Treatment of neuroblastoma in patients with neurocristopathy syndromes

Neuroblastoma, a neoplasm of neural crest cell origin, occasionally presents in association with other neural crest abnormalities such as Hirschsprung disease, congenital central hypoventilation, autonomic disturbances, and other tumors. These associations have been termed "neurocristopathy syndromes." In the past, chemotherapy has not been considered for patients with neurocristopathy-associated neuroblastoma because of their complicated medical problems. The authors describe two patients with neurocristopathy syndromes from our institution who underwent surgery and standard chemotherapy treatment of their intermediate-risk neuroblastoma. They represent two of only three long-term disease-free survivors of neurocristopathy-associated neuroblastoma reported in the literature.