alexa Rhabdomyosarcoma of the nose and paranasal sinuses in adults and children.


Otolaryngology: Open Access

Author(s): Callender TA, Weber RS, Janjan N, Benjamin R, Zaher M,

Abstract Share this page

Abstract 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.
This article was published in Otolaryngol Head Neck Surg and referenced in Otolaryngology: Open Access

Relevant Expert PPTs

Relevant Speaker PPTs

Recommended Conferences

Peer Reviewed Journals
Make the best use of Scientific Research and information from our 700 + peer reviewed, Open Access Journals
International Conferences 2017-18
Meet Inspiring Speakers and Experts at our 3000+ Global Annual Meetings

Contact Us

© 2008-2017 OMICS International - Open Access Publisher. Best viewed in Mozilla Firefox | Google Chrome | Above IE 7.0 version