Slovenia, a new country with 2 million inhabitants, including 350,000 children, was formerly part of Yugoslavia. It has only one pediatric cancer center situated in Ljubljana that takes care of approximately 60 children with cancer per year. All patients are subject to regular follow up indefinitely. The purpose of our study was to evaluate the survival of children treated for rhabdomyosarcoma (RMS) in Slovenia during a 23 year period. Neoadjuvant chemotherapy (Cht) was introduced during that interval [1,2].
Fifty one children with RMS, 27 boys and 24 girls, 1-15 years old, were managed between 1974 and 1996. For the purpose of this study, we assigned 21 treated between 1974-1985 to group A, and 30 treated between 1986-1996 to Group B. The distribution by stage, tumor location, and histology was similar for both groups. Patients were followed between 5 and 26 years (median 12), none were lost to follow up.
Tumors were staged according to the SIOP-UICC [3] and in the 43 histologically confirmed cases the types of RMS were embryonal 24, alveolar 14, botryoid 1, spindle cell 1, and sarcoma NOS 3.
Multidrug Cht varied during the study period and included vincristine, dactinomycin, cyclophosphamide, doxorubicin, and ifosfamide in standard multidrug regimens. Forty-eight patients with Stages I-III received Cht, 26 as neoadjuvant, and 20 after surgery. Cht was combined with irradiation (RT) in 40 patients. Two of the three Stage IV patients had bone marrow transplant (ABMT) at first treatment; the third had half body RT in combination with Cht and RT to all involved areas. Treatment was started with Cht in orbital, head and neck and in most with genitourinary RMS; surgery was first in paratesticular and in most extremity RMS cases.
Forty patients received RT to primary or metastatic sites (including the two who had ABMT). RT was given at first treatment only in two patients from Group A with maxillary tumors, while in 24 it was given after Cht and in 14 after surgery. The dose of RT varied between 30 and 50 Gy, given concomitantly with four or more drug combination Cht.