Zahedi Farah Dayana, Gendeh Balwant Singh, Husain Salina, Kumar Ramesh, Kew Thean Yean
Department of Otorhinolaryngology-Head and Neck Surgery, Universiti Kebangsaan Malaysia Medical Centre, Jalan Ya'acob Latif, Bandar Tun Razak, Cheras, 56000 Kuala Lumpur, Malaysia.
Neurosurgery Unit, Department of Surgery, Universiti Kebangsaan Malaysia Medical Centre, Kuala Lumpur, Malaysia.
Indian J Otolaryngol Head Neck Surg. 2017 Mar;69(1):125-129. doi: 10.1007/s12070-016-0978-0. Epub 2016 May 7.
Esthesioneuroblastoma is a rare malignant neoplasm of oflactory neuroepithelium and usually located at the olfactory cleft at superior nasal cavity. Ectopic localization of esthesioneuroblastoma is even rarer and usually posed with a diagnostic dilemma and delay in the diagnosis and management, We report a rare case of ectopic esthesioneuroblastoma of the sphenoclivus with the presentation of intermittent unilateral epistaxis, intermittent intractable headache without anosmia. Nasal endoscopy findings showed a pulsatile mass at the anterior face of the sphenoid sinus with extension posteriorly towards the clivus region and occupying the floor of the sphenoid sinus. Endonasal transclival endoscopic excision of tumour was performed which involved otorhinolaryngology surgeon and neurosurgeon with intraoperative navigation imaging and frozen section. The histopathological findings was esthesioneuroblastoma. Due to its rarity and unusual presentation, the diagnosis of ectopic esthesioneuroblastoma is difficult and can be misdiagnosed with the other type of malignancy. Therefore, the histopathological result is important in confirming the type of tumour and can lead to the next step of management.
嗅神经母细胞瘤是一种罕见的嗅神经上皮恶性肿瘤,通常位于鼻腔上部的嗅裂处。嗅神经母细胞瘤的异位定位更为罕见,通常会带来诊断难题,并导致诊断和治疗延迟。我们报告一例罕见的蝶枕部异位嗅神经母细胞瘤病例,表现为间歇性单侧鼻出血、间歇性顽固性头痛且无嗅觉减退。鼻内镜检查发现蝶窦前壁有一搏动性肿物,向后延伸至斜坡区并占据蝶窦底部。在术中导航成像和冰冻切片的辅助下,由耳鼻喉科医生和神经外科医生共同进行了经鼻经斜坡内镜下肿瘤切除术。组织病理学检查结果为嗅神经母细胞瘤。由于其罕见性和不寻常的表现,异位嗅神经母细胞瘤的诊断较为困难,可能会被误诊为其他类型的恶性肿瘤。因此,组织病理学结果对于确定肿瘤类型很重要,并可指导下一步的治疗。