异位原发性嗅神经母细胞瘤:极其罕见的临床治疗方法

Milena Fior , Francesco Mazzola , Mario Rigante , Joseph Khazen , Renato Covello , Antonello Vidiri , Raul Pellini
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引用次数: 0

摘要

背景嗅神经母细胞瘤(ONB)是一种罕见的神经外胚层恶性肿瘤。在这种已经非常罕见的肿瘤中,异位嗅神经母细胞瘤(ectopic ONB)是一种更为罕见和鲜为人知的肿瘤。造影剂增强核磁共振检查发现,病变的中心位于骨膜复合体,但未累及楔形板和嗅凹。内窥镜引导下活检显示为 ONB Hyams II。结果术后过程顺利。明确的组织学检查证实肿瘤为海姆斯II级,卡迪什B型ONB,证实了异位定位。鉴于肿瘤的分期和分级,没有进行辅助治疗。结论异位 ONB 应在鼻窦肿块的鉴别诊断中予以考虑。结论异位 ONB 应在鼻窦肿块的鉴别诊断中予以考虑,嗅球和硬脑膜的切除应根据楔形板的临床或放射学受累情况而定。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Ectopic primary olfactory neuroblastoma: Clinical management of an extremely rare entity

Background

Olfactory neuroblastoma (ONB) is a rare malignant neoplasm of neuroectodermal origin. Within an already very rare neoplasm, an even rarer and lesser-known entity is ectopic ONB.

Methods

A 57-year-old female patient was evaluated for a smooth ethmoidal mass. A contrast-enhanced MRI identified a lesion with epicenter in the osteo-meatal complex without cribriform plate and olfactory recesses involvement. Endoscopic guided biopsy showed ONB Hyams II. An endoscopic trans-nasal resection was performed.

Results

The postoperative course was uneventful. A definitive histologic examination confirmed a Hyams grade II, Kadish B ONB, corroborating the ectopic localization. Due to the tumor stage and grade, no adjuvant treatment was performed. The patient was free of disease at one-year postoperative follow-up.

Conclusions

Ectopic ONB should be considered in the differential diagnosis of a sinonasal mass. Olfactory bulb and dura resection should be dictated by the clinical or radiological involvement of the cribriform plate.
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