Introduction: Neuroblastoma is a tumor of nerve cell origin that usually occurs in the adrenal gland and sympathetic ganglia. It is the most common malignant tumor of infancy and the fourth most common tumor of childhood, with approximately 800 children diagnosed with neuroblastoma each year in the United States.
Neuroblastoma almost always develops before the age of 5. It can occur in infants before birth. Neuroblastoma is very rare in children over the age of 10.
Methods: This tumor usually occurs in early childhood or infancy and may be present at birth. Most cases are sporadic, but the disease may be transmitted as an autosomal dominant trait. Histological features include dense cellularity, small, round polygonal cells, and areas of calcification and necrosis. Abnormal pupillary reflex (leukocoria); pathological nystagmus; strabismus; and vision loss are common clinical features of the disease.
Infants often present with cervical or thoracic masses, and older children with abdominal masses.
Results: In this study, we investigated factors affecting the diagnosis and survival of patients with neuroblastoma.
Initial evaluation may be done with plain radiography or ultrasound, but magnetic resonance imaging (MRI) or computed tomography (CT) is necessary to further evaluate the primary tumor, the extent of local invasion, and metastasis. Also, the levels of urinary metabolites such as VMA and HVA, serum ferritin, and lactate dehydrogenase (LDH) are of clinical importance.
The absence of metastases significantly increases survival rates. For this reason, the median survival is longer in female patients than in male patients and in patients younger than one year of age than in older children. Neuroblastomas may be associated with opsoclonus-myoclonus syndrome.
The collected data indicate that the arrest of neural crest-derived sympathoadrenal lineage differentiation plays a role in the pathogenesis of neuroblastoma. This arrest of cell growth explains many of the biological features of the tumor, including cellular heterogeneity, mutation spectrum, spontaneous regression, and response to differentiation-inducing drugs.
Conclusion: As a result, infants under 18 months have an 88% survival rate, some recover without specific treatment, children 18 months to 12 years have a 49% survival rate, and patients over 12 years have an overall survival rate of less than 12%.