title: Survival following disease recurrence of primary localized alveolar rhabdomyosarcoma.
abstract: 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.
  
  
  CONCLUSIONS
  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.
authors: Olgahospital, Klinikum Stuttgart, Stuttgart, Germany. tobias.dantonello@olgahospital-stuttgart.de
authors: Tobias M
authors: TM
authors: Dantonello
authors: Christoph
authors: C
authors: Int-Veen
authors: Andreas
authors: A
authors: Schuck
authors: Guido
authors: G
authors: Seitz
authors: Ivo
authors: I
authors: Leuschner
authors: Michaela
authors: M
authors: Nathrath
authors: Paul-Gerhardt
authors: PG
authors: Schlegel
authors: Udo
authors: U
authors: Kontny
authors: Wolfgang
authors: W
authors: Behnisch
authors: Iris
authors: I
authors: Veit-Friedrich
authors: Stefanie
authors: S
authors: Kube
authors: Erika
authors: E
authors: Hallmen
authors: Bernarda
authors: B
authors: Kazanowska
authors: Ruth
authors: R
authors: Ladenstein
authors: Michael
authors: M
authors: Paulussen
authors: Gustaf
authors: G
authors: Ljungman
authors: Stefan S
authors: SS
authors: Bielack
authors: T
authors: T
authors: Klingebiel
authors: E
authors: E
authors: Koscielniak
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000401:mortality* / Q000628:therapy*; D012189:Retrospective Studies; D018232:Rhabdomyosarcoma, Alveolar / Q000401:mortality* / Q000628:therapy*; D015996:Survival Rate; D013997:Time Factors