Para-meningeal rhabdomyosarcoma with critical airway compromise: Role of endoscopic debulking surgery

Summary 

Paediatric para-meningeal rhabdomyosarcomas (RMSs) are treated with aggressive chemo-radiotherapy; so far, surgery has played a limited role in the management owing to the relative inaccessibility of lesion and the associated morbidity.

We present the management of two cases of para-meningeal embryonal RMS at our institution from 2005 to 2006, with critical airway compromise on presentation. Tumour stage and site were identical and both patients received chemo-radiotherapy based on the Malignant Mesenchymal Tumour Group MMT 953A protocol. In addition, one patient underwent debulking surgery to establish airway patency using trans-nasal endoscopic technique allowing a rapid return to normal activity. The second patient had higher morbidity and longer hospital stay due to prolonged intubation. We discuss the role of endoscopic surgery in the management of paediatric head and neck RMS.

Keywords: Rhabdomyosarcoma, Head and neck rhabdomyosarcoma, Paediatric, Para-meningeal, Endoscopic debulking