Rhabdomyosarcomas of the nose and paranasal sinuses: treatment results in 15 cases

Abstract

Objective: Primary rhabdomyosarcomas (RMS) of the nose and the paranasal sinuses occur very rarely. Treatment of these tumors usually is conducted according to standardized therapy protocols like the German Cooperative Soft Tissue Sarcoma Study (CWS) or the Intergroup Rhabdomyosarcoma Study (IRS). The role of surgery still remains controversial.

Study design and setting: A retrospective analysis of 15 patients with an RMS of the nose or paranasal sinuses treated between 1979 and 2000 is presented. Patients' age ranged from 2 to 60 years, with an average of 22 years. Histologic subtypes encompassed 9 embryonal (e) and 6 alveolar (a) RMS. Resection of the tumor with subsequent radiochemotherapy (RCT) was performed in 6 cases, and primary RCT, in 9 cases. The average duration of follow-up was 4 years and 10 months.

Results: Overall 5-year survival was 40%. In the patient group subjected to tumor resection with subsequent RCT, 5-year survival was 66%, compared with 33% after exclusive primary RCT. With respect to histologic subtype, 5-year survival was 55% for eRMS, as compared with 33% for aRMS. Moreover, infiltration of the skull base and the presence of a residual tumor after primary therapy constituted factors associated with an unfavorable clinical course.

Conclusions: Surgical removal of tumor with subsequent radiochemotherapy can be recommended if a complete resection and functionally and cosmetically satisfactory results appear possible. Patients with eRMS showed an overall more favorable clinical course than patients with aRMS.