OBJECTIVE: To determine the biologic behavior, risk factors for recurrence, and influence of multimodality therapy on outcome for children and adults with rhabdomyosarcoma of the nose and paranasal sinuses.
DESIGN: Retrospective review of medical records.
PATIENTS: During a 36-year period 37 patients (median age, 23.0 years) with histologically confirmed rhabdomyosarcoma of the nose and paranasal sinuses were treated for cure. Median follow-up was 28 months (range, 1 to 260 months). Histologic subtypes included embryonal, 16; alveolar, 15; mixed, 1; and unclassified, 5. Cervical metastases were present in 38%. Patients were treated with surgery, 4; radiotherapy, 3; chemotherapy, 3; surgery and radiotherapy, 1; surgery and chemotherapy, 1; chemotherapy and radiotherapy, 24; and chemotherapy, radiotherapy, and surgery, 1.
RESULTS: The overall 5-year survival was 44%. For patients treated with chemotherapy and radiotherapy or chemotherapy, radiotherapy, and surgery, the 5-year survival was 60%, compared with 19% for patients treated with the other forms of therapy. Factors associated with poorer survival were adult onset of disease, alveolar histology, and treatment with systemic chemotherapy for less than 1 year. Patients receiving chemotherapy for greater than 1 year had a 5-year survival of 82%, compared with 71 % for those with less than 1 year of treatment. Improved survival was associated with a lower incidence of distant metastasis.
CONCLUSIONS: A combination of chemotherapy and radiotherapy may provide the best means of obtaining local-regional control for rhabdomyosarcoma arising in the nose and paranasal sinuses. The risk of regional disease is high, requiring comprehensive radiotherapy to the neck in addition to the primary site. Surgical resection should be reserved for patients with residual disease after chemotherapy and radiotherapy, Administration of chemotherapy for more than 1 year is associated with improved survival because of a decreased incidence of metastatic disease.
Get full access to this article
View all access options for this article.
References
1.
MaurerHMBeltangadyMGehanEA. The intergroup rhabdomyosarcoma study I: a final reportCancer1988;61:209–20
2.
HarmerMH, ed. TNM classification of pediatric tumors.Geneva: Union Against Cancer, 1982:23–8
3.
KaplanELMeierP. Nonparametric estimations from incomplete observationsJ Am Stat Assoc1958;53:457–581
4.
PetoRPetoJ. Asymptotically efficient rank invariant proceduresJournal of the Royal Statistical Society (Series A)1972;135:185–207
5.
MacArthurCJMcGillTJHealyGB. Pediatric head and neck rhabdomyosarcomaClin Pediatr1992;31:66–70
6.
AndersonGJTomLWWomerRBHandlerSDWetmoreRFPotsicWP. Rhabdomyosarcoma of the head and neck in childrenArch Otolaryngol Head Neck Surg1990;116:428–31
7.
McGillT. Rhabdomyosarcoma of the head and neck: an updateOtolaryngol Clin North Am1989;22:631–6
8.
NewmanANRiceDH. Rhabdomyosarcoma of the head and neckLaryngoscope1984;94:234–9
9.
NakhlehRESwansonPEDehnerLP. Juvenile (embryonal and alveolar) rhabdomyosarcoma of the head and neck in adults: a clinical, pathologic, and immunohistochemical study of 12 casesCancer1991;67:1019–24
10.
LloydRVHajduSIKnapperWH. Embryonal rhabdomyosarcoma in adultsCancer1983;51:557–65
11.
PrestidgeBRDonaldsonSS. Treatment results of adults with childhood tumors: a 20 year experienceInt J Radiat Oncol Biol Phys1989;17:507–14
12.
NewtonWASouleEHHamoudiAB. Histopathology of childhood sarcomas, intergroup rhabdomyosarcoma studies I and II: clinicopathological correlationJ Clin Oncol1988;6:67–75
13.
ShimadaHNewtonWASouleEH. Pathology of fatal rhabdomyosarcoma. Report from intergroup rhabdomyosarcoma study (IRS-I and IRS-II)Cancer1987;59:459–65
14.
DonaldsonSS. Rhabdomyosarcoma: contemporary status and future directionsArch Surg1989;124:1015–20
15.
LawrenceWHaysDMMoonTR. Lymphatic metastasis with childhood rhabdomyosarcomaCancer1977;39:556–9
16.
MaurerHMMoonTRDonaldsonM. The intergroup rhabdomyosarcoma study: a preliminary reportCancer1977;40:2015–26
17.
SchullerDELawrenceTLNewtonWA. Childhood rhabdomyosarcoma of the head and neckArch Otolaryngol1978;105:689–94
18.
DonaldsonSSCastroJRWilburJR. Rhabdomyosarcoma of the head and neck in children: combination therapy by surgery, irradiation, and chemotherapyCancer1973;32:26–35
19.
MansonJKSouleEH. Embryonal rhabdomyosarcoma of the head and neck: report of eighty-eight casesAm J Surg1965;110:585–91
20.
FernandezCHSutowWWMerinoGR. Childhood rhabdo-myosarcoma: analysis of coordinated therapy and resultsAm J Roentgenol Radium Ther Nucl Med1975;123:588–97
21.
LiebnerEJ. Embryonal rhabdomyosarcoma of the head and neck in children: correlation of stage, radiation dose, local control and survivalCancer1976;37:2777–86
22.
PedrickTJDonaldsonSSCoxRS. Rhabdomyosarcoma: the Stanford experience using a TNM staging systemJ Clin Oncol1986;4:370–8
23.
WilburJR. Combination chemotherapy for embryonal rhabdomyosarcomaCancer Chemotherapy Reports1974;58:281–4
24.
HealyGBUptonJBlackPMFerraroN. The role of surgery in rhabdomyosarcoma of the head and neck in childrenArch Otolaryngol Head Neck Surg1991;117:1185–8
25.
BonillaJAHealyGB. Management of malignant head and neck tumors in childrenPediatr Clin North Am1989;36:1443–50
