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Unlabelled: In order to assess if any differences exist in children germ cell tumours depending on age, we compared some features of germ cell tumours in two age groups:younger than 10 and between 11 and 18 years., Material and Methods: Data of 146 patients with germ cell tumours treated in 15 Polish paediatric oncology departments between 1995 and 2005 were evaluated. They were divided into two groups: 76 children 0-10 years old (group I) and 70 patients 11-18 years old (group II). Tumour morphology, sex of patients, primary tumour and metastases localization, disease stage, biochemical markers, treatment response, disease relapse and long survival were analyzed. Every patient was treated according to the TGM 95 protocol., Results: In group 1, 67 tumours were assessed histologically. 64%t tumours had homogenous structure with yolk sac tumour in predominance and 36% were mixed. Yolk sac tumour (YST) or teratoma as components of mixed tumours were the most commonly found. In older group 64 tumours were examined, 41% were homogenous, and seminoma/dysgerminoma predominated. In 59% mixed tumours the most common components were YST embryonal carcinoma and teratoma. The most common primary site in group I was the sacrococcygeal region while in group II - the gonads. Disseminated disease was recognized mostly in older children. Among two evaluated serum markers, AFP was increased mostly in younger patients (76% vs 44%), and 3HCG in older group (40% vs 9%). Treatment response was comparable in both groups. Two relapses were observed in each group. Poor outcome was noted in 17/140 analyzed patients: 9 (12%) in group I and 8 (11%) in group II. In 12 of patients with poor outcome the cause of death was progression and in 5 of them - treatment complications., Conclusions: 1. Germ cell tumours in younger and older children differ in histology, primary localization and serum level of biochemical markers. 2. In older patients germ cell tumours are recognized more frequently in advanced clinical stages. 3. Treatment response was comparable in both groups. 4. There is a need to analyze the intensity of chemotherapy to precise the adequate risk groups according to primary treatment response.

Oesophageal atresia is a congenital defect of alimentary tract concerning the interruption of oesophagus with or without connection with the trachea. Its incidence is 1:3000-3500 of live-born. Associated anomalies including genetic disorders occur in 50% of patients. Edwards syndrome which is trisomy of chromosome 18 with poor prognosis. The incidence of Edwards syndrome is 1:5000 of live-born. About 5% of these children live more than 1 year. The aim of this article is a retrospective analysis of the course of treatment of newborn with oesophageal atresia and Edwards syndrome and making of therapeutic decision. The authors from different medical specializations: clinical genetics, paediatric surgery, paediatrics and neonatology, paediatric intensive care and palliative medicine, have undertaken a discussion regarding surgical treatment of children with oesophageal atresia and chromosomal, lethal syndrome.

Introduction: Bone marrow transplantation from HLA identical family donors is the treatment of choice for children with severe aplastic anaemia (SAA). When there is no donor available, combined immunosuppressive therapy is given., Aim: evaluation of results of immunosupressive therapy in children with severe aplastic anaemia., Material and Methods: SAA was diagnosed in 105 children (42 girls, 73 boys), aged 2-18 years, in the eleven haematological centres in Poland, between 1993-2007. All patients received the Severe Aplastic Anaemia Working Party of the EBMT protocol which included: antilymphocyte globulin or antithymocyte globulin, cyclosporin A, prednisolone. Granulocyto- or granulocytomacrophagic-cell stimulation factor was additionally administered during deep neutropenia. Haematological response was evaluated on day 84 or 112 and 180 of the therapy., Results: complete remission occurred in 53 patients (51.5%), partial remission in 27 (24.7%), no response was obtained in 25 children (23.8%) on day 180, of the therapy. Period of observation was from 12 months to 12.5 years. During this time relapse occurred in 10 patients (9.5%). We observed 22 deaths: 8 early, during the first 3 months of IS and 14 after the first 3 months of immunosuppresive therapy (IS). At present 70 children (66.6%) are in first remission with lasts from 12 months to 12.5 years. The survival at 12.5-years is 78.6%. During the 12.5 years of follow-up we had two cases with a late clonal complication (PNH and MDS). Transformation to acute nonlymphoblastic leukaemia was observed in two of our patients., Conclusions: 1. Immunosuppresive therapy (IS) in children with SAA, without bone marrow family donors, is more effective after introduction of combined IS (12.5 years survival in this study was 80% for children with very severe aplastic anaemia (v SAA). 2. In our studies among the children followed up after IS therapy, there were: 1 case of periodic nocturnal haemoglobinuria (PNH), 1 case of myelodysplastic syndrome (MDS) and 2 cases of myeloid leukaemia (probability of incidence was 3.8%).

