Diagnosis and treatment of renal cell carcinoma in children: a report from the Polish pediatric rare tumor study group.

BACKGROUND: Renal cell carcinoma (RCC) in children is rare, accounting for approximately 1.9-6% of all pediatric renal malignancies. The aim of this study was to transmit our experience in the treatment of RCC in Polish children. METHODS: Clinical data from 21 children (6.3-18 years old) with RCC treated between 1992 and 2009 at Polish pediatric oncological centers were analyzed. RESULTS: In 2 patients, RCC developed as a second malignancy after neuroblastoma or astrocytoma fibrillare, respectively. In 6 patients, initial diagnoses based on imaging studies were unilateral Wilms' tumor, leading to preoperative chemotherapy. The remaining patients underwent surgery at the beginning of treatment. According to the AJCC/TNM staging system, 14 patients had stage I, 5-II, 1-III, and 1-IV. Nephrectomy was performed in 19 patients, heminephrectomy in one, and biopsy in another. Histopathological diagnoses were clear-cell RCC (18 patients), papillary RCC (2 patients), and chromophobe RCC (1 patient). 10 patients were treated with chemotherapy, with or without IL-2, INFα, and antiangiogenic agents. 2 patients died due to disease progression. CONCLUSIONS: RCC in children is mostly operable at diagnosis, resulting in good prognosis. The role of adjuvant chemo- and immunotherapies is unclear. Neoadjuvant chemotherapy proven for children with Wilms' tumors is ineffective, but the delay in adequate therapy did not worsen the outcome if complete nephrectomy is done subsequently.

In vitro sensitivity of leukemic cells to nucleoside derivatives in childhood acute leukemias: good activity in leukemic relapses.

Nucleoside analogues such as fludarabine and cladribine are used in therapy of indolent lymphomas and leukemias in adults, while cytarabine is used mainly in protocols for acute leukemias. Mechanisms of their activity is based on inhibition of enzymes involved in DNA, RNA and protein synthesis. The objective of the study was the analysis of in vitro cellular drug sensitivity in childhood acute lymphoblastic (ALL) and myeloid (AML) leukemia. Isolated leukemic cells obtained from 264 patients, including 152 initial ALL, 45 relapsed ALL, 54 initial AML and 13 relapsed AML were tested for cytotoxicity for fludarabine, cladribine, and cytarabine by the MTT assay. Drug concentration lethal to 50% of tested cells was regarded as a value of drug resistance. Three tested nucleoside analogues showed highest cytotoxicity against initial ALL samples. Samples of relapsed ALL and initial AML were more resistant than ALL de novo ones. Unexpectedly, no differences were observed between initial and relapsed AML samples for all tested drugs, what suggests that nucleoside analogues are active drugs in relapsed AML, which is commonly regarded as a resistant disease. All tested drugs presented significant cross-resistance in each of analyzed subgroups. In summary, tested nucleoside analogues presented relatively good activity against childhood leukemias at relapse stage.

[Investigations of thyroid function in children and adolescents after treatment of Hodgkin's disease].

Radiotherapy focused on the neck, upper mediastinum or cranium can raise the risk of thyroid dysfunction. The influence of radio- and chemotherapy on thyroid morphology and function after treatment of Hodgkin's disease in 31 children and acute lymphoblastic leucaemia in 36 children was evaluated. Clinical examination, estimation of blood TSH, FT4 levels as well as ultrasonographic scan were performed. In the group of children with Hodgkin s disease the blood level of ATG Antithyreoglobulins andMAB, microsmal antibodies were estimated. The results of clinical examination were normal in all patients. Thyroid function tests were also normal, except for one case with elevated TSH values (22.76 microIU/ml) and decreased FT4 values (0.65 ng/ml). Three patients (9.7%) with Hodgkin s disease and three (8.5%) with ALL (only one had cranial radiotherapy) had an abnormal image of thyroid in ultrasonography (hypoechogenicity, heterogenous ultrasound scan, solid nodule). The results of our investigations indicate that abnormalities found in the ultrasound scan in children and adolescents with Hodgkin's disease in whom treatment has been terminated, can be a first prodrome of thyroid pathology.

Results of IDA-FLAG programme in the treatment of recurrent acute myeloblastic leukaemia--preliminary report.

BACKGROUND: Unsatisfactory treatment results of acute myeloblastic leukaemia inspire the search for new drugs, characterised by higher efficiency and lower toxicity. The aim of the study was the assessment of the efficiency and side effects associated with the implementation of IDA-FLAG protocol. MATERIAL AND METHODS: The assessment of treatment results and undesirable effects was based on the material of 4 children with the relapse of acute myeloblastic leukaemia, after a total of 6 IDA-FLAG protocols. RESULTS: Complete remission was obtained in 2 (50%) children, with mean remission time of 13.5 months. One of the children underwent the transplantation of haematopoietic cells from unrelated donor. The main side effect observed was bone marrow aplasia. Leucopenia, granulocytopenia, thrombocytopenia and anaemia persisted for approx. 20 days. Apart from haematological symptoms, the following were also observed: the symptoms of intolerance after ARA-C, aspergillosis of paranasal sinuses and lungs and severe coagulation disorders. CONCLUSION: IDA-FLAG protocol may be recommended for use in children with AML relapse and the undesirable effects observed are acceptable.

[Renal function in children after treatment of acute lymphoblastic leukaemia (ALL)]. / Ocena czynnosci nerek po zakonczeniu leczenia ostrej bialaczki limfoblastycznej u dzieci.

UNLABELLED: The aim of the study was to evaluate the renal function (on the basis of serum: cystatin C, creatinine, beta2-microglobulin(B2M) levels, creatinine and cystatin C clearance, and urine osmolality) in 37 children with ALL (acute lymphoblastic leukaemia) after completing intensive treatment (9 in course of maintenance therapy), depending on nephrotoxic antibiotics use ( 3 times) and methotrexate dose (1.0 g/m2 vs 5g/m2). The control group was formed by 15 healthy children. RESULTS: 1) Serum cystatin C, creatinine, beta2-microglobulin levels creatinine and cystatin C clearance, and urine osmolality were similar in the analysed and control groups. 2) We did not find any differences between the analyzed parameters in the groups of children: a) in maintenance therapy and after the end of the therapy, b) received methotrexate in the dose of 5.0 g/m2 vs 1.0 g/m2, c) not treated children and treated