【双语病例】嗅神经母细胞瘤1例MR影像特点

病史

A 58-year-old woman with a sinus mass

女性,58岁,鼻窦肿块。

【双语病例】嗅神经母细胞瘤1例MR影像特点

【双语病例】嗅神经母细胞瘤1例MR影像特点

【双语病例】嗅神经母细胞瘤1例MR影像特点

【双语病例】嗅神经母细胞瘤1例MR影像特点

【双语病例】嗅神经母细胞瘤1例MR影像特点

【双语病例】嗅神经母细胞瘤1例MR影像特点

【双语病例】嗅神经母细胞瘤1例MR影像特点

图例CT scan with bone algorithm (A) depicts an expanding mass within the nasal fossa, ethmoidal cell, and sphenoid sinus. It produces bone disruption and remodeling (arrows). Right exophtalmos is partially depticted. The mass shwos restricted diffusino on DWI (B) and ADC maps (C). Sagittal T2WI (D) and sagittal (E) and coronal (F) contrast-enhanced T1WI show heterogeneous signal intensity and enhancement of the mass, with a cystic component (arrow). The tumor extends to the intracranial compartment, producing mass effect on the adjacent brain parenchyma.

CT扫描骨算法重建显示:鼻前庭、筛窦、蝶窦内肿块,呈膨胀性生长,骨质破坏、塑形。右眼球稍突出。DWI示肿块弥散受限。矢状位T2WI及矢状位、冠状位的T1WI增强扫描示肿块信号不均匀,增强后肿块对比强化,强化信号不均匀,内可见囊变区。肿块突向颅内生长,部分脑实质受压。

【双语病例】嗅神经母细胞瘤1例MR影像特点

Esthesioneuroblastoma

嗅神经母细胞瘤

背景

  • Esthesioneuroblastoma (ENB) is an uncommon malignant tumor that arises from the olfactory epithelium. It was first described by Berger and colleagues in 1924.

    嗅神经母细胞瘤(ENB)是一种来源于嗅上皮的少见恶性肿瘤,1924年首次由Berger及其同事报道。

  • ENB constitutes aproximately 3% of all nasal neoplasms.

    ENB约占全部鼻肿瘤的3%左右。

  • Bimodal pattern of age distribution has been described, with peaks between 11–20 years of age and 40–70 years of age. A single peak during the sixth decade has also been reported.

    ENB的发病年龄呈双峰分布,好发于11-20岁青少年以及40-70岁中老年。也有报道该病好发年龄为60岁左右。

临床表现Nasal pressure, epistaxis, anosmia, nasal obstruction, visual symptoms, SIADH, and Cushing syndrome

鼻腔压力增高、鼻衄、嗅觉丧失、鼻塞、视力异常、抗利尿激素分泌异常综合征(SIADH)、库欣综合征。

影像诊断要点

  • On CT, ENB tends to be isodense or hyperdense compared to the surrounding soft tissues. There is moderate contrast enhancement. Bone erosion and remodeling are common.

    CT上,ENB与周围软组织相比,通常表现为等或高密度,增强扫描中度强化,常可见骨质侵蚀、塑形。

  • On MRI, tumors may be hypointense on T1WI and isointense or hyperintense (areas of cystic degeneration or hemorrhage) on T2WI. Avid enhancement after the gadolinium administration. Heterogeneous in areas of necrosis.

    MRI上,肿块T1WI呈低信号,T2WI呈等或高信号(内可有囊变、出血)。增强扫描后明显强化,强化信号不均匀,囊变区无强化。

鉴别诊断

  • Sinonasal undifferentiatted carcinoma (generally in older patients)

    鼻腔未分化癌:通常发生于老年人

  • Squamous cell carcinoma (more common in maxillary antrum, and with less enhancement)

    鳞状细胞癌:常见于上颌窦,强化不明显

  • Meningioma (may produce hyperostosis, and it is not associated with cysts at the tumor and brain interface)

    脑膜瘤:常可见临近骨质肥厚,且肿块与脑实质交界处通常无囊性区

  • Lymphoma (does not enhance to same intensity as ENB)

    淋巴瘤:强化程度与ENB不同。

治疗要点Surgery, chemotherapy, and radiation therapy. Prognosis depends on the Hyams grade (ENB maturation), tumor extension (Kadish stage), and lymph node metastasis.

主要治疗方法包括手术、化疗、放疗。

预后通常取决于Hyams分级(ENB分化程度)、肿瘤范围(Kadish分期)、淋巴结转移情况。

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