title: Role of surgery for nonmetastatic abdominal rhabdomyosarcomas: a report from the Italian and German Soft Tissue Cooperative Groups Studies.
abstract: BACKGROUND
  In the current study, the authors aim to evaluate clinical features and treatment results observed in patients from the German and Italian studies who had nonmetastatic abdominal rhabdomyosarcomas (RMS).
  
  
  METHODS
  One hundred sixty-one patients were observed; 78 registered in the German studies between October 1980 and August 1995, and 83 registered in the Italian studies between April 1975 and December 1995. The age range of the patients was 0-18 years (median, 4 yrs). The distribution of tumor sites was as follows: 32 intraperitoneal, 42 retroperitoneal, 75 pelvic, and 12 not otherwise specified (NOS). Most patients had a large and invasive primary mass (26 T1b, 114 T2b). The breakdown in histology was as follows: 116 embryonal, 34 alveolar, and 11 other (leiomyomatous, pleomorphic, and NOS); all cases were staged according to the Intergroup Rhabdomyosarcoma Studies (IRS) system. Nine Group I patients were treated after surgery with chemotherapy (CT) (radiotherapy [RT] was delivered to treat alveolar RMS in the 1991 German and 1988 Italian studies); 19 Group II patients received CT + RT (40-44 Gy); 133 Group III patients underwent neoadjuvant CT +/- surgery and/or RT (54 Gy) + CT. Different CT regimens (based primarily on the administration of vincristine, dactinomycin, doxorubicin, and cyclophosphamide or ifosfamide) were adopted. RT was not recommended for patients age < 3 years.
  
  
  RESULTS
  The 10-year overall survival (OS) and progression-free survival (PFS) were 47.2% and 43.9%, respectively. The OS was related significantly to the following variables: histology (alveolar, 29.4% vs. nonalveolar, 52.1% [P = 0.0156]), tumor size (> 5 cm, 42.1% vs. < 5 cm, 81% [P = 0.005]), age (< 10 yrs, 51.4% vs. >or= 10 yrs, 27.8% [P = 0.02]), complete surgery at diagnosis or after CT (+/-RT) (70.4% vs. 34.4% without it [P = 0.0015]). Most patients who achieved the delayed local control had responded well to neoadjuvant CT.
  
  
  CONCLUSIONS
  Tumor size, histology, age, and initial or delayed achievement of local control were important prognostic factors. Most relapsed patients had unfavorable outcomes.
authors.affiliation: Department of Pediatrics, Division of Pediatric Surgery, University of Padua, Padua, Italy. cecchett@pediatria.unipd.it
authors.forename: Giovanni
authors.initials: G
authors.lastname: Cecchetto
authors.forename: Gianni
authors.initials: G
authors.lastname: Bisogno
authors.forename: Jorn
authors.initials: J
authors.lastname: Treuner
authors.forename: Andrea
authors.initials: A
authors.lastname: Ferrari
authors.forename: Adrian
authors.initials: A
authors.lastname: Mattke
authors.forename: Michela
authors.initials: M
authors.lastname: Casanova
authors.forename: Patrizia
authors.initials: P
authors.lastname: Dall'Igna
authors.forename: Ilaria
authors.initials: I
authors.lastname: Zanetti
authors.forename: Sandra
authors.initials: S
authors.lastname: Volpato
authors.forename: Fortunato
authors.initials: F
authors.lastname: Siracusa
authors.forename: Gianni
authors.initials: G
authors.lastname: Scarzello
authors.forename: Camillo
authors.initials: C
authors.lastname: Boglino
authors.forename: Modesto
authors.initials: M
authors.lastname: Carli
mesh_terms: D000008:Abdominal Neoplasms / Q000601:surgery*; D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009367:Neoplasm Staging; D011379:Prognosis; D011446:Prospective Studies; D012208:Rhabdomyosarcoma / Q000601:surgery*; D015996:Survival Rate; D016896:Treatment Outcome