It arises from the sympathetic nervous system and can arise in the adrenal gland and anywhere from neck to the pelvic cavity. It is commonly diagnosed at around 20 months of age and mostly occurs below 5 years of age. This tumor has a variable course ranging from tumor not requiring any treatment to one requiring all modalities but still not a good outcome.
Why does it occur?
Some environmental factors have been thought to be responsible for it is but not significantly seen as associated like prenatal smoking etc. About 1-2 % percent of a tumor are familial and they can have multifocal disease and present early in life.
The symptoms depend on what site the tumor arises-
Neck – palpable swelling on the side of the neck
Thorax – Respiratory symptoms/dysphagia, Superior vena cava syndrome
Abdomen- pain, distension, hard mass palpable
Pelvis- Urinary dysfunction, Constipation or fecal incontinence
Paraspinal tumor- Back pain (radicular), Scoliosis
Spinal cord compression/paraplegia
- Hypertension (renin mediated)
- Weight loss, anorexia, fever
- Bone pain due to bone metastasis
- Periorbital ecchymosis (raccoon eyes/panda eyes)
- Opsomyoclonus syndrome (OMAS)- dancing eyes and jerky (involuntary) movement
- VIP syndrome (Kerner-Morrison syndrome)- intractable diarrhea
How do we diagnose
- Laboratory tests
- Routine blood investigations
- Urinary VMA levels – 24hours/spot
- CECT/MRI depending on local site
- MIBG/PET-CT for metastatic workup
- Bilateral bone marrow aspiration and biopsy
- Tissue diagnosis- image-guided biopsy from the tumor or if a non-metastatic small tumor can do upfront surgery. It would include histopathology, IHC, N-Myc amplification, and chromosomal aberrations.
Like most of the pediatric tumors, neuroblastoma is staged and risk-stratified before starting the treatment. This involves age, local stage, metastatic disease, type of tumor, chromosomal aberration and N-Myc amplification. The risk stratification helps to define treatment and outcome.
A low-risk disease might only need observation or only surgery depending on the location and presence or absence of the life-threatening condition. The intermediate risk group the chemotherapy is tailored according to the good or bad biology tumor and surgery. The high-risk neuroblastoma requires intensive chemotherapy followed by local treatment (surgery and radiation) with or without bone marrow transplant.
The survival in low, intermediate and high-risk neuroblastoma is between 85-100%, 80-90% to about 20-30% respectively.