The presentation of neuroblastoma (NBL) depends on:
1. The site of the primary tumor:
- Neuroblastoma may originate anywhere along the sympathetic chain
- Majority of primary tumors arise from the abdomen
- Infants have more thoracic and cervical primary tumors compared to children
2. Disease extent (presence of metastatic disease)
3. Presence of paraneoplastic syndromes
* 1% of patients will have no detectable primary tumor.
It is far more common for children with NBL to present with metastatic disease (for example with bone pain) than with symptoms from their primary tumor - unlike Wilms tumor where children often present with abdominal swelling secondary to a large primary tumor mass and rarely present with symptoms from metastatic disease.
Abdominal Tumors:
- 65% of primary tumors
- Most originate from the adrenal gland
- Potential symptoms include:
- Sense of fullness or abdominal pain
- Palpable mass that is:
- Firm
- Irregular
- Likely to cross the midline
Paraspinal Tumors:
- Occur in 5-15% of patients
- Can be associated with cord compression
Cervical Tumors:
- Occur in 5% of patients
- May present with Horner's syndrome
Thoracic tumors:
- May be found coincidentally on CXR
- May cause respiratory symptoms
- Cervical adenopathy may be palpable
- Associated with:
- Heterochromia iridis (depigmented iris on the side of the lesion)
- Horner’s syndrome
- Babies less than 1 year old have a higher proportion of thoracic tumors compared to older children.
