【双语病例】蝶窦淋巴瘤累及右侧海绵窦及眶尖影像表现与征象分析

【简要病史】

A 72-year-old woman presents with rapidly progressive horizontal and vertical diplopia as well as pain in the right forehead and right nasal region

72岁女性,进行性水平及垂直方向复视,进展迅速,伴有右侧前额、鼻部疼痛。


【双语病例】蝶窦淋巴瘤累及右侧海绵窦及眶尖影像表现

【双语病例】蝶窦淋巴瘤累及右侧海绵窦及眶尖影像表现

Question

1.Which of the following are present on these images? (Check all that apply.)

此组图像的可见下列那种表现?(选择全部正确选项)

Orbital apex involvement

眶尖部侵犯

Osseous sclerosis

骨质硬化

Mastoid air cell destruction

乳突气房破坏

Sphenoid sinuscortical disruption

蝶窦皮质破坏

正确选项:134

注释:

The lesioninvolves the lateral wall of the sphenoid sinus extending into the rightorbital apex, as demonstrated by loss of normal fat attenuation in thislocation.

此病变累及蝶窦外侧壁,侵入右侧眶尖,表现为此处正常脂肪消失。

The walls of thebilateral sphenoid sinuses are thickened and sclerotic, as is the intersphenoidalseptum. The sphenoid sinus is opacified.

双侧蝶窦壁及间隔增厚、硬化,蝶窦浑浊。

There is noevidence of a destructive process involving the mastoid air cells on theprovided images.

所示图像并无证据表明乳突气房破坏。

Focal corticaldisruption of the lateral wall of the right sphenoid sinus is present.

可见右侧蝶窦外侧壁局部骨皮质破坏。

【双语病例】蝶窦淋巴瘤累及右侧海绵窦及眶尖影像表现

【双语病例】蝶窦淋巴瘤累及右侧海绵窦及眶尖影像表现

Question

2. Brain MRimages demonstrate which of the following? (Check all that apply)

颅脑 MR图像的表现包括下列那种?(选择全部正确选项)

Sphenoid sinus opacification

蝶窦浑浊

Internal carotid artery occlusion

颈内动脉闭塞

Normal pattern of sphenoid sinus mucosal enhancement

蝶窦粘膜正常强化

Unilateral cavernous sinus expansion

单侧海绵的膨胀

正确选项:14

注释:

Thecontrast-enhanced T1-weighted images show diffuse loss of normal aeration ofthe sphenoid sinus with intrinsic T1 hyperintense signal and peripheral mucosalenhancement. There is some associated left sphenoid sinus expansion, consistentwith early mucocele-like changes.

对比增强T1WI示蝶窦内气体弥漫缺失,周围粘膜强化,T1像呈高信号,相应左侧蝶窦膨胀,与黏液囊肿早期改变相符。

There is noevidence of internal carotid artery occlusion in these images.

此组图像未见颈内动脉闭塞。

At thesuperolateral margins of the bilateral sphenoid sinuses, there is loss ofmucosal enhancement pattern, and nodular soft-tissue thickening is present.

双侧蝶窦侧上方未见粘膜强化,并见结节样软组织增厚。

Asymmetricexpansion of the right cavernous sinus with soft-tissue attenuation and outwardconvexity of the lateral wall of the right cavernous sinus are seen.

右侧海绵窦膨胀并见软组织信号,向右侧海绵窦侧壁外侵犯,与左侧不同。

Question

3.Which of the following symptoms can be explained by a cavernous sinus syndrome? (Check all that apply)

海绵窦综合征可引起下列哪些症状?(选择全部正确选项)

Diplopia

复视

Mandibular deviation with mouth opening

张口时下颌偏斜

Ptosis

上睑下垂

Forehead paresthesia

前额感觉异常

Unilateral blindness

单侧盲

正确选项:134

注释:

In the cavernoussinus, the abducens nerve runs lateral to the internal carotid artery andmedial to the oculomotor and trochlear nerves, which are contained within thelateral dural border of the cavernous sinus. Lesions affecting these cranialnerves can potentially result in diplopia.

海绵窦内外展神经在动眼神经及滑车神经之间,走形于颈内动脉外侧,为海绵窦硬脑膜包裹,当有病变累及这些颅神经时可导致复视。

The third division(V3) of the trigeminal nerve supplies the muscles of mastication. This branchof the trigeminal nerve exits inferiorly through the foramen ovale from Meckelcave and does not course through the cavernous sinus.

三叉神经第三支(V3)支配咀嚼肌,从Meckel腔经卵圆孔出颅,并不经海绵窦走形。

The oculomotornerve innervates the levator palpebrae superioris muscle and, when affected bya lesion of the cavernous sinus, can result in ptosis.

动眼神经支配提上睑肌,当海绵窦病变累及时可致上睑下垂。

The ophthalmicnerve (V1), the first division of the trigeminal nerve, runs through thelateral dural border of the cavernous sinus and carries sensory informationfrom the scalp, forehead, upper eyelid, conjunctiva and cornea of the eye, andnose. When affected, this may result in paresthesias of these areas.

