Background: Biphenotypic sinonasal sarcoma (BSNS) is a low-grade tumor of the sinonasal tract with frequent extension to the orbit and skull base. Due to its rare incidence and recent histopathological and molecular characterization, little data are available in regard to its natural history, treatment and surveillance protocol. Methods: A comprehensive literature review in Embase online electronic databases on BSNS was made. The analyzed factors included the patients’ sex and age, presenting symptoms and signs, anatomical origin and pattern of growth of the tumor, immunohistochemical and molecular features, time to treatment, type of treatment, surgical approach, extent of resection, peri- and post-operative complications, adjuvant therapies, clinical outcome, recurrence and overall survival rates. Results: This literature review involved 34 studies for an overall series of 149 cases of BSNS. The female (66.9%) and middle-aged populations (median 54.88 years old) were mainly affected. The most frequent clinical onset was nasal obstruction (81%), followed by facial discomfort (44%), epistaxis (15.5%) and ocular impairment (14.3%). Ethmoid sinus (67.8%) and nasal cavity (45.4%) were the most common anatomical site of tumor origin, while an extension to the orbit and skull base was registered in 28.7% and 24.5% of cases. Surgery was the main treatment, especially in the form of endoscopic endonasal approach (56.9%), and allowed for gross total resection in 79% of cases. The recurrence rate was 26.2%; three cases of tumor-related death were reported. Median follow-up was 4.6 years. Conclusions: Biphenotypic sinonasal sarcoma is a rare and unique tumoral entity in terms of biological and clinical behavior. Based on the current knowledge, surgery plays the leading role in treatment, accounting for gross total tumor resection in most cases, allowing for clinical symptom and sign resolution and presenting a low rate of perioperative complications. The type of approach and the aim of surgery should be assessed case by case according to patient and pathology features and the surgeon’s experience, as well as the aim of the treatment. Further studies including large surgical series and with long follow-up are required to define prognostic factors and guidelines of treatment for this peculiar pathological entity.
背景：双表型鼻窦肉瘤（BSNS）是一种低度恶性的鼻窦道肿瘤，常向眼眶和颅底扩展。由于其罕见发病率及近期才明确的组织病理学和分子特征，关于其自然病程、治疗及监测方案的数据较少。 方法：在Embase在线电子数据库中进行了关于BSNS的全面文献综述。分析因素包括患者性别与年龄、临床表现症状与体征、肿瘤的解剖起源与生长模式、免疫组化与分子特征、治疗时间、治疗类型、手术入路、切除范围、围手术期及术后并发症、辅助治疗、临床结局、复发率及总生存率。 结果：本综述共纳入34项研究，总计149例BSNS病例。主要受累人群为女性（66.9%）及中年群体（中位年龄54.88岁）。最常见的临床表现为鼻塞（81%），其次为面部不适（44%）、鼻出血（15.5%）和眼部功能障碍（14.3%）。筛窦（67.8%）和鼻腔（45.4%）是最常见的肿瘤解剖起源部位，而肿瘤扩展至眼眶和颅底的比例分别为28.7%和24.5%。手术是主要治疗方式，尤其以内镜经鼻入路为主（56.9%），79%的病例实现了大体全切除。复发率为26.2%；报告了3例肿瘤相关死亡。中位随访时间为4.6年。 结论：双表型鼻窦肉瘤在生物学行为和临床表现上是一种罕见且独特的肿瘤实体。基于现有认知，手术在治疗中起主导作用，在大多数病例中可实现肿瘤大体全切除，有效缓解临床症状与体征，且围手术期并发症发生率较低。手术入路选择及手术目标应根据患者与病理特征、外科医生经验以及治疗目的进行个体化评估。未来需要纳入大规模手术病例及长期随访的研究，以明确这一特殊病理实体的预后因素和治疗指南。
肿瘤(癌症)患者之家