Touihmi Safaa, Horrane Ichraq, Rkain Ilham
Department of Otorhinolaryngology and Head and Neck Surgery Department, University Hospital of Tangier, Tetouan-Al hoceima.
Ann Med Surg (Lond). 2023 Apr 11;85(5):2029-2033. doi: 10.1097/MS9.0000000000000532. eCollection 2023 May.
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.
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.
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.
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.
嗅神经母细胞瘤（ENB）是一种罕见肿瘤，起源于嗅上皮。它在鼻腔上部表现为侵袭性肿瘤。鼻窦症状最为常见。近10%的病例会出现颈部淋巴结转移，血行转移罕见。诊断依靠组织学检查。该肿瘤采用卡迪什等人的系统进行分期。计算机断层扫描（CT）和磁共振成像（MRI）等成像技术提供了治疗方式所需的所有重要信息。如今，结合颅面外切除术、放疗和化疗的标准多模式治疗提高了长期生存率。
一名27岁无病史男性患者，主诉头痛、右侧单侧鼻塞、鼻出血和嗅觉丧失2个月。鼻内镜检查显示一个粉灰色肿物充满右侧鼻腔。进行了增强CT扫描，发现蝶窦有一个轻度强化的广泛肿物，伴有窦左侧壁骨质侵蚀和颅内受累。进行了鼻内活检，组织病理学诊断为嗅神经母细胞瘤。根据卡迪什分期，我们的病例分期为C期。肿瘤无法手术切除，患者接受了化疗、放疗和疼痛管理。
ENB是一种源自鼻腔上部特殊嗅神经上皮的侵袭性恶性肿瘤。多篇已发表报告证实了ENB在整个鼻腔和中枢神经系统的异位病例。由于鼻窦恶性病变罕见且难以与良性病变区分。ENB表现为柔软、有光泽、息肉样或结节样肿物，表面覆盖完整黏膜，或表现为伴有溃疡和肉芽组织的易碎肿物。应进行通过颅底和鼻窦的静脉造影CT扫描。ENB是实性、强化的鼻腔肿物，可能表现为对附近骨质的侵蚀。MRI能更好地区分肿瘤与分泌物，并能最佳评估眼眶、颅内或脑实质受累情况。活检是确诊的下一个重要步骤。ENB的经典治疗策略基于手术或放疗作为单一方式，或手术与放疗联合。最近，由于已证明ENB对化疗敏感，化疗已被纳入治疗手段。选择性颈部清扫术仍存在争议。ENB患者必须进行长期随访。
虽然大多数ENB起源于鼻腔顶部，在疾病晚期出现典型的鼻塞和鼻出血症状，但也应考虑不常见的表现。晚期和无法切除的疾病患者应考虑辅助治疗。需要持续的随访期。
