Endoscopic transnasal ablation of a large fronto-ethmoidal sarcoma

Biphenotypic sinonasal sarcoma is a rare tumor (149 cases described so far in the literature) that arises from the mucosa of the upper respiratory tract, and it is characterized by aggressive biological behavior and a marked tendency to invade the skull base and the orbit. According to current knowledge, surgery represents the gold standard for treatment. The role of adjuvant therapies remains unclear. The particularity of the presented case: the tumor had its insertion point in the right frontal sinus, and from this level, it invaded both ethmoidal sinuses through partial destruction of the frontal intersinusal wall and dislocation of the structures in the bilateral nasofrontal canal. Although we were dealing with a large and highly vascularized tumor, with a clear indication for external surgery (at least through the supraorbital approach), efficient preoperative embolization and the use of angled endoscopic instruments, under the control of 70° and 90° endoscopes, made it possible to achieve a grossly complete ablation through an exclusive endoscopic transnasal approach of the Draf III type (modified Lothrop procedure). Further studies are needed, including large surgical series and long-term follow-up, to define prognostic factors and establish clear therapeutic guidelines for this rare and peculiar pathological entity.