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Management of paediatric sinonasal rhabdomyosarcoma

Published online by Cambridge University Press:  27 April 2009

G Fyrmpas ,
J Wurm ,
F Athanassiadou ,
T Papageorgiou ,
J-D Beck ,
H Iro  and
J Constantinidis
G Fyrmpas*
Affiliation:
Department of Otolaryngology Head and Neck Surgery, Aristotle University of Thessaloniki, AHEPA Hospital, Kiriakidi, Thessaloniki, Greece
J Wurm
Affiliation:
Department of Otorhinolaryngology, Head and Neck Surgery, University of Erlangen Nuremberg, Erlangen, Germany
F Athanassiadou
Affiliation:
Department of Pediatrics, Aristotle University of Thessaloniki, AHEPA Hospital, Kiriakidi, Thessaloniki, Greece
T Papageorgiou
Affiliation:
Department of Pediatrics, Aristotle University of Thessaloniki, AHEPA Hospital, Kiriakidi, Thessaloniki, Greece
J-D Beck
Affiliation:
Department of Pediatrics, University of Erlangen Nuremberg, Erlangen, Germany
H Iro
Affiliation:
Department of Otorhinolaryngology, Head and Neck Surgery, University of Erlangen Nuremberg, Erlangen, Germany
J Constantinidis
Affiliation:
Department of Otolaryngology Head and Neck Surgery, Aristotle University of Thessaloniki, AHEPA Hospital, Kiriakidi, Thessaloniki, Greece
*
Address for correspondence: Dr Georgios Fyrmpas, Department of Otolaryngology Head and Neck Surgery, Aristotle University of Thessaloniki, AHEPA Hospital, Kiriakidi 1, 546 36 Thessaloniki, Greece. Fax: 0030 2310994916 E-mail: drfirbas@hotmail.com
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Abstract

Background and aim:

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.

Methods:

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).

Results:

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.

Conclusions:

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.

Keywords

RhabdomyosarcomaChildParanasal Sinus NeoplasmsHead And Neck
Type
Main Articles
Information
The Journal of Laryngology & Otology , Volume 123 , Issue 9 , September 2009 , pp. 990 - 996
Copyright
Copyright © JLO (1984) Limited 2009

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