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Background: We evaluated the therapeutic results in 44 patients (17 girls and 27 boys) with osteosarcoma from 1997 to 2006.Their average age was 12.8 years (2.5-20.2). 41 patients had localised disease and 3 had primary metastases., Patients and Methods: We treated our 44 patients using CCG 7921 POG 9351 INT 0133, the therapeutic protocol of the North American cooperative Children's Oncology Group.The median of the follow up was 5.5 years (2-11 years)., Results: 40 patients went into complete remission. 19 patients suffered relapses. Of these, 17 patients died - 15 progressed, 1 died due to treatment-related toxicity, 1 died due to secondary acute myeloid leukaemia. As a whole, the patients had a 5-year overall survival rate (OS) of 58.4% and a 5-year event free survival rate (EFS) of 46.7%. The patients with localised extremity osteosarcoma (n = 40) had a 5-year EFS rate of 51%. The patients with good histological response (n = 22) had a 5-year EFS rate of 63.6%, while patients with poor histological response (n = 18) achieved a 5-year EFS rate of 30.5% (p = 0.009)., Conclusion: The results of treatment of patients with localised extremity osteosarcoma and patients with good histological response to preoperative treatment were very good. The prognosis of patients with axial localisation and metastatic involvement was poor.

Coevolution of cellular genetic compartments is a fundamental aspect in eukaryotic genome evolution that becomes apparent in serious developmental disturbances after interspecific organelle exchanges. The genus Oenothera represents a unique, at present the only available, resource to study the role of the compartmentalized plant genome in diversification of populations and speciation processes. An integrated approach involving cDNA cloning, EST sequencing, and bioinformatic data mining was chosen using Oenothera elata with the genetic constitution nuclear genome AA with plastome type I. The Gene Ontology system grouped 1621 unique gene products into 17 different functional categories. Application of arrays generated from a selected fraction of ESTs revealed significantly differing expression profiles among closely related Oenothera species possessing the potential to generate fertile and incompatible plastid/nuclear hybrids (hybrid bleaching). Furthermore, the EST library provides a valuable source of PCR-based polymorphic molecular markers that are instrumental for genotyping and molecular mapping approaches.

Purpose of the Study: Neoplastic diseases continue to arouse much concern in the general population. This also applies to bone tumors. Until recently, these neoplasms, most frequently occurring in children and adolescents, were considered to have the worst possible prognosis with a minimal opportunity for a successful outcome of treatment. This is a likely reason for the still deeply rooted belief that amputation of the affected limb is necessary and disease prognosis is uncertain, which also applies to Ewing's sarcoma. Only lately could these patients be offered a hope of successful treatment including limb salvage., Material: In the period from 1984 to mid-2003, 78 patients with Ewing's sarcoma were treated in our department. Their age ranged from 3 to 25 years, with the maximum occurrence between 13 and 17 years. We performed 27 reconstructions, 13 amputations or exarticulations and 24 resections without replacements. In 13 patients, the procedure was restricted to diagnostic biopsy only, because the tumor was inoperable., Methods: The diagnosis was made on the basis of diagnostic biopsy and, subsequently, neoadjuvant chemotherapy was administered. When this resulted in tumor regression, definitive surgery was performed, involving tumor resection and bone replacement either with autograft or allograft, or with an individual prosthesis. When an implant was not necessary for maintaining limb function, a simple resection was carried out. In the patients in whom the tumor failed to regress, amputation or exarticulation was inevitable. Inoperable tumors were managed by megadose chemotherapy and by radiotherapy. The patients after orthopedic surgery receive adjuvant therapy, including bone marrow transplantation in indicated cases., Results: The evaluation of our 78 patients showed that patient survival is not related to the surgical procedure used. In patients with an early diagnosis and a positive response of the tumor to chemotherapy, the reconstruction procedure appeared to be sufficiently radical in terms of cancer control while preserving limb function; these patients showed neither a significant increase in disease recurrence nor metastatic dissemination. Similar outcomes were also recorded in patients with a simple resection. However, amputation and exarticulation cannot completely be avoided and they are necessary in the patients whose tumor failed to respond to chemotherapy or in whom radical removal of the tumor is not possible. The poorest outcome was recorded in the patients with inoperable tumors, in whom it was not possible to carry out a radical orthopedic operation. The time between the onset of pain and initiation of therapy is also an important factor affecting the treatment outcome. Complications of reconstructive surgery for Ewing's sarcoma recorded in our patient group included osteomyelitis and graft fracture in addition to relapse and metastatic dissemination followed by death., Discussion: Reconstructive surgery for Ewing's sarcoma is carried out in our department in patients with a confirmed diagnosis, in whom neoadjuvant therapy has resulted in tumor regression and in whom the extent and site of a tumor permit this sort of procedure. If this is not possible, limb amputation is indicated. If amputation is not possible due to tumor localization, megadose chemotherapy is administered. The results of long-term survival evaluation of our patients undergoing resection and replacement show that the procedure has been sufficiently radical, because no local recurrence or metastatic dissemination followed by death were recorded. Disease recurrence, death, graft osteomyelitis and fracture of a graft inclusive of osteosynthetic material are the complications reported., Conclusions: This study evaluated the current techniques of treating Ewing's sarcoma, with emphasis on reconstructive surgery leading to limb salvation and maintenance of its full function. The importance of this approach is obvious when we realize that the patients are mostly children and adolescents.

