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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: [email protected]
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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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References

1 Gurney, JG, Severson, RK, Davis, S, Robison, LL. Incidence of cancer in children in the United States. Sex-, race-, and 1-year age-specific rates by histologic type. Cancer 1995;75:2186–95Google Scholar
2 SEER Cancer Statistics Review 1975–2002. National Cancer Institute. Bethesda, MD. In: http://seer.cancer.gov/csr/1975_2002/ [13 December 2007]Google Scholar
3 Hicks, J, Flaitz, C. Rhabdomyosarcoma of the head and neck in children. Oral Oncol 2002;38:450–9Google Scholar
4 Lee, KJ. Essential Otolaryngology Head & Neck Surgery, 7th edn. New York: McGraw-Hill, 1999Google Scholar
5 Gurney, JG, Young, JL Jr, Roffers, SD, Smith, MA, Bunin, CR. Chapter IX: Soft tissue sarcomas. In: Ries, LAG, Smith, MA, Gurney, JG, Linet, M, Tamra, T, Young, JL, Bunin, GR, eds. Cancer Incidence and Survival among Children and Adolescents: United States SEER Program 1975–1995, National Cancer Institute, SEER Program, NIH Pub. No. 99-4649. Bethesda, MD, 1999;120Google Scholar
6 Pappo, AS, Shapiro, DN, Crist, WM, Maurer, HM. Biology and therapy of pediatric rhabdomyosarcoma. J Clin Oncol 1995;13:2123–39Google Scholar
7 Wiener, ES. Head and neck rhabdomyosarcoma. Semin Pediatr Surg 1994;3:203–6Google Scholar
8 Lawrence, W Jr, Anderson, JR, Gehan, EA, Maurer, H. Pretreatment TNM staging of childhood rhabdomyosarcoma: a report of the Intergroup Rhabdomyosarcoma Study Group. Cancer 1997;80:1165–70Google Scholar
9 Newman, AN, Rice, DH. Rhabdomyosarcoma of the head and neck. Laryngoscope 1984;94:234–9Google Scholar
10 Sercarz, JA, Mark, RJ, Tran, L, Stopper, I, Calcattera, TC. Sarcomas of the nasal cavity and paranasal sinuses. Ann Otol Rhinol Laryngol 1994;103:699–704Google Scholar
11 Callender, TA, Weber, RS, Janjan, N, Benjamin, R, Zaher, M, Wolf, P et al. Rhabdomyosarcoma of the nose and paranasal sinuses in adults and children. Otolaryngol Head Neck Surg 1995;112:252–7Google Scholar
12 Sercarz, JA, Mark, RJ, Nasri, S, Wang, MB, Tran, LM. Pediatric rhabdomyosarcoma of the head and neck. Int J Pediatr Otorhinolaryngol 1995;31:15–22Google Scholar
13 Leunig, A, Lang, S, Jager, L, Grevers, G. Sarcoma of the nose and paranasal sinuses [in German]. Laryngorhinootologie 1995;74:90–4Google Scholar
14 Kraus, DH, Saenz, NC, Gollamudi, S, Heller, G, Moustakis, M, Gardiner, S et al. Pediatric rhabdomyosarcoma of the head and neck. Am J Surg 1997;174:556–60Google Scholar
15 Hess, A, Schroder, U, Schroder, R, Michel, O. Rhabdomyosarcoma in the area of the head-neck. A synopsis of some cases, therapeutic possibilities and prognoses [in German]. Laryngorhinootologie 1998;77:557–63Google Scholar
16 Herrmann, BW, Sotelo-Avila, C, Eisenbeis, JF. Pediatric sinonasal rhabdomyosarcoma: three cases and a review of the literature. Am J Otolaryngol 2003;24:174–80Google Scholar
17 Wurm, J, Constantinidis, J, Grabenbauer, G, Iro, H. Rhabdomyosarcomas of the nose and paranasal sinuses: treatment results in 15 cases. Otolaryngol Head Neck Surg 2005;133:42–50Google Scholar
18 Harnsberger, H, Hudgins, P, Wiggins, R 3rd, Davidson, H. PocketRadiologist. Head and Neck, 1st edn. Salt Lake City, Utah: Amirsys, 2002Google Scholar
19 Yousem, DM, Lexa, FJ, Bilaniuk, LT, Zimmerman, RI. Rhabdomyosarcomas in the head and neck: MR imaging evaluation. Radiology 1990;177:683–6Google Scholar
20 Heffner, DK. Prognostic factors in head and neck rhabdomyosarcoma. Head Neck 2003;25:416–17Google Scholar
