Osteosarcoma of the sphenoid sinus extending to ethmoid sinus—report of a rare case with review of literature giving special emphasis on treatment and outcome

Abstract

Abstract Background Osteosarcoma usually arises from primitive bone-forming mesenchymal cells. Metaphyseal growth plates of long bones are the usually affected site. Though it is the most common primary bone malignancy, only 6–10% of osteosarcomas are craniofacial. In the sinonasal region, the commonly involved sites reported in the literature are maxillary and ethmoid sinus. Sphenoid sinus has been the least commonly involved site among the paranasal sinuses and only a handful of cases have been reported. Methods A rare case of osteosarcoma involving the sphenoid and ethmoid sinus was presented to our institute and he was managed surgically and with postoperative chemotherapy. Further, an internet-based literature search was conducted to detect cases of osteosarcoma involving sphenoid sinus only as well as combined sphenoid and ethmoid sinus. Results About 13 articles (13 patients), which fulfilled our inclusion and exclusion criteria were included in our study. Out of the 14 patients (including the present case), 5 patients (100%) who underwent both modes of adjuvant therapy were alive during the final follow-up. On the other hand, among those who received single adjuvant therapy, only one patient was alive (33.3%) and two patients died (66.6%). Conclusion Osteosarcomas are highly malignant primary bone neoplasms with rare occurrence in the head and neck region. It constitutes a therapeutic challenge because of its anatomical location and the known side effects of chemotherapy. Timely intervention with a multidisciplinary approach is necessary to avoid death in these patients.

Keywords

• Osteosarcoma
• Osteogenic sarcoma
• Sphenoid sinus
• Ethmoid sinus
• Sinonasal