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.