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Survival following disease recurrence of primary localized alveolar rhabdomyosarcoma
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2013 (English)In: Pediatric Blood & Cancer, ISSN 1545-5009, E-ISSN 1545-5017, Vol. 60, no 8, 1267-1273 p.Article in journal (Refereed) Published
Abstract [en]

Background Recurrences in primary localized alveolar rhabdomyosarcoma (RMA) are common. Post-relapse survival is poor. We evaluated prognostic factors including relapse treatment in patients with recurrent RMA. Methods Relapses occurred in 115/235 patients with nonmetastatic RMA treated in four consecutive CWS-trials after achievement of a complete remission. Sufficient information about post-relapse treatment and outcome could be obtained in 99 patients and was retrospectively analyzed. Results Nine of 99 patients received no salvage therapy and died after a median of 2 months. The remaining 90 patients received multimodal relapse treatment including mandatory chemotherapy. Recurrences were grossly resected in 39 patients; 57 patients received radiation. At a median follow-up from relapse of 8 years, 20 patients were alive and disease-free (5-year post-relapse survival [PROS] 21.3 +/- 8). All surviving patients apart from a single individual had an isolated, circumscribed recurrence. Sixteen of 20 survivors were treated with adequate local relapse therapy (ALRT, i.e., either complete resection or gross resection+radiation). Survival in the subgroup of 27 individuals with circumscribed recurrences and ALRT was significantly better (PROS 53.7 +/- 19) compared with disseminated recurrences and/or tumors treated without ALRT. Absence of primary lymph node involvement, circumscribed relapses, ALRT, and achievement of a second CR were identified as independent favorable risk factors. Conclusion Post-relapse survival for primary localized RMA is generally poor. However, certain patient groups differed significantly in their likelihood of survival and 50% of patients with circumscribed relapses treated with ALRT survived. These findings may form the basis for an evidence-based risk-stratification for recurrent disease including relapse treatment. 

Place, publisher, year, edition, pages
2013. Vol. 60, no 8, 1267-1273 p.
Keyword [en]
alveolar rhabdomyosarcoma, children, recurrence
National Category
Medical and Health Sciences
Identifiers
URN: urn:nbn:se:uu:diva-204847DOI: 10.1002/pbc.24488ISI: 000320399800011OAI: oai:DiVA.org:uu-204847DiVA: diva2:640296
Note

On behalf of the Cooperative Weichteilsarkom Studiengruppe (CWS); Presented in part: At the International Society of Paediatric Oncology SIOP XXXX Congress, Berlin, Germany, October 2–6, 2008.

Available from: 2013-08-13 Created: 2013-08-12 Last updated: 2017-12-06Bibliographically approved

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Ljungman, Gustaf

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