Childhood Rhabdomyosarcoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Changes to This Summary (08 / 19 / 2014)
Added text about a retrospective study of 1,687 children with rhabdomyosarcoma enrolled in Intergroup studies from 1991 to 2004 that suggests that about one-third of patients can have limited staging procedures that eliminate bone marrow and bone scan examinations at diagnosis (cited Weiss et al. as reference 5).
Previously Untreated Childhood Rhabdomyosarcoma
Revised text to state that in a retrospective trial, starting radiation therapy (RT) within 2 weeks of diagnosis for patients with signs of meningeal impingement was associated with lower rates of local failure but was of borderline significance. Also added text to state that a subsequent comparison of local control, failure-free survival, and overall survival rates showed no statistical difference between early irradiation (day 0) for Group III patients in IRS-IV with cranial nerve palsy and/or cranial base erosion versus later initiation of RT (week 12) for Group III patients in D9603 with similar evidence of meningeal involvement, suggesting that early RT for this group of patients is not necessary. However, both studies administered early irradiation to all patients with intracranial extension of the primary tumor (cited Spalding et al. as reference 44 and level of evidence 2A).
Added text to state that an alternative strategy, used in European International Society of Pediatric Oncology protocols, has been to avoid major radical surgery when possible and omit external-beam RT if complete disappearance of tumor can be achieved by chemotherapy and conservative surgical procedures. The goal is to preserve a functional bladder and prostate without incurring the late effects of RT or having to perform a total cystectomy/prostatectomy. From 1984 to 2003, 172 patients with nonmetastatic bladder and/or bladder/prostate rhabdomyosarcoma were accrued, and the 5-year overall survival rate was 77%. Fifty percent of the 119 survivors had no significant local therapy, and only 26% received RT (cited Jenney et al. as reference 93 and level of evidence 3iiA).
Added Kirsch et al. as reference 99 and level of evidence 3iA.
Added text to state that high-dose chemotherapy with autologous and allogeneic stem cell rescue has been evaluated in a limited number of patients with rhabdomyosarcoma. The use of this modality has failed to improve the outcomes of patients with newly diagnosed or recurrent rhabdomyosarcoma (cited Thiel et al. as reference 132).
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.