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Rhabdomyosarcoma (RMS) is the most common form of soft tissue sarcoma in children. We report the results of the European paediatric Soft tissue sarcoma Study Group (EpSSG) RMS 2005 study, which prospectively evaluated the reduction of chemotherapy in patients with embryonal RMS (ERMS) after initial surgery.Between October 2005 and December 2016, all patients with localised ERMS with an initial microscopically complete resection (IRS group I) with lymph node-negative (N0) were prospectively enrolled in the low-risk (n = 70, subgroup A; age 10 years and tumour size ≤ 5 cm) or standard-risk group (n = 108, subgroup B; age ≥ 10 years or tumour size 5 cm. Subgroup A received 8 courses of vincristine and dactinomycin (VA) for 22 weeks; subgroup B received 4 courses of VA with ifosfamide (IVA) and 5 courses of VA for 25 weeks.The 5-year event-free survival (EFS) and overall survival (OS) were 90.8% (95% confidence interval [CI]: 85.0-94.4) and 95.7% (95% CI: 90.5-98.1), respectively (n = 178). The EFS and OS were 95.5% (95% CI: 86.8-98.5) and 100% (subgroupA), and 87.8% (95% CI: 79.3-93.0) and 93.0% (95% CI: 84.8-96.8)(subgroup B), respectively. Bearman stage 2 veno-occlusive disease (VOD) occurred in 4 very young patients.VA treatment for 8 courses was effective and well tolerated by the subgroup of patients with low-risk ERMS (group A). Four courses of IVA and 5 courses of VA instead of 9 courses of IVA also has very good results. Careful monitoring for liver toxicity is important in very young patients. European union drug regulating authorities clinical trials EUDRACT No. 2005-000217-35.

Aim : We evaluated the roles of the doctor and the medical system in Israel in the diagnostic delay of childhood malignancies. Methods : We investigated the factors affecting the delay in the diagnosis of childhood malignancies in 315 children with solid malignancies, diagnosed and treated in Rambam Medical Center (RMC), between 1993 and 2001. Those factors were divided into two groups: ‘Healthcare-system-related parameters', (factors directly related to the medical system), and ‘Patient-related parameters' (factors that are not part of the medical system, but directly affect medical judgment and decisions, including factors related to the tumor). We also took into consideration epidemiological, social, and medical issues. Results : The main ‘Healthcare-system-related parameters', which were found to influence the delay in diagnosis, were the type of medical authority that was initially consulted; the specialty of the first doctor; the number of additional doctors whom the child had visited, and the number of times the child had visited the first doctor. The main ‘Patient-related parameters' found to have an impact on the delay in diagnosis were the age of the child, the tumor type, and the presenting symptom. Conclusions : The education and awareness of doctors at the primary healthcare clinics must be improved. We suggest ways of improving the approach of doctors and managing a child with a suspected malignancy.

In an attempt to abrogate the deleterious effects of graft-versus-host disease (GVHD), allogeneic transplantation for nonmalignant diseases was performed using high-dose CD34-cell infusion, partial T cell depletion, and no posttransplantation GVHD prophylaxis. Between 1998 and 2004, 16 patients with matched related donors were treated. Median age was 1.5 years (range, 5 months-18 years). The conditioning regimen consisted of busulphan 16 mg/kg, cyclophosphamide 200 mg/kg, antithymocyte globulin (ATG) 25 mg/kg, and fludarabine 200 mg/m 2 . No GVHD prophylaxis was given. High doses of CD34 cells, positively selected by immunomagnetic beads, were infused at a median dose of 10.7 × 10 6 CD34/kg (range, 7.4-50 × 10 6 ). A total of 1 × 10 5 /kg T cells were given. All patients engrafted, with no graft rejections. All were alive and well at a median of 37 months posttransplantation (range, 18-89 months). Only 1 patient developed chronic GVHD. No episodes of severe infection occurred during or after transplantation. Immunologic reconstitution with CD3/CD4 T cells > 200/μL was observed at a median of 117 days and that with naive T cells (CD4/CD45RA) at a median of 188 days posttransplantation. Our findings suggest that allogeneic transplantation from a matched family donor for nonmalignant disorders can be successfully performed using high doses of CD34 cells, moderate T cell depletion, and no posttransplantation immunosuppression.

GPC 0.37 0.59 0.36 0.27 GNR 0.35 0.12 0.3 0.23 Viral 0.72 1.28 1.34 0.0002 Adeno 0.15 0.38 0.25 0.03 BK 0.03 0.1 0.33

We present a case of nearly total obstruction of the trachea in a 15-month-old boy suffering from tracheal fibrosarcoma. The diagnosis was confirmed on histological examination of a removed part of the tumor, with subsequent successful complete resection of tracheal fibrosarcoma. Follow-up to the age of 13 years showed complete recovery with normalization of respiratory functions. The diagnostic and therapeutic aspects of this case are discussed.