ONS Connect - December 2008 - (Page 8) JuSTiN [By Deborah McBride, RN, MSN, CPON ®, Contributing Editor] Gene May Cause Childhood Neuroblastoma M utations of a gene called anaplastic lymphoma kinase (ALK) are behind most incidences of hereditary neuroblastoma, a rare and deadly childhood cancer, as well as high-risk noninherited forms of the disease, a new study suggests. Researchers hope that this discovery will allow them to offer the first genetic tests to families affected by the inherited form of the disease. Drugs already are being developed that target the same gene in adult cancers, and the report indicated that it will not take as long to test these treatments in childhood neuroblastoma. The researchers collected genetic data from families around the world with a history of neuroblastoma. They performed genome-wide scans of the DNA of 10 families with a history of neuroblastoma. Of the six families with at least three cases of the disease, four had an extra copy of the mutation on the ALK gene. The other two families had mutations in a different gene, the PHOX2B gene, which has been found before in a small number of inherited cases of neuroblastoma. The researchers concluded that inheritable mutations of ALK are the main cause of familial neuroblastoma and that this cell-surface kinase is a therapeutic target for the disease. Researchers hope that these results will allow them to offer simple, noninvasive screening for patients with a family history of neuroblastoma. An ultrasound or urine test could be used to monitor children with ALK mutation and aid early detection of potential neuroblastoma. Mosse, Y.P., Laudenslager, M., Longo, L., Cole, K.A., Wood, A., Attiyeh, E.F., et al. (2008). Identification of ALK as a major familial neuroblastoma predisposition gene. Nature, 455(7215), 930–935. complete response, radiographically confirmed (median follow-up was one year); neither pulmonary function nor quality of life was affected significantly. Overall survival was promising for patients with NSCLC (70% at one year; 48% at two years). Compared to standard radiation therapy protocols, RFA could affect a smaller volume of normal lung tissue and can be performed in less time (one-day treatment plus median three-day hospitalization versus six to seven weeks of radiotherapy). Lencioni, R., Crocetti, L., Cioni, R., Suh, R., Glenn, D., Regge, D., et al. (2008). Response to radiofrequency ablation of pulmonary tumours: A prospective, intention-to-treat, multicentre clinical trial (the RAPTURE study). Lancet Oncology, 9(7), 621–628. Radiofrequency Ablation Treats Malignant lung Nodules adiofrequency ablation (RFA) has been found to generate a complete response in a high percentage of patients in an industry-sponsored, prospective, single-arm, multicenter trial involving patients with early-stage non-small cell lung cancer (NSCLC) or pulmonary metastases (overall tumor size < 3.5 cm; median size = 1.5 cm; total number of tumors in both groups = 183), who were ineligible for surgery, standard radiotherapy, or chemotherapy. RFA involves applying thermal energy to a probe inserted into or near a tumor mass to produce coagulation necrosis in a controlled fashion. Treatment was successful in 105 of the 106 patients (99%, 137 total procedures). Complications included 27 pneumothoraces and four pleural effusions. Of the 85 patients for whom radiographic tumor-response data were available, 75 (88%) had R Thin film Delivers Controlled Drug Doses ccording to a recent study, a new thin-film coating can deliver controlled drug doses to specific targets in the body following implantation, essentially serving as a micro pharmacy. The film could be used to deliver drugs for cancer, epilepsy, diabetes, and other diseases. It is among the first drug delivery coatings that can be remotely activated by applying a small electric field. According to the researchers, providers could control what is delivered when it is needed in a systematic fashion. The film, which is about 150 nanometers (billionths of a meter) thick, can be implanted in specific parts of the body. It consists of alternating layers of two materials: a negatively charged pigment and a positively charged drug molecule. The pigment sandwiches the drug molecules and holds them in place. The films can carry discrete packets of drugs that can be released separately, A 8 ONS CONNECT December 2008
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