[Neuroblastoma].

Abstract

Neuroblastoma (NB) is a common tumor of childhood, presenting "unique" characteristics: i.e., different prognosis in relation to age, high rate of metastases at diagnosis, capacity of spontaneous regression, strong immunogenicity. The embryologic derivation of NB has been recently clarified: NB derives from the embrional sympathetic nervous tissue; its enzymatic activity is determined mainly by environmental factors. A number of clinical and laboratory aspects influence the fate of children with NB: extention of disease and age are the most important, followed by site of primary, histology, pattern of metastatic spread, immunologic characteristics. Among laboratory tests, many are correlated with the clinical course: urinary excretion of sympathetic amines, serum levels of ferritin, C3 complement fraction, LDH, IgM, neurono-specific enolase. In the recent years the development of monoclonal antibodies techniques has greatly improved. In NB, a number of membrane molecule determinants have been discovered, against which specific monoclonal antibodies can be profitably directed for diagnostic and therapeutic purposes. NB cells grow in vitro in the soft agar system; in this assay resistance and sensitivity of tumor cells can be tested with sufficient accuracy and may predict drug effect in vivo. Therapy of disseminated neuroblastoma is unsatisfactory till now. Promising techniques include autologous or allogeneic bone marrow infusion following supralethal chemotherapy, and administration of substances, such as retnoids, able to promote neuroblastoma cells differentiation in vivo.