Neuroblastoma in 1993

Abstract

Neuroblastoma is the most common extracranial solid tumour of childhood, comprising approximately 10% of these. The annual incidence lies between 6 and 9.6 cases per million children, with minor racial differences. It is unclear whether the reported low incidence in areas of endemic Burkitt's lymphoma is due to a genuine lower number, or to lower rates of reporting. Neuroblastoma makes up 50% of all malignancies in infants, whilst the median incidence is at age 2 years. Tumours appearing after the age of 7 years are uncommon, although initial diagnosis has been made in adults up to the age of 75 years. In contrast to most other tumours of childhood, the majority of patients with neuroblastoma present with widespread disseminated disease. Long term survival for patients over the age of 1 year with disseminated disease has shown only minor improvement in the last 20 years, despite radiotherapy, intensive chemotherapy and surgery (Fig. 1). Neuroblastoma is unusual in that for no other tumour is age at diagnosis such an important predictor of prognosis: infants (< 1 year) in particular may show spontaneous turnout regression, most dramatically seen in the special case of stage 4S disease (see below). Induced maturation too is well recognized both in vitro and in vivo following chemotherapy. There is therefore considerable interest at present in the identification of potential prognostic groups for which cure may be possible with either conventional or high dose intensity chemotherapy, and of those for whom cure cannot yet be expected, for whom radically different strategies may offer an improved prognosis.