Childhood Rhabdomyosarcoma Treatment (PDQ®)–Health Professional Version

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Changes to This Summary (12/02/2016)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

General Information About Childhood Rhabdomyosarcoma

Revised text about the PAX3/PAX7-FOXO1 gene fusions in patients with alveolar rhabdomyosarcoma.

Added text about sentinel lymph node biopsy for evaluating lymph nodes in the extremities and trunk (cited Dall'Igna et al., Alcorn et al., Wright et al., and Parida et al. as references 55, 56, 57, and 58, respectively).

Cellular Classification for Childhood Rhabdomyosarcoma

Added text to state that anaplasia has been observed in 13% of embryonal rhabdomyosarcoma cases, and its presence may adversely influence clinical outcome in patients with intermediate-risk embryonal rhabdomyosarcoma. However, anaplasia was not shown to be an independent prognostic variable in a multivariate analysis.

Added text about characteristics and outcomes of sclerosing rhabdomyosarcoma.

The Alveolar rhabdomyosarcoma subsection was extensively revised.

Stage Information for Childhood Rhabdomyosarcoma

Revised text to state that bilateral bone marrow aspirates and biopsies and bone scans are for selected patients.

Treatment of Previously Untreated Childhood Rhabdomyosarcoma

Revised text to state that because of the inferior prognosis of patients with nodal involvement, the current recommendation is to sample the regional lymph nodes.

Revised text to state that for girls with genitourinary primary tumors who will receive pelvic irradiation, ovarian transposition (oophoropexy) before radiation therapy should be considered unless dose estimations suggest that ovarian function is likely to be preserved. Alternatively, ovarian tissue preservation is under investigation and can be considered.

Treatment of Recurrent Childhood Rhabdomyosarcoma

Added text about the ADVL1412, ADVL1621, and ADVL1312 trials as treatment options under clinical evaluation.

This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® - NCI's Comprehensive Cancer Database pages.

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