[Recent advances on retroperitoneal neuroblastoma].
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.
De Grazia
E
E
Cattedra e Divisione di Chirurgia Pediatrica, Università di Palermo.
Cimador
M
M
De Bernardi
B
B
D002648:Child; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D009447:Neuroblastoma / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics / Q000601:surgery