What is Neuroblastoma?
Neuroblastoma is a type of cancer that arises from immature nerve cells and primarily affects children. It most commonly starts in the
adrenal glands located above the kidneys but can also develop in the neck, chest, abdomen, or spine. Neuroblastoma is the most common cancer in infants and accounts for about 6% of all childhood cancers.
What Causes Neuroblastoma?
The exact
cause of neuroblastoma is not well understood. It is believed to occur due to genetic mutations in the developing sympathetic nervous system. While most cases are sporadic, around 1-2% are familial, involving inherited genetic mutations such as those in the
ALK gene (anaplastic lymphoma kinase).
Abdominal pain or swelling
Fatigue and weakness
Bone pain or limping
Fever
Weight loss
High blood pressure
Dark circles around the eyes
In more advanced cases, neuroblastoma may cause
neurological symptoms such as difficulty walking, changes in bladder or bowel function, and even paralysis.
How is Neuroblastoma Diagnosed?
Diagnosis typically involves a combination of imaging tests such as ultrasound, CT scan, MRI, and MIBG scan, which is a nuclear medicine scan specific for neuroblastoma cells. A definitive diagnosis is usually made through a
biopsy of the tumor. Additionally, blood and urine tests can detect elevated levels of catecholamines, which are often produced by neuroblastoma cells.
How is Neuroblastoma Staged?
Staging of neuroblastoma is crucial for determining the treatment plan and prognosis. The International Neuroblastoma Staging System (INSS) and the International Neuroblastoma Risk Group Staging System (INRGSS) are commonly used. The stages range from:
Stage 1: Localized tumor with complete gross resection
Stage 2: Localized tumor with incomplete resection
Stage 3: Unresectable unilateral tumor
Stage 4: Disseminated disease to distant lymph nodes, bone marrow, liver, skin, or other organs
Stage 4S: A special subset for children under 12 months with localized primary tumor and limited metastasis
Surgery: To remove the tumor, which is often the first step in treatment.
Chemotherapy: Used to shrink the tumor before surgery or to treat metastatic disease.
Radiation therapy: Employed in cases where surgery and chemotherapy are not sufficient.
Stem cell transplant: For high-risk neuroblastoma, high-dose chemotherapy followed by a stem cell transplant may be recommended.
Immunotherapy: Antibody-based therapies like Dinutuximab, which target neuroblastoma cells, are used for high-risk cases.
What is the Prognosis for Neuroblastoma?
The
prognosis for neuroblastoma varies based on several factors including the age of the child, stage of the disease, and biological characteristics of the tumor. Children diagnosed before the age of 18 months generally have a better prognosis. Low-risk neuroblastoma has a survival rate of over 90%, while high-risk neuroblastoma has a lower survival rate, often around 40-50% despite aggressive treatment.
Are There any Long-term Effects of Treatment?
Children who survive neuroblastoma may experience
long-term effects from the treatment, including hearing loss, growth and developmental delays, learning disabilities, and secondary cancers. Regular follow-up care is essential to monitor and manage these potential late effects.
Conclusion
Neuroblastoma is a complex pediatric cancer with a wide range of clinical behaviors. Early diagnosis and advances in treatment have improved outcomes, especially for low and intermediate-risk cases. Ongoing research is essential to develop more effective treatments for high-risk neuroblastoma and to minimize long-term side effects for survivors.
