Background:Biphenotypic sinonasal sarcoma is a rare low-grade tumor arising from the sinonasal tract, featuring locally aggressive biological behavior, with a tendency to invade the orbit and skull base. There are no defined guidelines of treatment; thus, the management varies among different institutions. The aim of the present study is to provide a modular system of surgical approaches according to the lesion pattern of growth from a literature review.Materials and Methods:A comprehensive and detailed literature review on the PubMed and Embase online electronic databases on biphenotypic sinonasal sarcoma with orbital invasion was conducted. A personal case exhibiting peculiar features was also added. Demographic (patient’s sex and age), clinical (presenting symptoms and time to treatment), neuroradiological (anatomical origin and pattern of growth), and treatment (type of treatment, surgical approach, extent of resection, peri- and postoperative complications, and adjuvant therapies) data, as well as clinical outcome, recurrence rates, and overall survival, were analyzed.Results:Thirty-one patients harboring biphenotypic sinonasal sarcoma with orbital invasion were identified. Tumors mainly affected female patients (66.7%) and a middle-aged population (median 55.2 years old). Simultaneous skull base involvement occurred in most cases (80.6%). Surgery was performed in all but one case (97%), as unique treatment (59%) or in association with radio—(23.5%) and/or chemotherapy (5.9%/2.9%), allowing for gross total tumor resection in most cases (66.7%). The endoscopic endonasal approach was the most adopted surgical corridor (51.7%). The local recurrence rate was 19.3%, and only two cases of tumor-related mortality occurred.Conclusions:Surgery is the only curative treatment, with the main goal to restore/improve/arrest progression of clinical manifestations. The endoscopic endonasal route represents the master approach for lesions confined to the midline. Microsurgical transcranial and endoscopic transorbital approaches have a complementary role for addressing the lesion’s component with large intracranial extension or affecting the paramedian aspect of the anterior cranial fossa and superior–lateral orbital compartment, respectively. The approach selection should be made case by case according to the tumor pattern of growth.
背景：双表型鼻腔鼻窦肉瘤是一种罕见的低度恶性肿瘤，起源于鼻腔鼻窦区域，具有局部侵袭性生物学行为，常侵犯眼眶和颅底。目前尚无明确的治疗指南，因此不同机构的治疗方案存在差异。本研究旨在通过文献综述，根据病变的生长模式，提出一套模块化的手术入路系统。 材料与方法：在PubMed和Embase在线电子数据库中对侵犯眼眶的双表型鼻腔鼻窦肉瘤进行了全面详细的文献综述，并加入了一例具有特殊特征的个人病例。分析了人口统计学（患者性别和年龄）、临床（就诊症状和治疗时间）、神经影像学（解剖起源和生长模式）和治疗（治疗类型、手术入路、切除范围、围手术期及术后并发症以及辅助治疗）数据，以及临床结局、复发率和总生存率。 结果：共确定了31例侵犯眼眶的双表型鼻腔鼻窦肉瘤患者。肿瘤主要影响女性患者（66.7%）和中年人群（中位年龄55.2岁）。大多数病例（80.6%）同时伴有颅底受累。除一例外（97%），所有患者均接受了手术治疗，作为唯一治疗手段（59%）或联合放疗（23.5%）和/或化疗（5.9%/2.9%），大多数病例（66.7%）实现了肿瘤大体全切除。内镜经鼻入路是最常用的手术通道（51.7%）。局部复发率为19.3%，仅发生两例肿瘤相关死亡。 结论：手术是唯一的根治性治疗方法，其主要目标是恢复/改善/阻止临床表现的进展。对于局限于中线的病变，内镜经鼻入路是主要的手术方式。显微外科经颅入路和内镜经眶入路分别对于处理颅内广泛扩展或累及前颅窝旁正中区域及眶上外侧区域的病变部分具有补充作用。手术入路的选择应根据肿瘤的生长模式进行个体化决策。
肿瘤(癌症)患者之家