Management of paediatric sinonasal rhabdomyosarcoma

• Published in: Journal of laryngology and otology 2009-09, Vol.123 (9), p.990-996
• Main Authors: Fyrmpas, G, Wurm, J, Athanassiadou, F, Papageorgiou, T, Beck, J-D, Iro, H, Constantinidis, J
• Format: Article
• Language: English
• Subjects: Adolescent; Biological and medical sciences; Biopsy; Bone marrow; Chemotherapy; Chemotherapy, Adjuvant; Child; Child, Preschool; Combined Modality Therapy; Dermatology; Diseases of the osteoarticular system; Endoscopy; Female; Head And Neck; Humans; Lymphatic system; Male; Medical prognosis; Medical sciences; Metastasis; Nose; Nose Neoplasms - diagnosis; Nose Neoplasms - therapy; Ostomy; Otolaryngology; Otorhinolaryngology. Stomatology; Paranasal Sinus Neoplasms; Paranasal Sinus Neoplasms - diagnosis; Paranasal Sinus Neoplasms - therapy; Patients; Pediatrics; Radiation therapy; Radiotherapy, Adjuvant; Remission (Medicine); Retrospective Studies; Rhabdomyosarcoma; Rhabdomyosarcoma - diagnosis; Rhabdomyosarcoma - therapy; Sarcoma; Sinuses; Surgery; Survival Rate; Treatment Outcome; Tumors; Tumors of striated muscle and skeleton; Tumors of the skin and soft tissue. Premalignant lesions
• ISSN: 0022-2151; 1748-5460
• DOI: 10.1017/S0022215109005337

Rhabdomyosarcoma is the commonest malignant tumour of the nose and paranasal sinuses in the paediatric population. Due to its rarity and largely unknown biological behaviour, the treatment of this tumour is complex and controversial. We present the results of multimodality treatment of paediatric sinonasal rhabdomyosarcoma, and we explore the role of surgery in the management of this malignancy. We retrospectively reviewed the records of 14 patients (median age 7.5 years) with sinonasal rhabdomyosarcoma. Six patients underwent major surgery with post-operative chemoradiation. Eight patients received multi-agent chemotherapy and radiotherapy. The mean follow-up time was 58 months (range seven to 276 months). The five-year overall survival rates for all patients and for the surgery group were 53.9 and 83.3 per cent, respectively. All patients with alveolar rhabdomyosarcoma had a poor prognosis, with a median survival time of 17 months. Intracranial extension and an age greater than 10 years were also associated with an unfavourable outcome. Non- or partial responders to initial chemoradiation died within a year of diagnosis. Management of paediatric rhabdomyosarcoma requires a combination of chemotherapy, radiotherapy and surgery. Primary chemoradiotherapy is the established treatment approach for advanced tumours. Early stage tumours with favourable histology can be treated successfully with radical surgery, provided that function and cosmetic appearance are preserved.