Survival following disease recurrence of primary localized alveolar rhabdomyosarcoma. Issue 8 (15th February 2013)

• Record Type: Journal Article
• Authors: Dantonello, Tobias M.; Int‐Veen, Christoph; Schuck, Andreas; Seitz, Guido; Leuschner, Ivo; Nathrath, Michaela; Schlegel, Paul‐Gerhardt; Kontny, Udo; Behnisch, Wolfgang; Veit‐Friedrich, Iris; Kube, Stefanie; Hallmen, Erika; Kazanowska, Bernarda; Ladenstein, Ruth; Paulussen, Michael; Ljungman, Gustaf; Bielack, Stefan S.; Klingebiel, T.; Koscielniak, E.
• Is Part Of: Pediatric blood & cancer. Volume 60:Issue 8(2013:Aug.)
• Page Start: 1267
• Page End: 1273
• Publication Date: 2013-02-15
• Subjects: Tumors in children -- Periodicals; Blood -- Diseases -- Periodicals; Cancer in children -- Periodicals; 618.92
• DOI: 10.1002/pbc.24488
• Languages: English
• ISSNs: 1545-5009

Abstract

<abstract abstract-type="main" xml:lang="en"> <title>Abstract</title> <sec id="pbc24488-sec-0001" sec-type="section"> <title>Background</title> <p>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.</p> </sec> <sec id="pbc24488-sec-0002" sec-type="section"> <title>Methods</title> <p>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.</p> </sec> <sec id="pbc24488-sec-0003" sec-type="section"> <title>Results</title> <p>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.</p> </sec> <sec id="pbc24488-sec-0004" sec-type="section"> <title>Conclusion</title> <p>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.</p> </sec> </abstract>