title: [Diagnostic and therapeutic difficulties in soft tissue sarcomas localized in nonparameningeal head and neck region--own experiences].
abstract: BACKGROUND
  Soft tissue sarcomas (MTM) localized within the nonorbital and non-parameningeal head and neck region in children are associated with favourable prognosis. However in our material we have observed many therapeutic failures in this group of patients. The aim of the study was to analyze the reasons for disappointing results of oncological therapy in children with MTM treated between 1992 and 2004.
  
  
  METHODS
  Nine children (M/F: 6/3; mean age 5,5 years). Five patients were diagnosed with rhabdomyosarcoma, four--with non-rhlabdomyosarcoma, including: angiosarcoma, malignant triton tumour, fibrosarcoma and leiomyosarcoma.
  
  
  RESULTS
  mean duration of initial symptoms was 7 months. The first sign of the neoplastic disease in all children was tumour. In as many as five of nine patients initially a false histopathological diagnosis was made based on material obtained from aspiration biopsy of the tumour performed in non-oncological centres. This resulted in a significant delay of the proper diagnosis of malignant disease ranging from 2 to 15 months (mean 7 months). That is why all our patients presented with highly advanced stages of MTM or even in the phase of disease recurrence. Therapy was conducted according to the schemes: MMT-89, CWS-91, CWS-96 and CWS-2002. Durable complete remission after the first line therapy was obtained in one child only. Six patients developed MTM relapse and two--progression during chemotherapy. Finally five children remain disease-free after treatment termination with follow-up of 1 to 1,5 years. Four of then had microscopically complete delayed resection of the tumour or the relapse. Four patients died of neoplasm recurrence and progression. In three of them the proper diagnosis was delayed significantly and they were diagnosed in the first or even second relapse of the tumour.
  
  
  CONCLUSIONS
  Unfavourable course and treatment results in MTM located in nonorbital and nonparameningeal head and neck region in our patients result from initial wrong histopathological diagnosis and delayed therapy institution.
authors.affiliation: Klinika Pediatrii, Hematologii, Onkologii i Endokrynologii Akademii Medycznej w Gdańsku. tsten@amg.gda.pl
authors.forename: Teresa
authors.initials: T
authors.lastname: Stachowicz-Stencel
authors.forename: Ewa
authors.initials: E
authors.lastname: Bień
authors.forename: Joanna
authors.initials: J
authors.lastname: Stefanowicz
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Połczytńska
authors.forename: Danuta
authors.initials: D
authors.lastname: Sierota
authors.forename: Anna
authors.initials: A
authors.lastname: Szołkiewicz
authors.forename: Elzbieta
authors.initials: E
authors.lastname: Drozyńska
authors.forename: Wojciech
authors.initials: W
authors.lastname: Kosiak
authors.forename: Czesław
authors.initials: C
authors.lastname: Stankiewicz
authors.forename: Mirosława
authors.initials: M
authors.lastname: Pietniczka
authors.forename: Andrzej
authors.initials: A
authors.lastname: Kukwa
authors.forename: Piotr
authors.initials: P
authors.lastname: Czauderna
authors.forename: Anna
authors.initials: A
authors.lastname: Balcerska
mesh_terms: D000293:Adolescent; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D005354:Fibrosarcoma / Q000175:diagnosis / Q000628:therapy; D006394:Hemangiosarcoma / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D007223:Infant; D007890:Leiomyosarcoma / Q000175:diagnosis / Q000628:therapy; D008297:Male; D009367:Neoplasm Staging; D011379:Prognosis; D012189:Retrospective Studies; D012208:Rhabdomyosarcoma / Q000175:diagnosis / Q000628:therapy; D012509:Sarcoma / Q000175:diagnosis* / Q000628:therapy*; D016896:Treatment Outcome