.
ALCL inhibitors – Target genetic mutations.
Survival rates depend on several factors, including the stage, age at diagnosis, and genetic features.
Age at diagnosis – Younger children tend to have better outcomes.
Tumor biology – Certain genetic markers indicate a more aggressive cancer.
Extent of spread – Localized tumors have a higher survival rate.
Response to treatment – Tumors that respond well to initial therapy have better outcomes.
Low-risk neuroblastoma – Over 90% survival rate.
Intermediate-risk neuroblastoma – 70-90% survival rate.
High-risk neuroblastoma – Less than 50% survival rate.
Since the exact cause of neuroblastoma is unclear, prevention strategies are limited.
Genetic screening – Recommended for families with a history of neuroblastoma.
Avoiding toxic exposure – Limiting exposure to harmful chemicals may help.
Healthy pregnancy practices – Proper prenatal care can support fetal development.
Early detection – Regular pediatric check-ups can aid in early diagnosis.
Managing neuroblastoma requires long-term follow-up and lifestyle changes.
Regular medical check-ups – Essential for monitoring recurrence.
Nutritional support – A healthy diet supports recovery.
Pain management – Medications and therapies help alleviate discomfort.
Emotional support – Counseling and support groups provide psychological help.
Physical activity – Light exercise improves well-being and immune function.