title: [Analysis of factors influencing treatment results in children with soft tissue head and neck sarcomas - in the material of the Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University of Gdansk].
abstract: The aim of the study was to analyze the prognostic factors for the outcome in childhood head/neck soft tissue sarcomas (STS) treated in the Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University of Gdansk between 1992 and 2007.
  
  
  METHODS
  Among 23 children with STS in ten it was located in non-parameningeal region, in eight it was in the parameningeal region, and in five in the orbit. Patients were qualified to particular disease stages according to the current diagnostic and therapeutic protocols (Cooperative Weichtel-sarkom Studie-CWS). The qualifications were based on the results of radiologic examinations (ultrasonography, computed tomography and magnetic resonance of the head and neck region, computed tomography of the chest and abdomen and bone scintigraphy) and also on myelogram smears and cerebrospinal fluid investigation.
  
  
  RESULTS
  The longest history concerned the parameningeal (8 months), the shortest the orbital soft tissue sarcomas. Six out of ten patients with non-parameningeal STS were referred to the oncologist with a delay of 6.5 months. This was due to the initially false histopathological diagnosis which excluded the neoplastic process. 82.6% of tumours were diagnosed in advanced, inoperable stages. In 16 children rhabdomyosarcomas (including embryonal subtype - RME in 10, alveolar - RMA in five and undifferentiated in one); in seven non-rhabdomyosarcomas (non-RMS) were found. Good response to chemo- and radiotherapy was observed in 60% of children, mainly with RME. Nine children (mainly with non-parameningeal STS) relapsed. 15 patients are alive (including all with orbital, 6/10 with non-parameningeal and 4/8 with parameningeal STS). Eight children died of disease progression.
  
  
  CONCLUSIONS
  1. Poor outcome in our patients with non-parameningeal head/neck STS results from false initial diagnosis cansing a delay in referring them to the oncologist. It is essential to give more training to the histopathologists about neoplasms in children. 2. Because complete tumour resection in parameningeal STS is rarely feasible, the prognosis in this group is uncertain despite intense chemo- and radiotherapy. 3. The prognosis in orbital STS is usually good; however, they need mutilating surgery in selected cases not responding to therapy.
authors.affiliation: Klinika Pediatrii, Hematologii, Onkologiii Endokrynologii Akademii Medycznej w Gdańsku, ul. Debinki 7, 80-211 Gdańsk, Poland. ebien@amg.gda.pl
authors.forename: Ewa
authors.initials: E
authors.lastname: Bień
authors.forename: Teresa
authors.initials: T
authors.lastname: Stachowicz-Stencel
authors.forename: Elzbieta
authors.initials: E
authors.lastname: Adamkiewicz-Drozyńska
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Połczyńska
authors.forename: Danuta
authors.initials: D
authors.lastname: Sierota
authors.forename: Joanna
authors.initials: J
authors.lastname: Stefanowicz
authors.forename: Anna
authors.initials: A
authors.lastname: Szołkiewicz
authors.forename: Marcin
authors.initials: M
authors.lastname: Hennig
authors.forename: Małgorzata
authors.initials: M
authors.lastname: Krawczyk
authors.forename: Anna
authors.initials: A
authors.lastname: Balcerska
mesh_terms: D000293:Adolescent; D002648:Child; D018450:Disease Progression; D005260:Female; D006258:Head and Neck Neoplasms / Q000175:diagnosis / Q000628:therapy*; D006801:Humans; D008297:Male; D011379:Prognosis; D012509:Sarcoma / Q000175:diagnosis / Q000628:therapy*; D016896:Treatment Outcome