The first guidelines of the French Expertise Network on Rare ENT Cancers (REFCOR) on the management of nasal cavity and sinus cancer date back to 2009. The objective of the present study was to update these guidelines using a search of the literature between 2009 and 2020.

The literature analysis was carried out using PRISMA criteria to enrich the previous literature review; 250 studies were selected out of 1696 analyzed. General considerations were formulated, common to all histologies and specific to each. The formalized expert consensus method was used to revise the old guidelines and propose new ones.

The scientific analysis led to 87 recommendations: common to all sinonasal cancers (1 to 10), and for adenocarcinoma (11 to 28), adenoid cystic carcinoma (29 to 42), squamous cell carcinoma (43 to 53), mucosal melanoma (54 to 66), neuroblastoma (67 to 72) and undifferentiated carcinoma (73 to 87). According to first 10 guidelines, management of sinonasal cancer requires multidisciplinary teamwork, specifically trained teams and multidisciplinary tumor board meetings, and the emergence of new anatomopathological entities requires in-depth histological and immunophenotypic investigation.

The present article provides a short version of the updated review validating the first 10 guidelines for diagnostic and therapeutic action on general themes common to all histologies.