Aims: The aim of the study was the analysis of risk factors of therapeutic failures in children with malignant germ cell tumours treated within the multicentre programme of PPGGL from 1999--2006., Materials and Methods: The investigated group included 18 (14.3%) patients, of 123 who have finished the treatment of malignant germ cell tumour, in whom no remission was obtained or relapse occurred. All the patients were treated according to the TGM 95 programme. Both clinical and morphological data of the group have been analysed., Results: Among 18 patients with therapeutic failures 12 died. Two patients from the high risk group died of complications of the treatment--sepsis during neutropenia after chemotherapy and one after haemorrhage to the central nervous system. The other 9 died from progression of malignancy, 6 of them belonged to the high risk group. 10 (82%) of 12 patients who died had extragonadal location and in 11 (92%) the tumour was in stage III or IV of the disease. The most frequent histology in this group was mixed germ cell tumour with component of yolk sac tumour or carcinoma embrionale. 92% patients had elevated AFP, in 4 it was above 15000 ng/ml. In 11 (92%) patients primary chemoresistance was observed, and radical surgery was not possible for the reason of advanced stage of the disease. In 6 patients relapse occurred. In 3 patients testis was the primary location (I and II stage), in 3 patients the tumour was localized in the sacrococcygeal region (III and IV stage). All the patients are alive in remission after second line therapy, with 78 months (median) of follow-up., Conclusions: 1. The main risk factor for therapeutic failures in malignant germ cell tumours was primary chemoresistance in inoperable tumours of the sacrococcygeal region. 2. The mortality of treatment complications was low. 3. The relapse of cancer was not a risk factor for therapeutic failure due to the high probability of second remission 4. Therapeutic failures are mainly observed in patients with mixed germ cell tumour with components of yolk sac tumour or carcinoma embrionale. 5. Tumour chemoresistance should be considered an essential factor in identifying high risk patients.

Aim of the Study: Measurement of c-myb expression in leukaemia cells in children and in normal cells of healthy controls., Material and Methods: 37 patients, 23 boys and 14 girls with acute leukaemia, aged 1-17 years, were included in the study (32 with acute lymphoblastic leukaemia and 5 with acute myeloblasts leukaemia) Control group consisted of 17 healthy children, 8 boys and 9 girls, 4-18 years old. After the isolation of mononuclear cells from bone marrow I peripheral blood mRNA was isolated, then with the use of reverse transcriptase cDNA was synthesized. The level of expression of c-myb was analyzed with polymerase chain reaction method. The final result was analyzed as the ratio between fluorescence of c-myb gene and the control gene., Results: Mean level of expression of c-myb gene in leukaemia cells was statistically significantly higher than in the control group (0.71+/-0.53 vs. 0.51+/-0.22; p=0.05), as well as c-myb level between leukaemia cells in relapse cases and controls (0.83+/-0.23 vs. 0.51+/-0.22; p=0.01). There was no difference between c-myb expression in different diagnosis. The comparison of c-myb expression in good and poor response group was not statistically significant (p=0.33)., Conclusions: Possible influence of increased expression of c-myb gene in the promotion of leukaemia was found. The role of c-myb expression as a prognostic factor in acute leukaemias of children was not confirmed.

