title: Pediatric nonrhabdomyosarcoma soft tissue sarcomas arising at visceral sites.
abstract: BACKGROUND
  Pediatric nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) may rarely occur in visceral tissues, and little is known about their clinical history. The present study retrospectively analyzed a group of patients prospectively registered in Italian pediatric protocols conducted between 1979 and 2004.
  
  
  METHODS
  Inclusion criteria for the study were as follows: a pathological diagnosis of "adult-type NRSTS," arising at visceral sites (lung-pleurae, liver, kidney, and mesentery-bowel); age under 18 years; no previous treatment except for primary surgery; available clinical data; and written consent.
  
  
  RESULTS
  Thirty cases with visceral NRSTS were collected and analyzed. Sites of origin were as follows: mesentery-bowel in 12 cases, lung-pleurae in 11, liver in 5, and kidney in 2. According to the Intergroup Rhabdomyosarcoma Study (IRS) surgical grouping system, patients were classified as follows: nine IRS group I, three group II, 12 group III, and six group IV. Patients were treated with a multimodal approach including surgery, radiotherapy, and/or chemotherapy, according to their characteristics. For the series as a whole, the 5-year event-free and overall survival rates were 33.3% and 40.0%, respectively. The IRS group (reflecting the feasibility of initial complete resection) emerged as the main prognostic factor. Survival rates also correlated with tumor size and local invasiveness, histological subtype, and tumor sites (the worst outcome was seen for tumors arising in the lung and pleurae).
  
  
  CONCLUSIONS
  This study confirmed that visceral NRSTS are aggressive tumors carrying a worse prognosis than pediatric NRSTS arising in soft tissues of the extremities. Local treatment remains the main challenge for these tumors.
authors.affiliation: Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Ferrari
authors.affiliation: Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy.
authors.forename: Chiara
authors.initials: C
authors.lastname: Magni
authors.affiliation: Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: Bergamaschi
authors.affiliation: Pediatric Surgery, Padova University, Padova, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Cecchetto
authors.affiliation: Pathology Department, Padova University, Padova, Italy.
authors.forename: Rita
authors.initials: R
authors.lastname: Alaggio
authors.affiliation: Hematology/Oncology, Ospedale Pediatrico Bambino Gesù IRCCS, Roma, Italy.
authors.forename: Giuseppe Maria
authors.initials: GM
authors.lastname: Milano
authors.affiliation: Pediatric Hematology Oncology, Azienda Ospedaliera Universitaria di Parma, Parma, Italy.
authors.forename: Patrizia
authors.initials: P
authors.lastname: Bertolini
authors.affiliation: Pediatric Onco-Hematology, Stem Cell Transplantation and Cellular Therapy Division, Regina Margherita Children's Hospital, Turin, Italy.
authors.forename: Eleonora
authors.initials: E
authors.lastname: Basso
authors.affiliation: Department of Pediatric Hematology/Oncology, Giannina Gaslini Children's Hospital, Genova, Italy.
authors.forename: Carla
authors.initials: C
authors.lastname: Manzitti
authors.affiliation: Pediatric Oncology Service, Department of Pediatrics, Second University, Napoli, Italy.
authors.forename: Martina
authors.initials: M
authors.lastname: Di Martino
authors.affiliation: Pediatric Oncology, Università di Trieste, Italy.
authors.forename: Nauga
authors.initials: N
authors.lastname: Giurici
authors.affiliation: Department of Pediatrics, University of Bologna, S. Orsola-Malpighi Hospital, Bologna.
authors.forename: Fraia
authors.initials: F
authors.lastname: Melchionda
authors.affiliation: Pediatric Hematology and Oncology Unit, Department of Hematology, Transfusion Medicine and Biotechnology, Pescara.
authors.forename: Valerio
authors.initials: V
authors.lastname: Cecinati
authors.affiliation: Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Chiaravalli
authors.affiliation: Pediatric Hematology and Oncology Division, Padova University, Padova, Italy.
authors.forename: Maria Carmen
authors.initials: MC
authors.lastname: Affinita
authors.affiliation: Pediatric Hematology and Oncology Division, Padova University, Padova, Italy.
authors.forename: Angela
authors.initials: A
authors.lastname: Scagnellato
authors.affiliation: Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy.
authors.forename: Michela
authors.initials: M
authors.lastname: Casanova
authors.affiliation: Pediatric Hematology and Oncology Division, Padova University, Padova, Italy.
authors.forename: Gianni
authors.initials: G
authors.lastname: Bisogno
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D011379:Prognosis; D012983:Soft Tissue Neoplasms / Q000401:mortality* / Q000473:pathology* / Q000628:therapy*; D014781:Viscera / Q000473:pathology
keywords: bowel; children and adolescents; kidney; liver; lung; mesentery; nonrhabdomyosarcoma soft tissue sarcomas; pediatric soft tissue sarcomas; pleurae; visceral sites