ACR Codes: 8.3
Neuroblastoma is a common childhood malignancy, third to leukemia and primary brain neoplasm. Neuroblastomas arise from primitive neuroblasts in the neural crest of sympathetic ganglia. Thus, neuroblastomas can arise anywhere from the cervical region down to the pelvis. 2/3 of neuroblastomas are in the abdomen. Of, these, 2/3 are adrenal origin. Of the remaining 1/3, most are in the chest.
Neuroblastoma is initially clinically silent. It becomes symptomatic once it encroaches on surrounding structures, once it has metastasized (most commonly to bone), or once it has started to secrete catecholamines (10% present with hypertension/sympathetic overstimulation). It is common for kids to present with an incidental palpable abdominal mass.
The majority of neuroblastoma patients are less than 4 years of age with a preponderance between 2 months and 2 years. The tumor is slightly more common in boys than in girls.
Neuroblastoma is unique among malignant pediatric lesions in that it can spontaneously transform into benign ganglioneuroblastoma.
Syndromes associated with neuroblastoma include Beckwith-Wiedemann and Klippel-Feil.
Two paraneoplastic syndromes have known associations: opsoclonus/myoclonus (2%) and WDHK (Watery diarrhea with hypokalemia)(7%).
In general, the overall survival rate is 72% at less than 1 year of age, 28% at less than 2 years of age, and 12% for more than 2 years of age. Staging is: I - confined to organ of origin, II - extension beyond organ of origin with +/- nodes. No midline crossover. III - midline crossover. IV - distant mets. Stages I,II - 75% two-year survival. Stages III,IV - 25% two-year survival.
Imaging characteristics include inhomogeneous echotexture, tumor encasement of vessels, calcification (2/3) - stippled, amorphous, or diffuse.
Reference(s): Kirks, DR, editor: Practical pediatric imaging, ed 3, Philadelphia 1997, Lippencott-Raven, pp 1009-1171.