Résumé
Sinonasal sarcomas are a heterogeneous group of rare tumors that are poorly documented and whose prognostic factors are not clearly established.
This was a retrospective multicenter observational study using the national REFCOR database to analyze the characteristics, management and prognostic factors of patients with sinonasal sarcomas among adolescents and adults. Overall survival and disease-free survival were assessed. The influence of age, gender, TNM classification, histological subtype, grade and treatment modality was calculated using Log-Rank tests and Cox proportional hazard models for multivariate analyses.
118 cases of sinonasal sarcomas were analyzed between 2010 and 2020. Median age was 50 years. Four histological subtypes predominated: rhabdomyosarcoma (21.2 %), chondrosarcoma (19.5 %), osteosarcoma (14.4 %) and biphenotypic sarcoma (9.3 %). Overall survival rates at 1, 3 and 5 years were respectively 99 %, 81.2 % and 68.8 %, and disease-free survival rates 96 %, 73.5 % and 58.3 %. Overall and disease-free survival of sinonasal sarcomas were significantly different by age (p < 0.001), gender (p < 0.001), histological subtype (p = 0.00062, with rhabdomyosarcomas and biphenotypic sarcomas being the most and least aggressive types, respectively), lymph node status (p = 0.05 and p < 0.0001), metastatic status (p = 0.004 and p < 10−4), multimodal treatment protocol (p < 10−4) including surgical resection (p < 10−4). Surgery by endoscopic endonasal approaches yielded similar or better outcomes compared to open surgery.
Age, histological subtype, N+ and M+ status at diagnosis and therapeutic management influenced survival in sinonasal sarcomas. Survival rates varied widely according to histological subtype, and not all sarcomas have a poor prognosis.
[Display omitted]
•Sinonasal sarcomas are a rare heterogeneous group of tumors with limited clinical data.•Rhabdomyosarcoma, chondrosarcoma, osteosarcoma and biphenotypic sarcoma were the dominant subgroup among 50 histological sub-types in this series.•Age, gender, histology, N+ and M+ status at diagnosis, treatment protocol and surgical margins influence survival.•Their treatment varies and multimodal management may not always be required (surgery alone being an option in specific scenarii).•Rhabdomyosarcomas were often N+M+ at diagnosis, requiring multimodal management compare to biphenotypic sarcoma N0M0 and treated by surgery alone.