表皮神经母细胞瘤(ENB)是一种罕见的肿瘤,来自嗅觉上皮。它表现为鼻腔上部的侵袭性肿瘤。鼻窦症状是最常见的。近10%的病例会出现颈淋巴结,并且很少见血行转移。诊断是组织学的。使用Kadish等人系统对该肿瘤进行分期。成像技术,使用计算机断层扫描(CT)和MRI提供了治疗方式所需的所有重要信息。今天,标准的多模式治疗结合颅面外切除术,放射治疗,化疗改善了长期生存率。
■一名27岁男性患者,无病史,抱怨头痛,单侧右鼻塞,鼻出血,和两个月的失眠症。鼻内窥镜检查显示右侧鼻腔有粉红色灰色肿块。进行了增强对比CT扫描,并对蝶窦轻度增强的大量肿块进行了客观分析,窦左壁骨侵蚀和颅内受累。进行了鼻内活检,导致嗅觉神经母细胞瘤的组织病理学诊断。根据卡迪什分期,我们的病例被分期为C期。肿瘤无法手术,病人接受了化疗,放射治疗,和疼痛管理。
■ENB是一种侵袭性恶性肿瘤,源自上鼻腔的专门嗅觉神经上皮。一些已发表的报告证实了整个鼻腔和中枢神经系统的ENB异位病例。因为鼻窦恶性病变罕见且难以与良性病变区分。ENBs显示为软,闪闪发光,息肉状,或由完整粘膜覆盖的结节状肿块或具有溃疡和肉芽组织的易碎肿块。放射科,应进行颅底和鼻旁窦的CT扫描,并进行静脉造影。ENBs是坚固的,增强鼻腔肿块,可能表现为侵蚀附近的骨。MRI可以更好地区分肿瘤和分泌物,并对眼眶进行最佳评估。颅内,或者脑实质受累.活检是确保诊断的下一个重要步骤。ENB的经典治疗策略基于手术或放射疗法作为独特的方式或手术和放射疗法的组合。最近,由于ENB已被证明对化学敏感,因此已在治疗性医疗设备中引入了化学疗法。选择性颈淋巴结清扫术仍然存在争议。ENB患者必须进行长期随访。
■虽然大多数ENBs起源于上鼻穹隆,并在疾病晚期出现典型的鼻塞和鼻出血症状,不常见的表现也应该考虑。对于晚期疾病和不可切除疾病的患者,应考虑辅助治疗。需要一个持续的后续期。
Esthesioneuroblastoma (ENB) is a rare tumor, arising from the olfactory epithelium. It manifests as an aggressive tumor in the superior aspect of the nasal cavity. Sinonasal symptoms are the most common. The cervical lymph nodes ensue in nearly 10% of cases and hematogenous metastases are rare. The diagnosis is histological. This tumor is staged using the Kadish et al System. The imaging techniques, using both computed tomography (CT) and MRI provides all the important information required for treatment modality. Today, the standard multimodal treatment combining external craniofacial resection, radiotherapy, and chemotherapy has improved long-term survival.
UNASSIGNED: A 27-year-old male patient with no medical history, complained of a headache, a unilateral right nasal obstruction, epistaxis, and anosmia for 2 months. Nasal endoscopy showed a pinkish-gray mass filling the right nasal cavity. An enhanced-contrast CT scan was performed and objectified a mildly enhancing extensive mass of the sphenoid sinus with bone erosion of the left wall of the sinus and intracranial involvement. An intranasal biopsy was performed, resulting in a histopathological diagnosis of olfactory neuroblastoma. Our
case was staged as stage C according to the Kadish staging. The tumor was inoperable, the patient had chemotherapy, radiotherapy, and pain management.
UNASSIGNED: ENB is an aggressive malignant tumor derived from the specialized olfactory neuroepithelium of the upper nasal cavity. Several published
reports confirm ectopic cases of ENB throughout the nasal cavity and the central nervous system. Because sinonasal malignant lesions are rare and difficult to distinguish from their benign counterparts. ENBs appears as a soft, glistening, polypoidal, or nodular mass covered by intact mucosa or as friable masses with ulceration and granulation tissue. A radiological, CT scan through the skull base and paranasal sinuses with intravenous contrast should be performed. ENBs are solid, enhancing nasal cavity masses that may manifest erosion into nearby osseous. MRI provides better discrimination between tumor and secretions and optimal assessment of orbital, intracranial, or brain parenchymal involvement. The biopsy is the next important step in securing a diagnosis. Classic treatment strategies of ENB are based on surgery or radiotherapy as unique modalities or a combination of surgery and radiation therapy. More recently, chemotherapy has been introduced in the therapeutic armamentarium since ENB has proven to be chemosensitive. The elective neck dissection remains controversial. Long-term follow-up is mandatory for patients with ENB.
UNASSIGNED: While most ENBs originate in the superior nasal vault and present with typical symptoms of nasal obstruction and epistaxis in the late stages of the disease, uncommon manifestations should be considered as well. Adjuvant therapy should be considered in patients with advanced disease and unresectable disease. A continuing follow-up period is needed.