Retrospective evaluation of soft tissue sarcomas followed in GUTF Pediatric Oncology Department

Soft tissue sarcomas are classified into two main groups called rhabdomyosarcomas and nonrhabdomyosarcomatous soft tissue sarcomas. Soft tissue sarcomas represent approximately 6.5% of childhood cancers in our country. Prognosis in these two groups depends on metastasis at the time of the diagnosis, origin of the tumor and the treatment procedure that is chosen. In our study we retrospectively evaluated the epidemiologic and clinical data of soft tissue sarcoma patients followed in our pediatric oncology department. We also analyzed the relationship between tumor regression after the induction therapy and prognosis, overall survival and event free survival of our patients.The mean age of 34 rhabdomyosarcoma patients was 7.0±4.7 years. Male/female ratio was 1/1.3. The most common symptom was mass other than proptosis. The mean duration of time from the beginning of symptoms until the administiration to the hospital was about a month.The origin of the tumors were as follows: 61.7% head and neck, 20.6% abdomen, 14.7% extremity and 2.9% thorax. Histopathologic subgroups were: 18 (52,9%) embryonal, 11 (32,3%) alveolar, 2 (5,8%) spindle cell and 3 (8,8%) undifferantiated RMS. Patients were staged according to IRS staging system: 12 (37,5%) stage I, 2 (6,7%) stage II, 11 (34,3%) stage III, 7 (21,2%) stage IV. Patients were treated with a combination of surgery+chemotherapy (CWS-96 or IRSG) and radiotherapy according to their stage.Age, stage, metastasis and response to induction therapy were the important prognostic factors for overall and event free survival. Involvement of lymph nodes was also a predictive factor for event free survival. Histology alone was not effective on prognosis. Orbita localisation had the best prognosis among the tumors originated from the head and neck region.There were 5 (71,4%) male and 2 (28,5%) female patients in nonrhabdomyosarcomatous soft tissue sarcoma group. The mean age was 10,1±4,6 years. Primary tumor localisations were extremity in 3 (42,8%) patients, abdomen in 3 (42,8%) patients and thorax in 1 (14,2%) patients. Four patients were diagnosed fibrosarcoma, 2 patients were diagnosed leiomyosarcoma and 1 patient was diagnosed angiosarcoma. Six patients had gross total resection and 1 patient had subtotal resection. Three patients received chemotherapy+radiotherapy because of high histological grade of their tumors. Three patients were totally cured and 3 patients lost follow up.In conclusion; in our pediatric oncology department, demographic features of the patients; diagnostic methods and treatment protocols; survival and prognostic factors correlate with the literature. Early diagnose and treatment is very important since it effects the survival.