Neuroblastoma screening data: an epidemiologic analysis

Article Abstract:

Neuroblastoma, a congenital tumor arising from nerve-related cells, is one of the most common cancers of early childhood. Long-term survival from advanced disease has not improved in 30 years, in spite of research and new chemotherapy protocols. Metabolites from the tumor can be detected in urine, supporting the hope for early diagnosis (soon after birth) and improved survival. Based on this reasoning, mass screening of six-month-old infants was instituted in Japan, and scientists have called for similar efforts in North America and Europe. However, although the Japanese program was perceived to be fully successful, it was not closely examined by standard epidemiologic methods. The results of standard analysis of these programs are described. Recent research has changed our understanding of the tumor from what was known when the screening programs were first initiated. In the past, the age of the infant at diagnosis and stage of the tumor were strong prognostic indicators. This now seems a rather simplistic view, as it appears there are two types of neuroblastoma. One seems to be a true congenital tumor, probably growing slowly at birth, and has a good prognosis, tending not to metastasize. The other tumor may lie dormant until cancer-related genes are activated at a later age, when it then grows aggressively and metastasizes, usually before detection. Screening programs use one of several methods of varying sensitivity to identify excesses of neurotransmitter (hormones made by nerves) breakdown products. One result of the screening program was that Japanese pediatricians became more aware of the need to examine the abdomen, so that a number of masses were identified by doctors rather than by urine screening. Analysis shows that not enough information was collected by the screening programs to assess their value. Overdiagnosis appeared to occur, as some cases would have been expected to regress spontaneously. The programs appear to have detected cases that would have had a good prognosis even if they were detected later. Further study is needed to understand whether screening for neuroblastoma is beneficial. (Consumer Summary produced by Reliance Medical Information, Inc.)

Japan, Diagnosis, Demographic aspects, Medical screening, Health screening, Neuroblastoma

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Congenital mesoblastic nephroma: possible prognostic and management value of assessing DNA content

Article Abstract:

Congenital mesoblastic nephroma (CMN), a kidney tumor present at birth, is one of the most common types of kidney tumors in infants. In most cases, surgical removal of the tumor cures the disease, but in cases where a subtype of CMN called atypical cellular mesoblastic nephroma is involved, tumors often reappear and spread (metastasize) to other parts of the body such as the lungs. It is not known why some CMNs are successfully treated with surgery, while others are persistent and difficult to treat (more aggressive). In the case of Wilms' tumor (another type of kidney tumor that develops in children), a relationship between the DNA content (the genetic material) of the tumor and its response to treatment has been reported. To determine whether differences in DNA ploidy characterize typical CMN versus atypical cellular mesoblastic nephroma, and whether these differences are correlated with different outcomes, a study was performed of 13 tumors from children with CMN. Tumor cells were analyzed in 11 cases for their DNA content using a technique called flow cytometry. Results showed that nine tumors had the normal amount of DNA (diploid) and two had more than the normal amount (aneuploid). Surgical removal of the tumors, carried out in all cases, was the only treatment given in seven cases. Three patients also underwent chemotherapy (treatment with anticancer drugs) after surgery; two underwent radiation therapy; and one underwent both chemotherapy and radiation therapy. Nine patients had conventional CMN, three had atypical cellular mesoblastic nephroma, and one had a tumor containing both cell types. Although the small size of this sample makes it impossible to determine the relationship between DNA ploidy and prognosis, it is likely that analysis of the DNA content of tumors in patients with CMN could be useful in identifying patients who may require additional treatment after surgery. (Consumer Summary produced by Reliance Medical Information, Inc.)

Care and treatment, Research, Measurement, Physiological aspects, Prognosis, Birth defects, DNA, Kidney cancer, Tumors in children, Pediatric tumors, Nephrology

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