[Results of treatment in osteosarcoma--chemotherapy with ADM and CDDP]. / Wyniki leczenia pacjentów z miesakiem kosciopochodnym z zastosowaniem chemioterapii z ADM i CDDP.

Malignant bone neoplasms contribute to about 7% of paediatric cancer. Within the last 20 years much has changed in cancer treatment. Neoadjuvant chemotherapy, as the first phase of comprehensive treatment, results in regression of the tumour and makes limb salvage surgery possible. An exact analysis of 36 patients with osteosarcoma of different localisation, treated at the National Research Institute of Mother and Child between 1991 and 1996 was carried out. Treatment was started with pre-operative adjuvant chemotherapy with ADM and CDDP, administrated during 6 weeks. The regimen and the length of administration depended on stage of disease and tumour reaction to chemotherapy. Amputations or limb salvage surgery was conducted as a second phase of therapy. Postoperative chemotherapy was given for 6 months. Tumour reaction to chemotherapy was described according to the Huvos scale - percentage map of necrosis and regression areas in the neoplastic tissue. The analysis shows good results after chemotherapy with ADM and CDDP.

[Histopathologic differentiation of tumors derived from neuroblastic cells in children depending on age]. / Zróznicowanie histologiczne guzów grupy neuroblastoma u dzieci w zaleznosci od wieku.

The histopathology of 224 tumours of neuroblastic origin was analysed in relation to the age of patients. Maturing neuroblastomas (NBS) dominated in infants, where as in the most numerous group of children 1-5 years of age, prevalence of neuroblastomas without any signs of maturation was noted. In children over 5 years of age a high incidence of tumours with evident maturation was noted (ganglioneuroblastomas and ganglioneuromas). The prevalence of maturing neuroblastomas in infants correlate well with an advantageous clinical course of neuroblastomas at this age.

Malignant humeral bone tumors in children: excision and reconstruction with the use of rotated clavicle.

The application of intensive multimodality therapy has made possible salvage surgery in bone tumors. Reconstruction of the removed part of bone is the great problem, especially in fast-growing children. In three patients (two osteosarcomas and one Ewing's sarcoma), the tumor was confined to the proximal half part of humerus, without invasion of shoulder joint. After induction chemotherapy, reduction of tumor size was observed both clinically and radiologically. During the operation, wide resection of the tumor together with a 12- to 14-cm-long fragment of humerus, was performed. Afterward, the clavicle was rotated in the place of the removed bone, with preservation of the coracoacromial ligament. The humeral stump and clavicle were fixed with the use of metal plate. Adjuvant chemotherapy was used a few days following surgery. After 3 months, the osteosynthesis had healed. The movements in shoulder joint are limited, but functions of elbow joint remained normal. All children are alive and disease free. Reconstruction of humerus with clavicle rotation is possible when the proximal bone loss is not longer than 10-14 cm. This method seems to be an alternative to allogeneic grafts and endoprostheses.

Antioxidant and glutathione-associated enzymes in Wilms' tumour after chemotherapy.

The present study demonstrates the activities of antioxidant and glutathione-associated enzymes and the level of glutathione in Wilms' tumour (nephroblastoma) samples after chemotherapy (mainly actinomycin D and vincristine). We observed higher activity of superoxide dismutase in Wilms' tumour compared to adjacent morphologically unchanged kidney. On the other hand, in this tumour lower activities of catalase and the glutathione-associated enzymes glutathione synthetase, gamma-glutamyl transpeptidase, glutathione reductase and total glutathione S-transferases (GST) were found. Using isoelectric focusing we separated different forms of GST in tested tissues and revealed lower activities of the basic enzymes in Wilms' tumour, which may be responsible for the decrease of total GST activity. Moreover, we found the acidic isoenzymes to be the predominant class of GST in nephroblastoma. In Wilms' tumours with unfavourable histology a high activity of these isoenzymes together with a high level of GSH were observed. We suggest that these parameters may participate in the known phenomenon of anticancer drug resistance of tumours with unfavourable histology.

[Prognostic factors in neuroblastoma in children]. / Czynniki rokownicze w neuroblastoma u dzieci.

The paper presents the review of prognostic factors which were investigated from the first interests of neuroblastoma, as a model for cancer biology in pediatric oncology. The authors on the basis of other authors experiences and on the own observations, as well stress the importance of basic sciences from the field of molecular biology, cytogenetics and biochemistry, leading to better understanding of the nature of neuroblastoma and to introduction of rational treatment. The stratification of neuroblastoma patients according to the known prognostic variables influenced favourably treatment results.

Urinary dopamine/noradrenaline and dopamine/vanillylmandelic acid ratios as a reflection of different biology of adrenergic clones in children's neuroblastic tumors.

The results of calculations of urinary dopamine/noradrenaline (DA/NAd) and dopamine/vanillylmandelic acid (DA/VMA) ratios in 54 untreated children with neuroblastic tumors are reported. Thirteen patients were in the prognostically favorable group (stages I, II, and IV-S and ganglioneuroma [GN]), and 41 had advanced neuroblastoma (stage III and IV). Among patients with ganglioneuroma and favorable neuroblastoma (n = 13), of whom all were survivors, the urinary DA/NAd and DA/VMA ratios exceeded 1.8 in only 2 cases of stage IV-S and stage I, respectively. In the advanced neuroblastoma group, the DA/NAd and DA/VMA ratios exhibited a wide range of values, but among the stage III and IV survivors (n = 10), DA/NAd ratios greater than 1.8 were noted in only 3 patients. The DA/VMA ratio was not greater than 1.8 in those 3 patients. The mean DA/NAd and DA/VMA proportions in the population comprising all survivors were 1.8 +/- 2.7 (mean +/- SD) and 1.1 +/- 0.4, respectively. The same computations carried out in patients who died showed higher values, ie, the mean DA/NAd and DA/VMA ratios were 5.2 +/- 6.3 and 5.6 +/- 10.5, respectively, showing the difference in DA/NAd and DA/VMA ratios between prognostically favorable and unfavorable groups. Of 23 survivors, only 4 had DA/NAd ratios greater than 1.8 (17%), while 24 of 31 children who died (77%) had DA/NAd ratios was greater than 1.8. The reported results suggest dissimilarity in the catecholamine metabolism of adrenergic clones with respect to the stage of advancement of neoplastic disease.