The rare problem of late recurrence in neuroblastoma

Two-year disease-free survival is considered highly predictive of therapeutic success with neuroblastoma and death from neuroblastoma greater than 5 years after diagnosis is a rare event. We report of an unusual case of death from neuroblastoma 20 years after diagnosis; a literature review and discussion of this rare phenomenon of late recurrence is also included. Five additional cases of late recurrence, defined as greater than 5 years after diagnosis, were found in the literature with adequate detail to be reviewed. Advanced disease was present at diagnosis in five of six cases; three of six had disseminated disease. No age or sex differences were noted. A peculiar tendency for recurrence during the teenage years, preceded by a long disease-free interval, was also present in all cases, perhaps a reflection of maturational alterations in the immune system or hormonal changes that previously had suppressed extensive disease. This phenomenon of late recurrence in neuroblastoma, albeit rare, adds another facet to the clinical behavior of an enigmatic and therapeutically unyielding childhood tumor.