title: Localized unresectable non-rhabdo soft tissue sarcomas of the extremities in pediatric age: results from the Italian studies.
abstract: BACKGROUND
  Treatment of initially unresectable nonrhabdo soft tissue sarcomas (NRSTS) in pediatric age is debated, due to their different chemosensitivity. The authors objective was to evaluate clinical features and treatment results observed in a series of Italian patients over a 24-year period.
  
  
  METHODS
  Fifty-two patients age 8-18 years (median 8 years) were observed (1979-2002). Primary sites were on the lower limbs in 41 and on the upper limbs in 11 cases. Clinical TNM and surgical Intergroup Rhabdomyosarcoma Staging systems were adopted. Therapeutic guidelines recommended an initial biopsy plus neoadjuvant chemotherapy. Aggressive delayed surgery of residual disease, including compartmental resections, was recommended. Radiotherapy was suggested only for patients age > 3 years when surgery was incomplete or not feasible.
  
  
  RESULTS
  The evaluation was performed dividing the patients into two categories according to their chemosensitivity. Chemosensitive (CTs) sarcomas, 21: synovial sarcoma, 11; extraosseous Ewing sarcomas, 5; primitive peripheral neuroectodermic tumors, 5. Nonchemosensitive (CTns) sarcomas, 31: fibrosarcoma, 11; malignant peripheral nerve sheet tumors, 10; liposarcoma, 2; hemangiopericitoma adult type, 2; epithelioid sarcoma, 2; and alveolar soft part sarcoma, leiomyosarcoma, clear cell sarcoma, and sarcoma NOS, each 1. Nineteen of 21 patients with CTs-NRSTS were alive without disease: the 5-year overall survival (OS) and progression-free survival (PFS) were 94.4% and 79.3%, respectively; 23 of 31 patients with CTns-NRSTS were alive without disease: 5-year OS and PFS were 75.3% and 68.3%, respectively. Response to neoadjuvant chemotherapy was complete or partial in 10 of 20 evaluable CTs and in 8 of 26 evaluable CTns tumors. The achievement of complete delayed resection was particularly important for CTns-NRSTS. Tumor size < 5 cm, distal site, and tumor grading for CTns sarcomas were often linked to a favorable outcome; no conclusive results were detected concerning age of the patients or T status of the tumor.
  
  
  CONCLUSIONS
  Multidisciplinary treatment without mutilating procedures allowed the cure of most patients with CTs and CTns-NRSTS. Relapses were cured in several cases of CTs tumors, whereas almost all patients with relapsed CTns tumors died due to the high rate of metastatic spread.
authors.affiliation: Department of Pediatrics, Pediatric Surgery, University of Padua, Padua, Italy. cecchett@pediatria.unipd.it
authors.forename: Giovanni
authors.initials: G
authors.lastname: Cecchetto
authors.forename: Rita
authors.initials: R
authors.lastname: Alaggio
authors.forename: Patrizia
authors.initials: P
authors.lastname: Dall'Igna
authors.forename: Gianni
authors.initials: G
authors.lastname: Bisogno
authors.forename: Andrea
authors.initials: A
authors.lastname: Ferrari
authors.forename: Cosimo
authors.initials: C
authors.lastname: Gigante
authors.forename: Michela
authors.initials: M
authors.lastname: Casanova
authors.forename: Guido
authors.initials: G
authors.lastname: Sotti
authors.forename: Ilaria
authors.initials: I
authors.lastname: Zanetti
authors.forename: Modesto
authors.initials: M
authors.lastname: Carli
mesh_terms: D000293:Adolescent; D000367:Age Factors; D017024:Chemotherapy, Adjuvant; D002648:Child; D005121:Extremities; D005260:Female; D006801:Humans; D007558:Italy; D008297:Male; D020360:Neoadjuvant Therapy; D012509:Sarcoma / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D016019:Survival Analysis