Adult sinonasal soft tissue sarcoma: analysis of 48 cases from the French Sarcoma Group database

Abstract

Objective: The aim of this study was to determine the frequency of primary sinonasal adult sarcoma, identify histological subtypes, and analyze prognostic factors.

Study design: Retrospective review.

Method: Forty-eight adult sinonasal sarcomas included in the French Sarcoma Group database (Conticabase) were reviewed.

Results: The most frequent tumor types were alveolar rhabdomyosarcoma (33.3%), embryonal rhabdomyosarcoma (14,6%), unclassified sarcoma (14.6%), and leiomyosarcoma (12.5%). All round cell tumors were rhabdomyosarcomas. The 5-year overall survival (OS), metastasis-free survival (MFS), and local recurrence-free survival (LRFS) rates were 62.3%, 73%, and 88.8%, respectively. Histotype was a prognostic factor for OS, MFS, and LRFS, with the worst prognosis associated with rhabdomyosarcomas, regardless of the subtype. The tumor grade influenced the OS and MFS. Surgery was a predictive factor for a complete response.

Conclusions: These results suggest that sinonasal tract should be considered as an unfavorable site for rhabdomyosarcoma. Moreover, surgery should always be considered in treatment.

Keywords: adults; prognosis; rhabdomyosarcoma; sinonasal tract; soft tissue sarcoma.