Chemorefractory rhabdomyosarcoma treated with radiotherapy, bevacizumab, statins and surgery and maintenance with bevacizumab and chemotherapy.

BACKGROUND: Rhabdomyosarcoma is a rare disease in children and young adults, usually responsive to chemotherapy. Here we report on a patient with chemorefractory disease, treated in an unconventional approach.

CASE REPORT: A young woman presented with an embryonal rhabdomyosarcoma of the retromaxillary space. She exhibited progressive disease to two chemotherapy regimens. The patient started hyperfractionated radiotherapy that due to lack of response was changed to 3 Gy fractions once daily to a total absorbed dose of 53 Gy. The therapeutic predicament led us to add bevacizumab and hydroxymethylglutaryl-CoA reductase inhibitors (statins), for which some experimental support could be found.

RESULTS: The tumour responded clearly, and a radical R0 resection followed. The patient was treated postoperatively with bevacizumab and chemotherapy. The combined treatment was accompanied by side effects such as mucositis grade IV, impaired healing of the mandibulotomy as well as a prominent cystitis. Side effects subsided following treatment with hyperbaric oxygen.

CONCLUSION: Rhabdomyosarcoma refractory to chemotherapy may respond to radiotherapy and the addition of bevacizumab and statins. Although the contribution of the adjuncts is unclear, the unexpected tumour control in this case may help in decision-making in similar cases and may encourage the investigation of these adjuncts in protocols.