Orbital rhabdomyosarcoma in children

Abstract

Purpose. To analyze the results of treatment and active monitoring the children with orbital rhabdomyosarcoma (RMS) with an emphasis on vital prognosis. Material and methods. We examined 32 children with RMS (18 boys, 14 girls) aged 2 months to 12 years, whose case history ranged from 1 week to 16 weeks (median, 5 weeks). The median follow-up was 60 months. The tumor was localized in the upper (n = 13), upper internal (n = 9), lower (n = 4), internal (n = 3), and external (n = 3) quadrants. The patients underwent primary surgical treatment: orbitotomy using transcutaneous (n = 26), transconjunctival (n = 2) and subperiosteal access (n = 4) followed by cytological, histological and immunohistochemical tissue verification in all cases. Results. The highest number of RMS cases was noted in in the 2- to 7-year-old group (66 % of patients). All patients were treated in in-patient settings. Complete macroscopic removal of tumor was performed in 17 cases, partial removal in 9 cases, and biopsy sampling in 6 cases. The embryonic type of RMS accounted for 87 % (n = 28), and the alveolar type, for 13 % (n = 6). In the postoperative period all children received combined therapy (systemic polychemotherapy and distant radiation therapy) in in-patient facilities of special oncological and radiological clinics. The survival rate of the whole group was 100%. In the long-term follow-up period (after 3 to 5 years), reconstructive surgeries were performed to correct ptosis and strabismus, and/or spectacle or prismatic correction of complex or mixed astigmatism. In cases of “dry” eye syndrome, conservative local therapy was offered. After orbital exenteration, ectoprosthesis procedures was applied. Conclusion. The analysis of our observations of 32 children with orbital rhabdomyosarcoma showed a 100 % survival rate, which testifies to the importance of timely diagnosis and combined treatment of the tumor.