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Survival following ...
Survival following disease recurrence of primary localized alveolar rhabdomyosarcoma
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Dantonello, Tobias M. (author)
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Int-Veen, Christoph (author)
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Schuck, Andreas (author)
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Seitz, Guido (author)
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Leuschner, Ivo (author)
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Nathrath, Michaela (author)
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Schlegel, Paul-Gerhardt (author)
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Kontny, Udo (author)
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Behnisch, Wolfgang (author)
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Veit-Friedrich, Iris (author)
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Kube, Stefanie (author)
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Hallmen, Erika (author)
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Kazanowska, Bernarda (author)
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Ladenstein, Ruth (author)
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Paulussen, Michael (author)
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- Ljungman, Gustaf (author)
- Uppsala universitet,Pediatrik,Barnonkologisk forskning/Ljungman
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Bielack, Stefan S. (author)
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Klingebiel, T. (author)
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Koscielniak, E. (author)
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(creator_code:org_t)
- 2013-02-15
- 2013
- English.
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In: Pediatric Blood & Cancer. - : Wiley. - 1545-5009 .- 1545-5017. ; 60:8, s. 1267-1273
- Related links:
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https://urn.kb.se/re...
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https://doi.org/10.1...
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Abstract
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- 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.
Keyword
- alveolar rhabdomyosarcoma
- children
- recurrence
Publication and Content Type
- ref (subject category)
- art (subject category)
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- By the author/editor
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Dantonello, Tobi ...
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Int-Veen, Christ ...
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Schuck, Andreas
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Seitz, Guido
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Leuschner, Ivo
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Nathrath, Michae ...
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show more...
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Schlegel, Paul-G ...
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Kontny, Udo
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Behnisch, Wolfga ...
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Veit-Friedrich, ...
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Kube, Stefanie
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Hallmen, Erika
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Kazanowska, Bern ...
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Ladenstein, Ruth
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Paulussen, Micha ...
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Ljungman, Gustaf
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Bielack, Stefan ...
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Klingebiel, T.
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Koscielniak, E.
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show less...
- Articles in the publication
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Pediatric Blood ...
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Uppsala University