Unit of Otorhinolaryngology - Head and Neck Surgery, ASST Spedali Civili di Hospital, University of Brescia, Piazza Spedali Civili 1, Brescia, 25123, Italy.
Department of Medical and Surgical Specialties, Radiological Sciences, and Public Health, University of Brescia, Brescia, Italy.
Eur Arch Otorhinolaryngol. 2024 Nov;281(11):5627-5640. doi: 10.1007/s00405-024-08813-8. Epub 2024 Jul 23.
Nasal vestibule squamous cell carcinoma (NVSCC) is an exceedingly rare malignancy, often misclassified due to its anatomical location and lack of a standardized definition. This review aims to consolidate current evidence on NVSCC, focusing on epidemiology, risk factors, classification, clinical presentation, treatment modalities, and prognostic factors. The NV anatomy is delineated, emphasizing the need for a clear definition to avoid misclassification. Risk factors include smoking, sunlight exposure, and debated associations with chalk exposure or viral factors. Clinical presentation includes symptoms like nasal obstruction, pain, burning, and bleeding, often misdiagnosed as inflammatory conditions. NVSCC exhibits distinct local spread patterns along cartilaginous surfaces, with the facial and submandibular lymph nodes at higher metastatic risk. Current classifications lack consensus, hindering comparison of outcomes. Treatment varies, with surgery or radiotherapy for early-stage tumors and multimodality approaches for advanced cases. The choice between surgery and radiotherapy is debated, with potential advantages and drawbacks for each. Radiotherapy, especially with Interventional RadioTherapy (IRT, previously known as brachytherapy), is gaining prominence, showing promising outcomes in terms of local control and cosmetic results. Prophylactic neck treatment remains controversial, with indications based on tumor characteristics. Prognostic factors include T classification, tumor size, surgical margins, nodal involvement, and histological features. Long-term survival rates range widely, emphasizing the need for further studies to refine management strategies for this rare malignancy. In conclusion, NVSCC poses diagnostic and therapeutic challenges, warranting multidisciplinary approaches and continued research efforts to optimize patient outcomes.
鼻腔前庭鳞状细胞癌 (Nasal Vestibule Squamous Cell Carcinoma, NVSCC) 是一种极为罕见的恶性肿瘤，由于其解剖位置和缺乏标准化定义，常被误诊。本综述旨在综合目前关于 NVSCC 的证据，重点关注流行病学、危险因素、分类、临床表现、治疗方式和预后因素。本文阐述了 NV 的解剖结构，强调需要明确的定义以避免误诊。危险因素包括吸烟、阳光暴露，以及与粉笔暴露或病毒因素的争议性关联。临床表现包括鼻塞、疼痛、烧灼感和出血等症状，常被误诊为炎症性疾病。NVSCC 沿软骨表面呈独特的局部扩散模式，面部和颌下淋巴结转移风险较高。目前的分类缺乏共识，阻碍了结果的比较。治疗方式因肿瘤分期而异，早期肿瘤采用手术或放疗，晚期病例采用多模态治疗。手术和放疗之间的选择存在争议，每种方法都有其优缺点。放疗，特别是介入放疗（Interventional RadioTherapy，以前称为近距离放疗），越来越受到关注，在局部控制和美容效果方面显示出良好的结果。预防性颈部治疗仍存在争议，适应证基于肿瘤特征。预后因素包括 T 分类、肿瘤大小、手术切缘、淋巴结受累和组织学特征。长期生存率差异较大，强调需要进一步研究以完善这种罕见恶性肿瘤的治疗策略。总之，NVSCC 诊断和治疗具有挑战性，需要多学科方法和持续的研究努力，以优化患者的治疗效果。
