title: Complications of pelvic exenteration in children who have genitourinary rhabdomyosarcoma.
abstract: BACKGROUND
  Rhabdomyosarcoma is the most frequently encountered soft tissue sarcoma in children younger than 15 years of age.
  
  
  METHODS
  The authors reviewed the early and long-term morbidity rate and clinical outcome of pelvic exenteration for genitourinary rhabdomyosarcoma (GU-RMS) in children. Of 43 patients with this diagnosis who were seen at St Jude Children's Research Hospital between 1963 and 1994, 17 underwent pelvic exenteration. These 13 boys and four girls ranged in age from 6 months to 15 years (median, 3.5 years). Their primary sites included prostate (n = 12), bladder (n = 4), and uterus (n = 1). All patients received multiagent chemotherapy and either preoperative or postoperative radiation therapy. Anterior exenteration was performed in 15 patients and total exenteration in two. The complications were classified as early or late (occurring within or after 30 days from the date of surgery) and as major or minor (depending on the risk of death or physical or functional disability).
  
  
  RESULTS
  Thirteen of the 17 patients (76%) had 49 surgery-related complications. Wound infection (24%), fistula, abscess, and malnourishment (12% each) were the most common early major complications, with hydronephrosis (35%), bowel obstruction (24%), acute pyelonephritis, fistula, lymphedema, and ureter stenosis (12% each) making up the majority of late complications in this category. Relatively frequent minor complications included protracted paralytic ileus and hematuria (18%), cholelithiasis, chronic diarrhea and, peristoma skin irritation (12%). Disease-free survival was associated with the timing of surgery (P = .002). All but one of the 12 patients who underwent surgery within 6 months of diagnosis are alive, compared with only one of five whose surgery was performed after a longer interval. Pelvic exenteration for GU-RMS was associated with a high rate of serious complications in this series of patients treated over 31 years.
  
  
  CONCLUSIONS
  With recent advances in surgical techniques, as well as improvements in preoperative and postoperative care, this procedure should continue to be applied in cases resistant to conventional therapy.
authors.affiliation: Department of Surgery, St Jude Children's Research Hospital and the University of Tennessee, Memphis 38105, USA.
authors.forename: E L
authors.initials: EL
authors.lastname: Michalkiewicz
authors.forename: B N
authors.initials: BN
authors.lastname: Rao
authors.forename: E
authors.initials: E
authors.lastname: Gross
authors.forename: X
authors.initials: X
authors.lastname: Luo
authors.forename: L C
authors.initials: LC
authors.lastname: Bowman
authors.forename: A S
authors.initials: AS
authors.lastname: Pappo
authors.forename: S C
authors.initials: SC
authors.lastname: Kaste
authors.forename: M M
authors.initials: MM
authors.lastname: Hudson
authors.forename: C A
authors.initials: CA
authors.lastname: Greenwald
authors.forename: J J
authors.initials: JJ
authors.lastname: Jenkins
authors.forename: C B
authors.initials: CB
authors.lastname: Pratt
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D006869:Hydronephrosis / Q000209:etiology; D007223:Infant; D007415:Intestinal Obstruction / Q000209:etiology; D008297:Male; D010385:Pelvic Exenteration / Q000009:adverse effects*; D012189:Retrospective Studies; D012208:Rhabdomyosarcoma / Q000601:surgery*; D013530:Surgical Wound Infection / Q000209:etiology; D013997:Time Factors; D016896:Treatment Outcome; D014565:Urogenital Neoplasms / Q000601:surgery*