Neuroblastoma, a malignant
tumor of infancy and childhood, has some very interesting peculiars: good prognosis, even with disseminated disease, propensity to occasionally undergo
spontaneous regression, its ability to undergo spontaneous or induced differentiation to a benign
ganglioneuroma.
Neuroblastoma may originate anywhere along the sympathetic nervous system chain. The most common site of primary
tumor is, however, within the abdomen either in the adrenal gland or in a paraspinal
ganglions. A great deal of progress has been made in advancing the knowledge of human
neuroblastoma at the cellular and molecular viewpoint. The
genetic predisposition to develop the
tumor is clarified, a specific oncogene amplified (N-myc) in
neuroblastoma cells shows precise prognostic significance and the deletion of chromosome 1's short arm has been defined. Work-up in
neuroblastoma's diagnosis include the urine assay for
catecholamine metabolites (VMA,
HAVA, VLA) and serum assay for the specific markers as
neuron-specific enolase (NSE),
ferritin,
GD2 ganglioside. Imaging include CT-scan, MIGB body-scan and the newest
monoclonal antibodies scan. Abdominal
tumors are shown in about 75% of children > 12 months old. In 2/3 of cases,
tumor is widely disseminated at the time of diagnosis. In the period 1979-94 the Italian Group for
Neuroblastoma (GCN-AIEOP) collected 1083 cases of
tumors and 5-yrs survival was 45% +/- 2.4 for the patients studied in the period 1979-84, which is increased to 58% +/- 3 for the group of patients 1990-94 (p < 0.001). The overall survival was 53 +/- 1.7. About 5-yrs survival at different stages, AIEOP shows that it is increased from 88% +/- 3.3 (1979-84 group) to 91% +/- 2.8 (1985-92) in the stage I and II (280 cases). In the stage IV survival value improved from 79% +/- 7.1 to 84% +/- 7 (132 cases). No statistical improvement can be observed, anyway. Better improvements can be pointed out in stage III (221 cases, survival from 48% +/- 5.2 (79-84 group) to 69% +/- 4.8 (85-92) and stage IV (483 cases, survival from 16% +/- 2.6 to 28% +/- 3.4) (p < 0.001). Finally we can summarize about
neuroblastoma: 1) better prognosis in the first year of life; 2) ability to
spontaneous regression, first of all, in stage IVs; 3) partial and provisional response to
therapy in advanced stages; 4) no recovery increasing despite advancing in surgery and
chemotherapy.