title: Soft tissue sarcoma as a second malignant neoplasm in the pediatric age group.
abstract: BACKGROUND
  Survivors of childhood malignancies have an increased risk of developing second malignant neoplasms (SMN) due to their prior treatment and/or genetic susceptibility. A small proportion of SMNs are soft tissue sarcomas (STS), whose prognosis is generally thought to be poor, though publications on such patients' treatment and outcome is limited.
  
  
  METHODS
  The authors analyzed 25 patients who were registered for the Italian Cooperative Group protocols for pediatric STS from 1979 to 2000. The primary tumor was STS in five patients; Hodgkin disease in five patients; leukemia in four patients; retinoblastoma, neuroblastoma, and Wilms tumor in two patients each; and other tumor types in five patients. SMNs occurred after a median of 8 years (range, 1.9-15.0 years) and included rhabdomyosarcoma (RMS) in 4 patients, malignant peripheral nerve sheath tumor in 4 patients, extraosseous Ewing family tumor (EFT) in 4 patients, leiomyosarcoma in 3 patients, fibrosarcoma in 2 patients, synovial sarcoma in 2 patients, and other tumor types in 6 patients. Treatment generally was administered according to the guidelines for primary STS.
  
  
  RESULTS
  Seven non-RMS patients with STS underwent surgery alone, whereas 18 patients received chemotherapy and 8 patients received radiotherapy. Retreatment was feasible with acceptable toxicity. Fifteen patients were alive in complete remission of their SMN at the time of last follow-up. Responses to chemotherapy and survival were satisfactory for patients with tumors such as RMS and EFT. Complete tumor resection was correlated with a favorable prognosis in patients with other types of STS and in patients with postirradiation sarcoma. Two patients developed a third malignancy.
  
  
  CONCLUSIONS
  Although prior treatment may hinder the management of these patients, pediatric STS second malignancies can be cured using the same strategies used for de novo pediatric sarcomas. Long-term follow-up is mandatory given the risks of further malignancies and more severe, treatment-related side effects.
authors.affiliation: Division of Hematology/Oncology, Department of Pediatrics, University Hospital of Padua, Padua, Italy. gianni.bisogno@unipd.it
authors.forename: Gianni
authors.initials: G
authors.lastname: Bisogno
authors.forename: Guido
authors.initials: G
authors.lastname: Sotti
authors.forename: Yohann
authors.initials: Y
authors.lastname: Nowicki
authors.forename: Andrea
authors.initials: A
authors.lastname: Ferrari
authors.forename: Alberto
authors.initials: A
authors.lastname: Garaventa
authors.forename: Ilaria
authors.initials: I
authors.lastname: Zanetti
authors.forename: Claudio
authors.initials: C
authors.lastname: Favre
authors.forename: Amalia
authors.initials: A
authors.lastname: Schiavetti
authors.forename: Paolo
authors.initials: P
authors.lastname: Tamaro
authors.forename: Modesto
authors.initials: M
authors.lastname: Carli
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D016609:Neoplasms, Second Primary; D011379:Prognosis; D012509:Sarcoma / Q000188:drug therapy / Q000209:etiology / Q000532:radiotherapy / Q000601:surgery*; D012983:Soft Tissue Neoplasms / Q000188:drug therapy / Q000209:etiology / Q000532:radiotherapy / Q000601:surgery*; D016019:Survival Analysis; D016896:Treatment Outcome