Lepera Davide, Volpi Luca, Facco Carla, Turri-Zanoni Mario, Battaglia Paolo, Bernasconi Barbara, Piski Zalán, Freguia Stefania, Castelnuovo Paolo, Bignami Maurizio
*Department of Otorhinolaryngology, Ospedale di Circolo e Fondazione Macchi, University of Insubria †Unit of Pathology, Ospedale di Circolo e Fondazione Macchi ‡Department of Surgical and Morphological Sciences, Unit of Pathology, University of Insubria, Varese, Italy §Department of Otorhinolaryngology-Head and Neck Surgery, University of Pecs, Medical Center, Pecs, Hungary.
J Craniofac Surg. 2016 Jun;27(4):1001-6. doi: 10.1097/SCS.0000000000002701.
The extra-skeletal form is an unusual type of Ewing sarcoma (ES) arising from soft tissue and in the literature there are reports of less than 50 patients describing the tumor in the paranasal sinuses and skull base. The histological diagnosis is crucial to plan the correct treatment and the molecular confirmation is mandatory in equivocal patients. A multimodality treatment with chemotherapy, surgery and radiotherapy improved the outcomes of these diseases during the last decades and a free-margin resection with the endoscopic transnasal technique is one of the most recent ways to manage these pathologies in selected patients, reducing the morbidities of the external approaches and preserving the quality of life of the patient.Here, the authors present the first patient of primary sinonasal ES free from disease after 5 years of follow-up and treated with an endoscopic endonasal approach and a second patient of sinonasal metastases of ES treated with and endoscopic transnasal approach.
骨外型是一种罕见的尤因肉瘤(ES),起源于软组织,文献中报道描述鼻窦和颅底肿瘤的患者少于50例。组织学诊断对于规划正确的治疗至关重要,对于诊断不明确的患者,分子确认是必不可少的。在过去几十年中,化疗、手术和放疗的多模式治疗改善了这些疾病的治疗效果,采用内镜经鼻技术进行切缘阴性切除是治疗部分患者这些病变的最新方法之一,可减少外部入路的并发症并保留患者的生活质量。在此,作者报告了首例经内镜鼻内入路治疗的原发性鼻窦ES患者,随访5年后无疾病复发,以及第二例经内镜经鼻入路治疗的鼻窦ES转移患者。