[Rhabdomyosarcomas in the naso-paranasal area]

The nasoparanasal region rhabdomyosarcoma belongs to the group of the rare malignant diseases, however it nevertheless represents major diagnostic and therapeutic problem. The study is aimed at presenting clinical casuistry of the nasoparanasal region rhabdomyosarcoma in adults and children, for the purpose of assessment of modern diagnostic and therapeutic possibilities. The study was retrospective and included the patients with evidenced and pathohistologically verified rhabdomyosarcoma of the nasoparanasal region diagnosed and treated at the Institute of Otorhinolaryngology and Maxillofacial Surgery of the Clinical Centre of Serbia, during the period 1988-2001, and subsequently at the Institute of Oncology in Belgrade. The total of 9 patients aged 3-50 years (median 15) were assessed, with the female to male ratio being 4:5. Seven patients were previously hospitalized in other health institutions. The following parameters were analyzed: sex, age group, number of hospitalizations, diagnostic procedures, histological tumour types, mode of treatment and disease outcome. In 7 patients, the tumour spread beyond the nasoparanasal region--in 6 into the orbit, in 5 into the epipharynx, in 3 intracranially, in 1 into the pterygopalatine pit and in another 1 into the parapharyngeal space. Regional lymph nodes were affected only in one patient, while distant bone metastases were evidenced on another one. Embryonal type of the rhabdomyosarcoma was verified in 7 patients, while two had the alveolar type. In the affected children mean survival was significantly longer (76.3 months) in comparison to the one observed in adults (11.5 months). Therapeutic procedure included combined application of the chemotherapeutic protocols (adjusted with respect to the age group and stage of the disease) and radiotherapy. Radical surgical procedures were applied on in case of stage I tumours, and they may also be used in rest-relapse cases subsequent to the applied chemo-, i.e., radiotherapy.