title: Childhood leiomyosarcoma: a report from the soft tissue sarcoma Italian Cooperative Group.
abstract: BACKGROUND
  Only a few reports on the clinical features and management of childhood leiomyosarcoma are available. To contribute additional information on the management of this rare tumor, we report on a series of 16 pediatric patients treated from 1982 to 1998 by the Soft Tissue Sarcoma Italian Cooperative Group.
  
  
  METHODS
  Primary surgery was conservative in all but two patients, and consisted of biopsy--three cases, non-radical excision--four, and radical resection--nine (involving a primary re-excision in 4 of 9). In two cases secondary radical surgery was performed after primary chemotherapy. Chemotherapy was administered to 9 of 16 patients, radiotherapy to three.
  
  
  RESULTS
  After a median follow-up of seven years (range 3-18), the five-year event-free survival (EFS) and overall survival were 56.3% and 72.9%, respectively; 12 of 16 patients were alive (nine of them in continuos complete remission). Univariate analysis was performed to compare EFS according to different subgroups: size represented the most significant prognostic factor.
  
  
  CONCLUSIONS
  Complete surgical resection is the mainstay of treatment for leiomyosarcoma. The role of both adjuvant chemotherapy and radiotherapy has yet to be established, and awaits cooperative multicentric studies.
authors.affiliation: Pediatric Oncology Unit, Istituto Nazionale Tumori, Milano, Italy. ferrari@istitutotumori.mi.it
authors.forename: A
authors.initials: A
authors.lastname: Ferrari
authors.forename: G
authors.initials: G
authors.lastname: Bisogno
authors.forename: M
authors.initials: M
authors.lastname: Casanova
authors.forename: C
authors.initials: C
authors.lastname: Meazza
authors.forename: G
authors.initials: G
authors.lastname: Cecchetto
authors.forename: M A
authors.initials: MA
authors.lastname: Mancini
authors.forename: I
authors.initials: I
authors.lastname: Zanetti
authors.forename: R
authors.initials: R
authors.lastname: Alaggio
authors.forename: V
authors.initials: V
authors.lastname: Ninfo
authors.forename: M
authors.initials: M
authors.lastname: Carli
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007558:Italy; D007890:Leiomyosarcoma / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery*; D008297:Male; D016019:Survival Analysis