[Soft tissue sarcomas of the parameningeal region in children--own observations].

Soft tissue sarcomas (STS) non-Hodgkin's lymphomas and less frequently nasopharyngeal carcinomas are the most common malignancies located in the parameningeal region in children.

OBJECTIVE

To assess diagnostic and therapeutic problems in children with parameningeal STS treated in the Departments of Paediatric Oncology in Gdansk and Lublin between 1992 and 2006.

METHODS

The study includes 17 patients with parameningeal STS; mean age of patients was 5.6 years. In one boy an undifferentiated STS was diagnosed 7 years after treatment of retinoblastoma.

RESULTS

Initial symptoms lasted from 2 weeks to 24 months, mean 5.5 months. Symptoms associated with parameningeal location of the tumour (snoring, breathing through the mouth, epistaxis, chronic purulent rhinitis, dysphagia and earache) predominated and were treated initially as upper respiratory tract infections. All analysed patients presented with highly advanced stages of STS. Oncological treatment was conducted according to the schemes approved by the Polish Paediatric Solid Tumours Study Group. Good response to therapy was stated only in 24% children with STS. These patients (all with embryonal subtype) entered complete remission after standard I line therapy. 13 children required more aggressive II line treatment because of poor response to therapy (NR - 5 children) or relapse (8 children). Seven of the analysed patients (41%) are in lasting complete remission, from 32 months to 13 years 2 months (mean 5 years) after therapy discontinuation. In four children (23%) persistent complications of oncological treatment occurred, including postradiation defect of the orbital bulb, postsurgical facial nerve palsy and cranio-nasal fistula complicated with pneumocephaly. A patient with STS of maxillary sinus developed a second neoplasm 2 years after first therapy. This was a glioblastoma multiforme located in the left parietal lobe (outside the radiation field). At present, the boy is in complete remission nearly 4.5 years after treatment for the second tumour. Ten patients died, all in the phase of disease progression. In two of them myelosupressive, gastrotoxic and infectious complications of antitumour therapy were the direct cause of death.

CONCLUSIONS

1. Non-specific initial symptoms of soft tissue sarcomas located in parameningeal region in children suggesting inflammatory process result in diagnostic dilemmas and proper diagnosis delay. 2. Because complete resection of the parameningeal STS is unfeasible, the prognosis is poor in spite of aggressive chemo- and radiotherapy. 3. Complex therapy carries a risk of severe complications, thus it should be conducted in highly specialized oncological centres.