Neuroblastoma is an embryonic tumor of the sympathtic nervous system. Each year 25 to 30 children with neuroblastoma are diagnosed in the Netherlands. The clinical symptoms greatly vary from accidentally found localized tumors with excellent prognosis after surgical removal to skin or bone marrow metastasizing spontaneously regressing tumors to rapidly progressing fatal neuroblastomas with bone metastases. The overall survival is 60%. The clinical course depends on the genetic aberrations of the tumor cells. Amplification of N-myc oncogen, loss of chromosome 1p or extra copies of chromosome 17q are associated with agressive disease and dismal outcome. Clinical prognostic factors like age (< 1 year is favorable) and stage (stage 4 is unfavorable) are now used for theraphy assignment, but within clinical risk groups treatment has been differentiated using biological characteristics. This means that children with localized tumors or children with stage 4 neuroblastoma under 1 year of age will get more intensive treatment if their tumor is also N-myc amplified. Prospective multicenter trials are now studying the clinical value of other genetic markers. The outcome of high risk patients with stage 4 neuroblastoma older than 1 year of age has only slightly improved. New treatment modalities for this group are studied like targeted radiotherapy by the neuroblastoma-specific radiopharmacon metaiodobenzylguanidine (MIBG). Better knowledge of biological factors in neuroblastoma development and progression may identify tumor-specific therapeutic targets and better treatment and outcome assessment.
|Tijdschrift||Tijdschrift voor Kindergeneeskunde|
|Nummer van het tijdschrift||5|
|Status||Gepubliceerd - okt. 1999|