Update on childhood rhabdomyosarcoma

Abstract

The overall survival of childhood rhabdomyosarcoma has improved dramatically over the past 10 years. Early diagnosis and appropriate referral to a specialised centre leading to an accurate and timely diagnosis reflects on overall outcome. Recent molecular studies have identified different biological subtypes resulting in the recognition of poorer subgroups and allowing more appropriate treatment to be administered. Clinical trials remain the cornerstone to further improve outcome, now carried out on an international basis.

Figure 1

Distribution of RMS by primary site.

Figure 2

Diagram of the balanced chromosomal translocations resulting in the fusion genes involving PAX3 and PAX7 and the forkhead gene on chromosome 13.

Figure 3

Survival of UKCCSG patients diagnosed 1977–98 by trial entry period. All stages included.