Extra-articular arthrodesis after Grice is indicated for the treatment of serious pes planovalgus or equinovalgus after the stabilisation of muscular relations in patients with infantile cerebral palsy. The surgical technique itself inspired the authors to use glass-ceramic blocks BAS-0 which were manufactured in co-operation with LASACK. In the period of 1995-1996 they used the glass-ceramic blocks 15times in 14 patients (1 patient operated bilaterally). Thirteen times they used glass-ceramic block in extra-articular arthrodesis, of which in 9 cases in combination with the Young technique, in two cases as part of subtalar arthrodeses. The criterion for the evaluation of the results was the axial alignment between talus and the long axis of the first metatarsus. Excellent and good results were achieved in 9 cases. In these cases the bioactive glass-ceramic block was always encased by bone graft and combined with the surgery after Young. In extraarticular arthrodesis the bones are not in contact. According to authors the glass ceramic block behaves in loading of the neighbouring bone as a "corpus alienum". As a result the ingrowth does not occur. Consequently, the radiograph shows a radioluscent line around the block. Even though the blocks were suitable from the viewpoint of their strength, they did not meet the expectations of authors in this indication. There did not occur a stable incorporation in the surrounding bone. Key words: BAS-0 bioactive glass-ceramics, Grice technique, Young technique, glass ceramic block.

Background: Bone marrow transplantation has become the therapeutic method in some forms of malignant haemotopoietic diseases, malignant tumours, inborn errors of metabolism and immunodeficiency states. The objective of the presented work is the analysis of 40 allogenic bone marrow transplantations in children made in 1989-1994., Methods and Results: Bone marrow transplantation was made in 40 children (26 boys and 14 girls), mean age 10.5 years (range 1.5-17.5 years). Indications were acute lymphoblastic leukaemia in 11, acute myeloid leukaemia in 10, chronic myeloid leukaemia in 6, myelodysplastic syndrome in 2, aplastic anaemia and Fanconi's anaemia in 7, non-Hodgkin lymphoma in 2 and inborn errors in 2 children. The donor was in 33 patients in HLA identical sibling and in seven instances a monozygotic twin, HLA non-identical sibling or relative or unrelated matched donor. Bone marrow engraftment was achieved in 35 (87.5%) patients, in one instance the bone marrow was rejected (2.5%) and in four patients (10%) early death occurred before the bone marrow engraftment. On Aug. 15, 1995 20 patients (50%) survived, a relapse developed in 7 (17.5%) and 13 patients died in conjunction with the transplantation (32.5%). The most frequent cause of death were infectious complications (9 children) either in conjunction with the development of graft versus host reaction (6x) or without signs of this reaction (3x). As a prophylaxis of graft versus host disease 24x Cyclosporine A with corticosteroids was used, 16x with methotrexate. A chronic graft versus host disease developed in 6 of 28 children surviving 100 days after transplantation. The greatest problem are infectious (bacterial and mycotic) complications in the phase of bone marrow aplasia before engraftment of the transplanted bone marrow or in conjunction with a graft versus host reaction which cannot be completely avoided by preventive measures., Conclusions: Bone marrow transplantation is also in children an effective therapeutic method of some forms of malignant haematopoietic diseases, malignant tumours and immunodeficiency states. The correct indication, suitable donor, preventive measures against the graft versus host reaction and protection against infectious complications are essential for the success of this pretentious treatment.

Histiocytosis from Langerhans cells is a new term for a group of diseases formerly called histiocytosis X. In The Faculty Hospital Motol the authors treated between July 1974 and December 1980 84 children with this disease. Twenty one suffered from the malignant form (formerly Letterer-Siwe and Hand-Schüller-Christian disease) and in 63 patients the diagnosis of eosinophil granuloma was established. In 21 children with unequivocally malignant disease the authors started chemotherapy immediately after establishment of the diagnosis and in six they indicated in addition radiotherapy. Of these 21 children 16 are in complete remission and 5 children died from progression of the disease during treatment. In the group of 63 children with eosinophil granuloma in 44 only excochleation of the focus was performed. Chemotherapy was administered to 12 children, incl. 5 where it was combined with radiotherapy. A relapse of the disease was recorded in 8 children. At present all patients suffering from the disease are in complete remission.