Sinonasal Osteosarcoma: Report of 14 New Cases and Systematic Review of the Literature

Abstract

Objective The objective of this study is to describe the clinical presentation, tumor characteristics, natural history, and treatment patterns of sinonasal osteosarcoma. Methods Fourteen patients who had been treated for osteosarcoma of the nasal cavity and paranasal sinuses at a tertiary care center were reviewed. In addition, a systematic review of the literature for osteosarcoma of the sinonasal cavity was performed. Results In a systematic review, including 14 patients from the authors' institution, 53 total studies including 88 patients were assessed. Median follow-up was 18 months (interquartile range: 8–39 months). The most common presenting symptoms were facial mass or swelling (34%), and nasal obstruction (30%). The most common paranasal sinus involved by tumor was the maxillary sinus (64%), followed by the ethmoid sinuses (52%). The orbit (33%), dura (13%) and infratemporal fossa (10%) were the most common sites of local invasion. The majority of patients underwent surgery followed by adjuvant therapy (52.4%). Increasing age was associated with decreased overall survival rate (unit risk ratio [95% confidence interval (CI)] = 1.02 [1.003–1.043]; p = 0.0216) and T4 disease was associated with decreased disease-specific survival rate (hazard ratio [HR] = 2.87; p = 0.0495). The 2- and 5-year overall survival rates were 68 and 40%, respectively, while 2- and 5-year disease-specific survival rates were 71% and 44%, respectively. Conclusions Sinonasal osteosarcomas are uncommon tumors and can pose a significant therapeutic challenge. Increasing age and T4 disease are associated with worse prognosis. This disease usually warrants consultation by a multidisciplinary team and consideration of multimodality therapy.

Poorly differentiated sarcoma of the maxillary sinus: a histopathology dilemma of a rare tumor

“…Most cases, related to different types of head and neck sarcomas, primarily have been treated by surgical resection and adjuvant therapy. A small number of cases were treated only with radiotherapy and chemotherapy [ 3 , 5–9 ]. In our case, neoadjuvant therapy stopped aggressive tumor growth but did not significantly reduce the tumor mass.…”

“…Non-randomized chromosomal translocations that lead to the formation of oncogenic proteins, telomere and tumor suppressor genes dysfunction underlie in the development of sarcoma [ 2 ]. Paranasal sinuses sarcomas are extremely rare, including data on the involvement of a single paranasal sinus, as the literature is limited to sporadic cases and smaller series of individual subtypes [ 3 ].…”

International Consensus Statement on Allergy and Rhinology: Sinonasal Tumors

“…Nineteen articles with sufficient information on sinonasal osteosarcomas have been identified (Table XXIII.B.2). 1266–1283 Overall, 310 patients were included. In 10 studies, osteosarcoma of the superior craniofacial area and mandible were included together.…”

“…The following negative prognostic factors were identified: male gender for RT‐associated osteosarcomas, 1270 female gender for primary osteosarcomas, 1275 post‐RT presentation, 1283 high/intermediate grade, 1273,1276,1283,1284 nonmaxillary sinonasal/skull base localization, 1273 microscopic margin involvement, 1273,1276,1277,1279,1280,1283 R2 surgery, 1272 and low‐to‐null neoplastic bone formation at imaging 1270 . The prognostic role of age is debated 1276,1282 …”

Chondroblastic osteosarcoma of the nasal cavity: an exceptional and misdiagnosed presentation

“…Osteosarcoma (OS) arises from primitive bone-forming mesenchymal cells [ 1 ]. As the most common primary bone malignancy, it exhibits two age distribution peaks and typically occurs in the metaphyseal growth plates of long bones [ 1 ].…”