Desmoplastic fibroma of the maxilla in a 5-year-old patient with Tuberous Sclerosis Complex (TSC): Case report and review of the literature

The following case report details the presentation of a left maxillary painless expansile lesion in a five-year-old female that was proven to be desmoplastic fibroma (DF) of the maxilla, which was treated via a conservative excision. Given the paucity of DF cases in the Maxillofacial literature, there are no formally agreed-upon guidelines for the treatment of DF, especially in the maxillary sinus. A thorough review of the literature was completed and discussed, highlighting the correlation of DF with Tuberous Sclerosis Complex (TSC) and the proposed treatment when encountered in the maxillary sinus of a pediatric patient. • Many surgical options have been suggested for maxillofacial DF, without agreed upon consensus. • Maxillofacial DFs are rare tumors with fewer than 160 reported in the English literature. • Although benign, this tumor can grow rapidly and be destructive, complicating treatment. • Maxillofacial DFs may be a manifestation of Tuberous Sclerosis Complex (TSC). • In this case we propose a conservative excision of DF in the maxillary sinus.