Fusions Involving Paired Box and Fork Head Family Transcription Factors in the Pediatric Cancer Alveolar Rhabdomyosarcoma
Rhabdomyosarcoma (RMS) is a soft tissue tumor with striated muscle differentiation which usually presents in pediatric patients (Raney et al. 1993). Though histopathologic classification has evolved over time, two major forms of childhood RMS are consistently recognized, embryonal (ERMS) and alveolar (ARMS). These two histopathologic entities have distinct clinical behaviors. ERMS presents mainly in children less than 10 years old; occurs predominantly in the head and neck, genitourinary tract, and retroperitoneum; and is associated with a favorable prognosis. In contrast, ARMS presents mainly in adolescents and young adults, often occurs in the extremities, and is associated with an unfavorable prognosis (Tsokos et al. 1992). This unfavorable prognosis is related to the propensity for early and wide dissemination, often involving bone marrow, and to the poor response to chemotherapy.
KeywordsPaired Domain Translocation Breakpoint Chimeric Transcript Alveolar Rhabdomyosarcoma Fork Head Domain
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