Multidisciplinary Management of Refractory Orbital Rhabdomyosarcoma

Perioperative Intensity-Modulated Brachytherapy for Refractory Orbital Rhabdomyosarcomas in Children

“…In 1972, the IRSG standardized the classification and treatment of RMS by establishing chemotherapy in optional combination with EBRT as standard treatment, improving the 5-year survival of children with primary orbital, nonparameningeal RMS to > 90% [31].…”

“…Since refractory orbital RMS, accounting for 10-17% of cases [15,31], are bearing a comparably worse prognosis, function-destroying orbital exenteration is still often considered the method of choice in these cases until today [1,31]. Mannor et al [31] reported five children, who had RMS refractory to combined chemotherapy and radiation therapy, undergoing orbital exenteration.…”

Orbital rhabdomyosarcoma

“…VAC has been the gold standard for combination chemotherapy in the treatment of most cases of RMS. 19,20 In our case we did combination therapy, surgical, chemotherapy, and radiotherapy. The surgical approach in this case was exenteration of the bulbi oculi sinistra and continued with chemo- B.…”

“…Localized (group I) and regional resectable (group II) tumors are frequently curable and have a good prognosis). 2,6,18 Mannor et al, 19 reported that more than 90% of children with orbital RMS respond to combination therapy. Crist et al, 20 reported estimated survival at 5 year from the start of treatment was 65-75% in IRS-I (localized disease, completely resected).…”

Orbital rhabdomyosarcoma: A case series

“…The exenteration procedure for such cases of refractory rhabdomyosarcoma was found to be of value in prolonging the survival rate. [15] In conclusion, the primary modality of treatment of rhabdomyosarcoma is combined radiotherapy and chemotherapy, which appears to permit effective control and possible cure of this disease. At the time of biopsy, maximum debulking is essential.…”