Neuroblastoma : Epidemiology

Neuroblastoma is the most common extra-cranial solid tumor in childhood. It makes up almost 7% of all cancers in children under 15 years of age in the U.S. Almost 90% of patients are younger than 5 years at diagnosis and 98% are diagnosed by the age of 10 years. The median age at diagnosis is 19 months. Rare cases have been documented in adults. There are some well-established associations, including Beckwith-Wiedemann syndrome, Hirschsprung disease, Neurofibromatosis, and as a complication of Fetal Hydantoin syndrome. About 1% to 2% of neuroblastomas are familial. Familial cases appear to have an autosomal dominant pattern of inheritance and are diagnosed much earlier than sporadic cases at a median age of 9 months. Bilaterality and multifocality is more common in familial cases. The image shows a well-circumscribed tumor with deeply hemorrhagic red color punctuated by gray-white areas. Such tumors are usually undifferentiated or poorly-differentiated on histopathologic examination. Note the thin stretched out bright yellow rim of adrenal cortex on the left and the right edges of the tumor. Image courtesy of Dr. Jean-Christophe Fournet, Paris, France; humpath.com; Used with permission