Risk of leptomeningeal metastasis in children with parameningeal rhabdomyosarcoma with intracranial spread

Introduction. Soft tissue tumors account for up to 8 % of all malignant neoplasms in children. According the international histological classification, about 150 different morphological variants soft have been registered, which 45 are rhabdomyosarcoma (RMS). Most often, RMS occurs early childhood – average age patients at time diagnosis is 5 years. The incidence rate 0.9 per 100,000 In 25 cases, initial reveals distant metastasis lungs, bones, bone marrow, and leptomeningeal metastasis. More than 7 with localized parameningeal develop metastasis, according Rhabdomyosarcoma Study Group. Purpose study report incidence, prognosis treatment outcomes children intracranial spread RMS. Materials methods. included aged 1 17 years a localization spread, who received special from 2003 2020. 6 (20 %) whom tumor developed (up 3 years). boys predominated (55.5 study. primary orbit (15 skull base middle ear 4 (8 nasopharynx (6 brain substance was affected 16 (35 %). Metastases regional lymph nodes were determined patients. Multiple metastatic lesions bones marrow (9 metastases 2 (4 (100 drug protocols approved by Academic Council Research Institute Pediatric Oncology Hematology N. Blokhin National Medical Centerof Oncology, Ministry Health Russia. Radiation therapy performed 33 (74 patients, while total focal dose 50 Gy administered tumor. Affected neck irradiated 10 (23 45.0 Gy. Surgical 15 (34 Multicomponent Results. During observation period 12 months 14 years, 21 (49 alive. (51 died progression, (2 complications treatment. Conclusions. this group one urgent problems pediatric oncology, based on polychemotherapy, supplemented intrathecal administration anticancer drugs craniospinal irradiation. development new therapies requires multidisciplinary approach achieve significant improvement survival localization.