21 Newton, WA Jr, Gehan, EA, Webber, BL, Marsden, HB, van Unnik, AJM, Hamoudi, AB et al. Classification of rhabdomyosarcoma and related sarcomas: pathologic aspects and proposal for a new classification—an Intergroup Rhabdomyosarcoma Study. Cancer 1995;76:1073–85Google Scholar
22 Raney, RB, Meza, J, Anderson, JR, Fryer, CJ, Donaldson, SS, Breneman, JC et al. Treatment of children and adolescents with localized parameningeal sarcoma: experience of the Intergroup Rhabdomyosarcoma Study Group protocols IRS II through IV, 1978–1997. Med Pediatr Oncol 2002;38:22–32CrossRefGoogle Scholar
23 Stevens, MC, Rey, A, Bouvet, N, Ellershaw, C, Flamant, F, Habrand, JL et al. Treatment of nonmetastatic rhabdomyosarcoma in childhood and adolescence: third study of the International Society of Paediatric Oncology – SIOP Malignant Mesenchymal Tumor 89. J Clin Oncol 2005;23:2618–28Google Scholar
24 Donaldson, SS, Anderson, JR. Rhabdomyosarcoma: many similarities, a few philosophical differences. J Clin Oncol 2005;23:2586–7Google Scholar
25 Koscielniak, E, Harms, D, Henze, G, Jürgens, H, Gadner, H, Herbst, M et al. Results of treatment for soft tissue sarcoma in childhood and adolescence: a final report of the German Cooperative Soft Tissue Sarcoma Study CWS-86. J Clin Oncol 1999;17:3706–19Google Scholar
26 Raney, RB, Asmar, L, Vassilopoulou-Sellin, R, Klein, MJ, Donaldson, SS, Green, J et al. Late complications of therapy in 213 children with localized, nonorbital soft-tissue sarcoma of the head and neck: a descriptive report from the Intergroup Rhabdomyosarcoma Studies (IRS)-II and - III. IRS Group of the Children's Cancer Group and the Pediatric Oncology Group. Med Pediatr Oncol 1999;33:362–71Google Scholar
27 Paulino, AC, Simon, JH, Zhen, W, Wen, BC. Long-term effects in children treated with radiotherapy for head and neck rhabdomyosarcoma. Int J Radiat Oncol Biol Phys 2000;48:1489–95Google Scholar
28 Anderson, GJ, Tom, LW, Womer, RB, Handler, SD, Wetmore, RF, Potsic, WP. Rhabdomyosarcoma of the head and neck in children. Arch Otolaryngol Head Neck Surg 1990;116:428–31Google Scholar
29 Healy, GB, Upton, J, Black, PM, Ferraro, N. The role of surgery in rhabdomyosarcoma of the head and neck in children. Arch Otolaryngol Head Neck Surg 1991;117:1185–8Google Scholar
30 Daya, H, Chan, HS, Sirkin, W, Forte, V. Pediatric rhabdomyosarcoma of the head and neck: is there a place for surgical management? Arch Otolaryngol Head Neck Surg 2000;126:468–72Google Scholar
31 Blatt, J, Snyderman, C, Wollman, MR, Mirro, J Jr, Janecka, IP, Albo, VC et al. Delayed resection of non-orbital rhabdomyosarcoma of the head and neck in childhood. Med Pediatr Oncol 1997;29:294–8Google Scholar
32 Buwalda, J, Schouwenburg, PF, Blank, LE, Merks, JH, Copper, MP, Strackee, SD et al. A novel treatment strategy for advanced stage head and neck rhabdomyosarcomas in children: results of the AMORE protocol. Eur J Cancer 2003;39:1594–602Google Scholar
33 Raney, RB, Anderson, JR, Barr, FG, Donaldson, SS, Pappo, AS, Qualman, SJ et al. Rhabdomyosarcoma and undifferentiated sarcoma in the first two decades of life: a selective review of intergroup rhabdomyosarcoma study group experience and rationale for Intergroup Rhabdomyosarcoma Study V. J Pediatr Hematol Oncol 2001;23:215–20Google Scholar
34 Paulino, AC, Bauman, N, Simon, JH, Nguyen, TX, Ritchie, JM, Tannous, R. Local control of parameningeal rhabdomyosarcoma: outcomes in non-complete responders to chemoradiation. Med Pediatr Oncol 2003;41:118–22Google Scholar
35 Buwalda, J, Blank, LE, Schouwenburg, PF, Copper, MP, Strackee, SD, Voûte, PA et al. The AMORE protocol as salvage treatment for non-orbital head and neck rhabdomyosarcoma in children. Eur J Surg Oncol 2004;30:884–92CrossRefGoogle Scholar