Unlabelled: Approximately 2% of of all malignant tumours in boys are localised in the testis. Among them 80% are germ cell tumours with the malignant elements of yolk sac tumour. AIM of the study was evaluation of the efficacy of malignant testicular tumour treatment programme in children., Material and Methods: Since 1998 31 boys aged 1 month to 18 years (median 14 years) with malignant testicular tumours were enrolled in the multicentre trial. Patomorphologically clear yolk sac tumour (33%) and mixed germ cell tumour (42%) with the majority of yolk sac tumour component or carcinoma embryonale, occurred most often. Alfa-feto-protein was increased in 63% and choriogonadotropin in 26 patients. 61% patients had local clinical stage and the tumour was localized in the testis. In 39% patients tumour exceeded the testis margin. 4 patients were excluded from analysis as 3 are actually treated and 1 died on the second day of admittance to hospital. All patients received TGM 95 regimen (Tumeurs Germinales Malignes). Surgery (orchidectomy) was applied in 27 boys, 26 were primary (81% complete), 3 secondary (100% complete). 33% received no chemotherapy after surgery, in 41% VBP protocol (vinblastine, bleomycin, cisplatin) was given and in 26%o VIP protocol (ethoposide, ifosphamide, cisplatin). Two patients received also ABK (adriamycine, bleomycin, carboplatin)., Results: Among 26 children with germ cell tumours, 25 (96%) are alive, 23 (88%) are in first remission after completion of treatment. One child died due to central nervous system metastases. 2 children had local recurrence treated with chemotherapy or surgery with good result. Median follow-up is 45 months., Conclusions: TGM regimen is highly efficient in the treatment of malignant testicular tumours. Problems occur in cases of disseminated disease.

Unlabelled: Approximately 1% of all malignant tumours among children are localized in the ovary. The majority belongs to germ cell tumours and occurs in the peripubertal period. AIM of the study was the evaluation of the efficacy of malignant ovarian germ cell tumour treatment programme in children., Material and Methods: Since 1998, 40 girls with malignant ovarian tumours were enrolled in the multicentre trial. Mixed germ cell tumours with yolk sac elements and dysgerminoma occurred the most often. Alfa-fetoprotein (AFP) was increased in almost one half of patients. Tumour exceeded the ovary margin in more than half the patients and 25% were qualified as high risk group. 38 children completed the treatment. All but one patient with neuroblastoma received TGM protocol (Tumeurs Germinates Malignes). A VBP regimen (vinblastine, bleomycin, cisplatin) was applied in 19 girls, VIP regimen (etoposide, ifosfamide, cisplatin) in 16, two received no chemotherapy. Due to delayed remission after first-line chemotherapy it was prolonged with ABK (adriamycine, bleomycine, carboplatin) in 3 patients, 1 megachemotherapy regimen with autologous bone marrow transplantation was realized, one patient received a 1.5 year long oral chemotherapy. All the children underwent surgery, 34 primary (56% complete), 12 secondary (75% complete). 8 children were operated twice., Results: Among 34 children with germ cell tumours and 3 with sex cord tumours who completed the treatment all are alive in the first remission. 1 child with neuroblastoma localised in the ovary died due to recurrence. A median follow-up period was 42 months., Conclusions: The TGM protocol appears to be highly efficient in treatment of germ cell tumours even in advanced stages.

Introduction: Bone marrow transplantation from HLA identical family donors is the treatment of choice for children with severe aplastic anaemia (SAA). When no donor is available, combined immunosuppressive therapy is given., Aim: Evaluation of results of immunosuppressive therapy in children with severe aplastic anaemia., Material and Methods: SAA was diagnosed in 85 children (31 girls, 54 boys) aged 2-17.5 years in the eleven centres of the Polish Paediatric Leukaemia and Lymphoma Study Group (PPLLSG) in Poland between 1993-2003 years. All patients received protocol of the Severe Aplastic Anaemia Working Party of the Europe Bone Marrow Transplant (EBMT): antilymphocyte globulin or antithymocyte globulin, cyclosporin A, prednisolone and granulocyto- or granulocyto-macrophagic-cell stimulation factor was additionally administered during deep neutropenia. Haematological response was evaluated on day 84, 112 or 180 of the therapy., Results: complete remission occurred in 43 patients (50.5%), partial remission in 22 (25.4%), no response was obtained in 20 children (23.7%) in 180 day of the therapy. Period of observation was from 12 months to 10.5 years. During this time relapse occurred in 6 patients (7%). We observed 16 deaths: 7 early during the first 3 months of immunosuppressive therapy (IS) and 9 after the first 3 months of IS., Conclusion: the actual survival at 10-years, after immunosuppressive therapy is 81.2% in our group. Transformation to leukaemia or myelodysplastic syndrome (MDS) was not observed in any of our patients. We observed one case with paroxysmal nocturnal haemoglobinuria (PNH).