Publication Date:
2012-11-17
Description:
Rhabdomyosarcoma is the most common soft tissue sarcoma in the pediatric population, accounting for 5-8% of all childhood cancers. 1 There are two major histological subtypes: alveolar (ARMS) and embryonal (ERMS). The ARMS is genetically characterized by the reciprocal translocation t(2;13)(q35;q14) and its variant t(1;13)(p36;q14) resulting in PAX3/FOXO1 and PAX7/FOXO1 fusion genes, respectively. The embryonal subtype has a relatively better prognosis and different genetic features, generally gain of whole chromosomes 2, 7, 8, 11, 12, 13, and 20, 2,3 not associated with any consistent structural anomaly. Although ERMS is the most frequent subtype, accounting for 68% of RMS, 4 its genetic characterization remains incomplete and the clinicopathologic implications are not well established. We report here the histopathological and genetic findings in six ERMS (primary tumors and its recurrences) derived from three patients. © 2012 Blackwell Publishing Ltd
Print ISSN:
0309-0167
Electronic ISSN:
1365-2559
Topics:
Medicine