Low Christopher M, Gruszczynski Nelson R, Moore Eric J, Price Daniel L, Janus Jeffrey R, Kasperbauer Jan L, Van Abel Kathryn M, Stokken Janalee K, Van Gompel Jamie J, Link Michael J, Choby Garret
Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota, United States.
Mayo Clinic Alix School of Medicine, Mayo Clinic, Rochester, Minnesota, United States.
J Neurol Surg B Skull Base. 2021 Jul;82(Suppl 3):e138-e147. doi: 10.1055/s-0040-1701221. Epub 2020 Jan 24.
The objective of this study is to describe the clinical presentation, tumor characteristics, natural history, and treatment patterns of sinonasal osteosarcoma. 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. 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]; = 0.0216) and T4 disease was associated with decreased disease-specific survival rate (hazard ratio [HR] = 2.87; = 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. 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.
本研究的目的是描述鼻窦骨肉瘤的临床表现、肿瘤特征、自然病史和治疗模式。对在一家三级医疗中心接受鼻腔和鼻窦骨肉瘤治疗的14例患者进行了回顾。此外，还对鼻窦骨肉瘤的文献进行了系统回顾。在一项系统回顾中，包括作者所在机构的14例患者，共评估了53项研究，涉及88例患者。中位随访时间为18个月（四分位间距：8 - 39个月）。最常见的首发症状是面部肿块或肿胀（34%）和鼻塞（30%）。肿瘤最常累及的鼻窦是上颌窦（64%），其次是筛窦（52%）。眼眶（33%）、硬脑膜（13%）和颞下窝（10%）是最常见的局部侵犯部位。大多数患者接受了手术，随后进行辅助治疗（52.4%）。年龄增加与总生存率降低相关（单位风险比[95%置信区间（CI）]=1.02[1.003 - 1.043]；P = 0.0216），T4期疾病与疾病特异性生存率降低相关（风险比[HR]=2.87；P = 0.0495）。2年和5年总生存率分别为68%和40%，而2年和5年疾病特异性生存率分别为71%和44%。鼻窦骨肉瘤是罕见肿瘤，可带来重大治疗挑战。年龄增加和T4期疾病与预后较差相关。这种疾病通常需要多学科团队会诊并考虑多模式治疗。
