A strategy to avoid facial mutilation in orbital embryonal rhabdomyosarcoma

An orbital embryonal rhabdomyosarcoma (RMS) is a rare malignancy in children, but clinical findings are typical. We detail the case of an 8-year-old female with orbital RMS and discuss the therapeutic options. Orbital RMS was apparent with painless exophthalmos of the right globe and diplopia. Head MRI showed tumor masses behind and inferior to the globe. Open biopsy led to the histological diagnosis. Metastasis or infiltration of orbital bone was not observed. Chemotherapy was carried out in accordance with the Cooperative Weichteilsarkom Studie (CWS) 2002 protocol. Tumor regression was detected after the first course of chemotherapy; we decided to excise the residual tumor with preservation of the globe. The CWS 2002 protocol was subsequently completed. Radiotherapy was not done. One year after treatment, RMS recurrence was not observed. After interdisciplinary treatment, mutilation was avoided after exenteration of the orbit or radiation treatment to the growing facial skeleton. That was possible due to excision of the residual tumor in a second step, leading to down-staging of the RMS.