嗅觉神经母细胞瘤,也称为美学神经母细胞瘤(ONB),是一种罕见的神经外胚层肿瘤,起源于鼻-窦道的嗅觉上皮。它通常发生在鼻出血,鼻塞,复视,和嗅觉缺失.
一名十六岁女性因鼻塞而入住本组,复发性鼻出血,嗅觉缺失,第六个月的间歇性头痛。在ENT咨询之后,体检,鼻内镜检查,并进行了多次活检。器乐图像(CT,已要求MRI)对上述病理进行分期。
器乐图像(CT,MRI)显示肿块充满右鼻腔和上颌骨,并累及筛板,没有硬脑膜浸润的证据。通过双额开颅术结合改良的鼻侧切面途径进行颅面切除术。采用厚度裂开的颅骨移植物重建下眶壁和内侧眶壁,向下旋转的带蒂galea-pericranium皮瓣。患者在6周内接受了56Gy的外部束放射治疗。
由多学科ENT团队协调的早期诊断和治疗,神经外科医生,肿瘤学家,病理学家,放射科医师是良好预后的先决条件.出色的手术切除,负边距,通过放射治疗对病理进行局部控制是至关重要的。
Olfactory neuroblastoma, also called esthesioneuroblastoma (ONB), is a rare neuroectodermal neoplasm that originates from the olfactory epithelium of the nose-sinus tract. It generally occurs with epistaxis, nasal obstruction, diplopia, and anosmia.
A 16-year-old female was admitted to our Unit with a complaint of nasal obstruction, recurrent epistaxis, anosmia, and intermittent headache of sixth month\'s duration. After the ENT consultation, physical examination, endonasal endoscopy, and multiple biopsies were performed. Instrumental images (CT, MRI) have been requested to stage the aforementioned pathology.
Instrumental images (CT, MRI) showed a mass filling the right nasal cavity and the maxillary bone and involving the cribriform plate without evidence of dural invasion. Craniofacial resection by means of a bifrontal craniotomy combined with a modified lateral rhinotomic transfacial route was performed. The reconstruction of the inferior and medial orbital walls with employing split-thickness calvarial grafts, pedicled galea-pericranium flap rotated downwards was performed. The patient received 56 Gy of external beam radiotherapy over a 6-week period.
Early diagnosis and treatment coordinated by a multidisciplinary team of ENTs, neurosurgeons, oncologists, pathologists, and radiologists are a prerequisite for a good prognosis. An excellent surgical debulking, negative margins, and subsequent locoregional control of the pathology through radiotherapy is fundamental.