title: [Malignant neoplasms localised in the parameningeal region in children treated at two Polish oncological centres-diagnostic and therapeutic dilemmas].
abstract: Malignant neoplasms localized in the parameningeal region include mainly soft tissue sarcomas (MTM), non-Hodgkin s lymphomas (NHL-B) and, less frequently, nasopharyngeal carcinomas. The aim of the study was to analyze diagnostic and therapeutic problems in children with parameniingeal neoplasms treated in Departments of Paediatric Oncology in Gdansk and Lublin between 1992 and 2004.
  
  
  METHODS
  The study includes 32 patients (M/F: 23/9), aged 2 to 17 years, mean 6,3 years. In 17 children MTM was diagnosed: in nine NHL-B-cell and in six--nasopharyngeal carcinoma (lymphoepithelioma). The diagnosis of NHL-B and undifferentiated MTM were made in two children treated previously for NHL-nonB and retinoblastoma. Two cases of NHL appeared in a girl with ataxia-teleangiectasia syndrome.
  
  
  RESULTS
  Initial symptoms lasted from 2 weeks to 24 months, mean 4,5 months for the whole group. In NHL patients mean period ofsymptoms was 4,5 weeks, in MTM- 5,5 months and in lynmphoepithelioma--7 months. Symptoms associated with the tumours localisation (snoring, breathing through the mouth, epistaxis, chronic purulent rhinitis, dysphagia and earache) predominated and were treated initially as upper respiratory tract infections. Cervical lymph nodes enlargement was observed in 30% children with MTM and 83% with lymphoepithelioma. Most of patients presented with highly advanced stages of neoplasms. MTM and NHL-B treatment was conducted according to the protocol approved by the Polish Paediatric Solid Tumours and Leukaemia/Lymphoma Studies Group. In patients with lymphoepithelioma different treatment schemes were administered, including chemo- and radiotherapy. Good response to therapy was found in 13/32 patients (41%). The group included 24% children with MTM (all with embryonic subtype), 56% with NHL-B and 67% with lymphoepithelioma. All these patients attained complete remission after standard line I therapy. But 13 children with MTM, four with NHL-B and two with lymphoepithelioma required more aggressive line II treatment because ofpoor response to therapy (NR) or relapse. Finally, 20 of 32 followed-up patients (62,5%) are in durable complete remission from 10 months to 11 years 4 months (mean 4 years) after therapy discontinuation. This group consists of all nine patients with NHL-B, 67% children with lymphoepithelioma and 41% with MTM. In six children (30%) persistent complications of oncological treatment occurred, including: hypoacusia, postradiation defect of the eye ball, postsurgical facial nerve palsy and cranio-nasal fistula complicated with pneumocephaly. A patient with MTM of maxillary sinus developed a second neoplasm 2 years after first therapy. This was glioblastoma multiforme located in the left parietal lobe (outside the radiation field). At present, the boy is in complete remission 2,5 years after treatment for the second tumour Among 32 children with parameningeal neoplasms 11 patients died (nine with MTM and two with lymphoepithelioma), all in the phase of disease progression (five NR and six after relapse). In two of them (with MTM) the direct cause of death was myelosupressive, gastrotoxic and infectious complications of antitumour therapy. One child still undergoes treatment for MTM relapse.
authors.affiliation: Klinika Pediatrii, Hematologii, Onkologie i Endokrynologii AM w Gdańsku. ebien@amg.gda.pl
authors.forename: Ewa
authors.initials: E
authors.lastname: Bieań
authors.forename: Ninela
authors.initials: N
authors.lastname: Irga
authors.forename: Teresa
authors.initials: T
authors.lastname: Stachowicz-Stencel
authors.forename: Joanna
authors.initials: J
authors.lastname: Stefanowicz
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Połczyńska
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: Adamkiewicz-Drozyńska
authors.forename: Dorota
authors.initials: D
authors.lastname: Birkholz
authors.forename: Joanna
authors.initials: J
authors.lastname: Zawitkowska-Klaczytska
authors.forename: Ewa
authors.initials: E
authors.lastname: Dudkiewicz
authors.forename: Krzysztof
authors.initials: K
authors.lastname: Katski
authors.forename: Joanna
authors.initials: J
authors.lastname: Nurzyńska-Flak
authors.forename: Anna
authors.initials: A
authors.lastname: Balcerska
authors.forename: Czesław
authors.initials: C
authors.lastname: Stankiewicz
authors.forename: Jerzy R
authors.initials: JR
authors.lastname: Kowalczyk
mesh_terms: D000293:Adolescent; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006258:Head and Neck Neoplasms / Q000175:diagnosis* / Q000628:therapy*; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000175:diagnosis / Q000628:therapy; D008297:Male; D008577:Meningeal Neoplasms / Q000175:diagnosis* / Q000628:therapy*; D009303:Nasopharyngeal Neoplasms / Q000175:diagnosis / Q000628:therapy; D009367:Neoplasm Staging; D011044:Poland; D018714:Radiotherapy, Adjuvant; D012008:Recurrence; D012189:Retrospective Studies; D012509:Sarcoma / Q000175:diagnosis / Q000628:therapy; D016019:Survival Analysis; D016896:Treatment Outcome