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Researchers at Children's Hospital Boston have identified a new type of cancer with a rapidly fatal course. Their discovery will be published in the September 1, 2001, issue of Cancer. The disease affects the upper respiratory tract of young patients, predominantly girls, and usually results in rapid death. The cancer is characterized by a specific chromosomal abnormality called a 15;19 translocation. This condition is caused by abnormal cell division, during which pieces of chromosome 15 becomes stuck to chromosome 19, and pieces of chromosome 19 becomes stuck to chromosome 15. ''The discovery of this cancer syndrome is an important advance'', said Sara Vargas, M.D., in the Department of Pathology at Children's Hospital Boston, and lead author of this study. ''Having found this chromosomal abnormality, scientists have gained a deeper understanding about the biology of cancer, and eventually, will be able to search these regions of the chromosome for the specific gene, or genes, that are responsible for the out-of-control growth seen in cancer.'' Doctors at Children's recognized the disease syndrome by examining the chromosomes in biopsy specimens from two patients with cancer, a 12-year-old girl who died after 13 weeks, and a 13-year-old girl who died after 36 weeks. The abnormalities found had not been seen previously at Children's Hospital Boston, but a handful of patients with the same translocation were described at other institutions. By putting the Children's Hospital Boston findings together with the reports of others, a specific syndrome emerged. The patients involved in this study were young (age 5 to 34 years) and 5 of 6 were female. The tumors were located in or near the upper respiratory tract; they were classified as ''carcinoma,'' and obstructed the neck veins. Eventually, all patients died rapidly of their disease, and all tumors contained the 15;19 translocation. This discovery illustrates the power of cytogenetic analysis, a relatively new technique in pathology that allows for the study of tumor chromosomes. For the disease newly identified at Children's Hospital Boston, named t(15;19) carcinoma, the chromosomal abnormality is a key element that defines the disease entity. Furthermore, new techniques that go beyond looking under a microscope are adding a powerful new dimension to the understanding of cancer.
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