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Neuroblastoma
Submitted by Peter Wang, MD
General Considerations
- Malignant tumor of neural crest cells
- Most common extracranial solid tumor in infancy
- 7.8% childhood cancers
- Most common posterior mediastinal mass in children
Clinical Findings
- Age at presentation
- < 1 year – 40%, good prognosis
- 1-2 years – 35%
- > 2 years – 25%
- Older patients, metastatic disease - poor prognosis
- Lymph nodes, liver, bone
- 50% present with metastatic disease
- Clinical signs
- Mass effect on adjacent organs
- Metastatic disease
- >90% with urine homovanillic acid (HVA) and vanillylmandelic acid (VMA)
- Locations
- Abdominal Cavity (40% adrenal, 25% paraspinal ganglia)
- Thoracic 15%
- Pelvic 5%
- Cervical 3%
- Other 12%
Imaging Findings
- Plain Film
- Abdominal, chest, cervical mass
- Calcification
- Bone erosion (ribs, pedicles)
- US
- Heterogeneously echogenic
- Calcification
- Doppler for flow in encased vessels
- CT
- Encases vessels
- Inhomogeneous (hemorrhage, necrosis, calcifications)
- MRI
- T2WI – bright, heterogeneous
- Variable enhancement
- Neural foramina, spinal canal extension
- Scintigraphy
- Uptake by catecholamine producing tumors
- Primary tumor and metastatic disease
- Tc-99m MDP (Bone Scan)
Differential Diagnosis
- Extramedullary hematopoiesis
- Another neural tumor
- Sequestration
Treatment
- Surgery
- Chemotherapy
- Bone marrow transplantation
Mediastinal Neuroblastoma. On the left is a contrast enhanced coronal reformatted CT image of the chest that shows a large left-sided mass of inhomogeneous enhancement that is displacing the heart to the right. A vessel arises from the aorta to feed the mass (black arrow). On the right, a T1 weighted Gadolinium-enhanced MR image shows inhomogeneous enhancement of the mass which appears to have a component arising from the neural foramen (white arrow).
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For this same photo without the annotations, click here and here
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