[Neuroblastoma at the Giannina Gaslini Institute. Analysis of 94 cases diagnosed 1972-1981]. / Neuroblastoma all'Istituto Giannina Gaslini. Analisi di 94 casi diagnosticati nel periodo 1972-1981.

The authors have analysed 94 consecutive previously untreated children affected by NB diagnosed at the Giannina Gaslini Children's Hospital in the period January 1972 - December 1981. Age at diagnosis ranged between 1 month - 16 years (median 2 years). Diagnosis was made on histological grounds in 82 cases, on clinical, instrumental and laboratory data in the remaining 12 cases. Evans' staging system was adopted for classifying the disease extent. Patient recruitment was 9.4 cases for year. Female sex slightly prevailed in our series. 17 patients had localized disease (stage I and II), all surviving since 27 - 90 months. 28 children had regional disease (stage III) half of whom are presently alive. Five of the 47 cases with disseminated disease are alive at the time of this study; only one of these 5 can be considered cured, having been followed since more than 4 years. One of the two IV-s stage children survives disease-free at 89 months. 49% of patients were 2-year old or less at diagnosis while only 2 patients were older than 10. Survival was best in children diagnosed under one year of age, lowest in the 2 - 6 year age group. 74% of patients had their primary located in the abdomen (36 in the adrenal, 16 in sympathetic ganglia, 18 in un unidentifable site). Prognosis was worse in these patients compared with those with primary in the thorax, neck and pelvis. The clinical presentation of the disease has been extremely various: most patients had a diagnosis different from the definitive one, and were accordingly treated usually for several weeks or even months. Among laboratory data, urinary catecholamine metabolites, serum LDH and serum ferritin represented the most suitable indices of disease activity. Among immunological studies, none has shown a good correlation with the extent of the disease, nor with the clinical course. Stage I children had only surgery as treatment, while in stage II surgical ablation was followed by short-lasting chemotherapy and radiotherapy in few cases. In stage III and IV more complex multidisciplinary approach was utilized in the attempt to achieve a complete tumor regression. While about a half stage III cases appears curable by these treatment modalities, little impact has been demonstrated by this therapy in widespread disease, although the use of well-designed protocols seems have improved the complete remission rate and the median length of survival.

[Ewing's sarcoma. Results of treatment in 16 consecutive cases]. / Il sarcoma di Ewing. Risultati del trattamento in 16 casi consecutivi.

In the period January 1974-August 1981, 16 previously untreated cases of Ewing's Sarcoma have been diagnosed at the Giannina Gaslini Children's Hospital Genova. Eight were male, eight female. Median age at diagnosis was 11 years. Two patients presented with a unique metastatic lesion, in the right lung and in an illiac lymph node, respectively. Fourteen patients have been initially treated with local radiotherapy (dosages ranging form 4,800 to 6,600 rads) in association with antiblastic polichemotherapy utilizing 4 drugs (Adriamycin, Actinomycin D, Vincristine, Cyclophosphamide). The Rosen et al.'s T-2 protocol was adopted, modifying the initial phase in order to give more weight to Adriamycin and reduce the toxic effects related to radio-chemotherapy combination. Two patients bearing a costal primary were immediately treated with a more complex and aggressive chemotherapy (T-6 Protocol), followed by local irradiation (in one case preceded by surgical ablation) and then chemotherapy again (T-2 protocol, second phase) for 10 months. Treatment determined a fast subjective relief in the 13 symptomatic patients. All 16 cases achieved a status of complete remission. Four of them subsequently relapsed: locally in two, in distant sites in the remaining 2. All 4 died 12-27 months form diagnosis. Twelve patients are presently alive without evidence of disease at 3-92 months (median 37 months) following diagnosis. Treatment has caused early and delayed toxicity in all cases. However, the entity of these complications varied considerably from one patient to an other. Age at diagnosis and site of primary tumor were the factors most relevant in this respect.