El Omri Malika, Njima Maroua Ben, Mesbah Linda, Kermani Wassim, Bellakhdher Mouna, Andelkefi Mohamed
Department of Ear, Nose, Throat and Head and Neck Surgery, Farhat Hached University Hospital, Sousse, Tunisia.
Department of Ear, Nose, Throat and Head and Neck Surgery, Farhat Hached University Hospital, Sousse, Tunisia.
Int J Surg Case Rep. 2024 Jun;119:109757. doi: 10.1016/j.ijscr.2024.109757. Epub 2024 May 15.
Olfactory neuroblastoma or esthesioneuroblastoma is a rare malignant tumour, that develops in the olfactory neuroepithelium and is one of the rarest tumours of the nasal cavity. Ocular manifestations are uncommon. The diagnosis is based on histology: biopsy, immunohistochemistry and ultrastructural findings.
We report a case of olfactory neuroblastoma of the olfactory placode in a 36-year-old woman with orbital involvement. Computed tomography and magnetic resonance imaging of the skull, showed a suspicious lesion with significant orbital and cranial extension. After anatomopathological study of the biopsy, a protocol palliative radiotherapy was established.
We discuss the clinical, radiological, anatomopathological and therapeutic aspects of this condition, emphasising the importance of evoking this diagnosis in the presence of unilateral tumour-like exophthalmos associated with suggestive rhinological signs.
Ophthalmological involvement usually occurs at an advanced stage of esthesioneuroblastoma. This case highlights the fatal course of olfactory neuroblastoma. As it can present with the comlex symptoms related to ocular and nasal sites. Early diagnosis is the key to better therapeutic choices according to its level of extension, purposing at the best possible prognosis for the patient.
嗅神经母细胞瘤或嗅神经上皮瘤是一种罕见的恶性肿瘤，发生于嗅神经上皮，是鼻腔最罕见的肿瘤之一。眼部表现并不常见。诊断基于组织学：活检、免疫组织化学和超微结构检查结果。
我们报告一例36岁女性嗅基板嗅神经母细胞瘤伴眼眶受累的病例。头颅计算机断层扫描和磁共振成像显示一个可疑病变，有明显的眼眶和颅内扩展。活检经解剖病理学研究后，制定了姑息性放疗方案。
我们讨论了该疾病的临床、放射学、解剖病理学和治疗方面，强调在出现与提示性鼻科体征相关的单侧肿瘤样眼球突出时考虑这一诊断的重要性。
眼部受累通常发生在嗅神经母细胞瘤的晚期。本病例突出了嗅神经母细胞瘤的致命病程。由于它可表现出与眼部和鼻部相关的复杂症状。早期诊断是根据其扩展程度做出更好治疗选择的关键，目的是为患者争取最佳预后。
