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Dantonello, T.M.* ; Int-Veen, C.* ; Schuck, A.* ; Seitz, G.* ; Leuschner, I.* ; Nathrath, M. ; Schlegel, P.G.* ; Kontny, U.* ; Behnisch, W.* ; Veit-Friedrich, I.* ; Kube, S.* ; Hallmen, E.* ; Kazanowska, B.* ; Ladenstein, R.* ; Paulussen, M.* ; Ljungman, G.* ; Bielack, S.S.* ; Klingebiel, T.* ; Koscielniak, E.* ; Cooperative Weichteilsarkom Studiengruppe (CWS)*

Survival following disease recurrence of primary localized alveolar rhabdomyosarcoma.

Pediatr. Blood Cancer 60, 1267-1273 (2013)
DOI PMC
Open Access Green as soon as Postprint is submitted to ZB.
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. Pediatr Blood Cancer 2013;60:1267-1273. © 2013 Wiley Periodicals, Inc.
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Publication type Article: Journal article
Document type Scientific Article
Corresponding Author
Keywords Alveolar rhabdomyosarcoma; Children; Recurrence
ISSN (print) / ISBN 1545-5009
e-ISSN 1096-911X
Journal Pediatric Blood and Cancer
Quellenangaben Volume: 60, Issue: 8, Pages: 1267-1273 Article Number: , Supplement: ,
Publisher Wiley
Non-patent literature Publications
Reviewing status Peer reviewed
Institute(s) CCG Osteosarcoma (PATH-KOS)