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Patients affected by neurofibromatosis type 1 (NF1) are at higher risk of developing soft‐tissue sarcomas (STS) than the general population. The clinical findings and outcome in 43 children and adolescents with NF1 treated for STS in the Italian protocols between 1988 and 2004 are reported.The study included 37 patients with neurogenic sarcomas (36 malignant peripheral nerve sheath tumors [MPNST], 1 triton tumor) and 6 cases of rhabdomyosarcoma (RMS). The prevalence of NF1 observed during the study period was 43% in the MPNST population and 1% in the RMS group.Most patients with neurogenic sarcomas had large, invasive tumors. Five‐year event‐free and overall survival rates were 19% and 28%, respectively. Two of 16 patients with evaluable disease responded to chemotherapy. All 6 RMS patients were ≤3 years old and had embryonal subtype, 5 of 6 arising in the genitourinary tract or pelvis (paravesical); 4 were alive in first remission at the time of the analysis, 1 was alive in second remission after a local recurrence, and 1 died of disease.The occurrence of STS in pediatric patients with NF1 syndrome in Italy is discussed, confirming that NF1 patients have a high risk of developing STS, and particularly MPNST, often with an aggressive clinical presentation and poor outcome. Cases of RMS tended to have particular features (early age, embryonal histotype, genitourinary site) and their outcome seemed to resemble that of the general RMS population. Cancer 2007 © 2007 American Cancer Society. [ABSTRACT FROM AUTHOR]

Abstract Introduction?? We report a simplified method of performing antibiotic lock therapy (ALT) based on a disposable central venous catheter (CVC) hub device, CLC 2000, enabling an open-ended CVC to be flushed with normal saline solution without heparin. Methods?? ALT was administered through a CLC 2000 connector for recurrent CVC-bloodstream infections (BSI) by the same organism in four patients and for CVC colonization in five patients. Results?? The antibiotic concentration obtained in the lumen of the CVC with ALT was 2,500-fold higher than the minimum inhibiting concentration of targeted bacteria for patients treated with vancomycin, 2,500?80,000-fold higher for patients treated with teicoplanin, and 10,000-fold higher for the patient treated with amikacin. All CVC-BSIs treated with ALT resulted in complete clinical and microbiological responses. No case of malfunction in withdrawing or flushing the CVC and no precipitation during the administration of the antibiotic solution was observed. No recurrence of CVC-BSI or CVC colonization by the same organism was diagnosed during subsequent follow-up, despite the fact that all patients had further periods of severe neutropenia. At the last follow-up, three CVCs had been removed for other infections (fever of unknown origin in two; fungemia in one), four CVCs had been removed at the end of therapy, and one CVC is still in situ 20?months after ALT. Conclusions?? In conclusion, a course of ALT is feasible in cancer patients with infected but much-needed CVCs before resorting to removal. The use of the CLC 2000 connector device simplifies the procedure for preparation and administration of ALT without compromising its efficacy. [ABSTRACT FROM AUTHOR]