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Abstract：
The esthesioneuroblastoma (ENB) is a rare malignant sinonasal tumor of neuroectodermal origin. This study aimed to improve the understanding of the clinical features by reviewing the literature and analyzing the medical records of patients diagnosed with ENB in our institution between 2012 and 2019. A total of 6 cases of ENB were available for analysis. The mean age at the time of diagnosis was 36 years. The main complaints at presentation were the rhinologic signs. Tumors were classified as stage C of Kadisk in 3 cases and stage D in the others. According to TNM (modified by Dulguerov), 2 patients were T3N0M0, one T4N0M0, one T3N1M0, and two T4N1M0. The diagnosis of ENB was based on pathological examination. According to Hyams histological grading, low-grade lesions (Hyams I and II) were seen in 2 cases, high-grade undifferentiated lesions (Hyams III and IV) were seen in 4. Of the 6 patients, 4 received surgery. The surgical approaches mainly included an endoscopic endonasal resection in 2 cases, a cranial-facial resection surgery in 1 case, and an expanded endoscopic endonasal approach in combination with craniotomy in 1 case. Four patients received adjuvant radiotherapy (RT). RT dose ranged from 60 to 70 Gy. A total of 3 patients had lymph node metastasis and received RT of the neck. Chemotherapy was delivered in 2 patients. After a mean follow-up of 4.5 years, 4 patients were free of recurrence. Unfortunately, 1 patient died from a progressive disease 6 months after RT. ENB is a rare locally aggressive tumor of the nasosinusal cavities. The first-line treatment for resectable tumors should include primary surgical resection with adjuvant RT. However, this tumor remains of poor prognosis. Therefore, long-term close follow-up based on symptoms, endoscopy, and imaging is essential.
摘要：
神经母细胞瘤（ENB）是一种罕见的神经外胚层起源的恶性鼻窦肿瘤。本研究旨在通过回顾文献和分析2012年至2019年在我们机构诊断为ENB的患者的病历来提高对临床特征的理解。共有6例ENB可用于分析。诊断时的平均年龄为36岁。介绍时的主要抱怨是鼻学体征。3例肿瘤分为KadickC期，其余为D期。根据TNM(由Dulguerov修改)，2例患者为T3N0M0，1例T4N0M0，1例T3N1M0，2例T4N1M0。ENB的诊断基于病理检查。根据Hyams的组织学分级,2例见低度病变（HyamsI和II），高级别未分化病变(HyamsIII和IV)见于4例.6名患者中,4接受手术。手术入路主要包括2例经鼻内镜切除。颅面切除手术1例，扩大经鼻内镜入路联合开颅手术1例。4例患者接受辅助放疗（RT）。RT剂量范围为60至70Gy。共有3例患者有淋巴结转移，并接受了颈部RT。2例患者进行了化疗。经过4.5年的平均随访，4例患者无复发。不幸的是,1例患者在RT后6个月死于进行性疾病。ENB是一种罕见的鼻腔局部侵袭性肿瘤。可切除肿瘤的一线治疗应包括辅助RT的原发性手术切除。然而,这种肿瘤仍然预后不良。因此,根据症状进行长期密切随访，内窥镜检查,成像是必不可少的。