三叉神经第一支即眼支(V1),穿行于海绵窦硬脑膜,传递头皮、前额、上眼睑、结膜及角膜、鼻部的感觉信息,当受累及时可导致这些区域感觉异常。

The optic nerve isnot located in the cavernous sinus and is not affected in a cavernous sinussyndrome.

视神经不从海绵窦走形,不受海绵窦综合征的影响。

图片说明:

【双语病例】蝶窦淋巴瘤累及右侧海绵窦及眶尖影像表现

This axialcontrast-enhanced CT of the brain in bone window shows cortical disruption ofthe lateral wall of the right sphenoid sinus (arrow) with a permeative patternof bone destruction.

此骨窗轴位增强颅脑CT示右侧蝶窦外侧壁骨皮质中断(箭),呈穿凿样骨质破坏。

【双语病例】蝶窦淋巴瘤累及右侧海绵窦及眶尖影像表现

This axialcontrast enhanced CT image depicts asymmetric soft-tissue attenuation withinthe right cavernous sinus and orbital apex (arrows).

轴位增强颅脑CT示右侧海绵窦及眶尖软组织密度影(箭),与左侧不对称。

【双语病例】蝶窦淋巴瘤累及右侧海绵窦及眶尖影像表现

This T1 coronalpostcontrast MR image shows hyperintense mucosal enhancement of the sphenoidsinus with disruption of the enhancement pattern superolaterally by a nodularmass (arrow) that is less intense than adjacent mucosal.

冠状位增强T1 MR示蝶窦粘膜强化呈高信号,侧上方可见结节样肿块(箭),强化信号低于临近粘膜。

【双语病例】蝶窦淋巴瘤累及右侧海绵窦及眶尖影像表现

This T1 coronalpostcontrast image shows hyperintense mucosal enhancement of the sphenoidsinus. There is diffuse mass-like infiltration into the right cavernous sinuswith sinus expansion and convexity of the lateral wall (arrow).

此冠状位增强T1WI示蝶窦粘膜强化呈高信号,右侧海绵窦膨胀,并见弥漫肿块样病变侵入海绵窦及其外侧壁(箭)。

【双语病例】蝶窦淋巴瘤累及右侧海绵窦及眶尖影像表现

This nonenhancedT1 MR image in the coronal plane shows asymmetric T1 isointense signal andexpansion of the right cavernous sinus secondary to an intracavernous mass(arrow).

T1WI冠状位平扫MR像示右侧海绵窦肿块(箭)呈等信号,致海绵窦膨胀,与左侧海绵窦不对称。

【双语病例】蝶窦淋巴瘤累及右侧海绵窦及眶尖影像表现

This T2 coronalimage demonstrates that the T2 signal of the right cavernous sinus lesion isisointense to gray matter. The mass encases the cavernous segment of the rightinternal carotid artery without apparent luminal narrowing (arrow).

T2WI示右侧海绵窦病变与脑灰质呈等信号,此肿块包绕右侧颈内动脉海绵窦段,而管腔无明显狭窄(箭)。

【双语病例】蝶窦淋巴瘤累及右侧海绵窦及眶尖影像表现

This T2 axialimage shows hyperintense material occupying the sphenoid sinus and hypointenseirregular lesions along the lateral wall of the sphenoid sinus, extending tothe posterior ethmoid cells (arrowhead). The lesion extends to the orbital apex(arrow). There is expansion of the ipsilateral cavernous sinus related to thelesion.

T2轴位像示蝶窦高信号病变,沿海绵窦外侧壁见不规则低信号病变,并侵入筛窦后部蜂房(箭头)及眶尖(箭),病变导致同侧海绵窦膨胀改变。

Diagnosis

Bilateral non-Hodgkin lymphoma of the sphenoid sinus with extension into the right cavernous sinus and orbital apex
双侧蝶窦非何杰金氏病淋巴瘤累及右侧海绵窦及眶尖

Case Points

Lymphoma is an uncommon cause of cavernous sinus syndrome and usually arises from metastatic deposits or direct infiltration.

淋巴瘤并非海绵窦综合征的常见原因,其常为转移性或直接侵润。

MR findings of a T2-weighted iso- to-hypointense contrast-enhancing cavernous sinus mass with diffusion restriction, permeative bone remodeling, and dural infiltration are findings in line with lymphoma.

MR T2WI呈等低信号,增强扫描海绵窦强化肿块并扩散受限,骨质呈穿凿样重构,硬膜浸润等表现与淋巴瘤相符。

Discussion

Approximately 50% of patients with malignant lymphoma clinically present with head and neck involvement, with the majority of cases showing nodal disease. Extranodal involvement of the head and neck is present in approximately 10% of cases and most commonly occurs in tonsillar tissue, sinonasal cavities, and the thyroid. Sinonasal lymphoma is found most commonly in the nasal fossa and maxillary sinuses with rare frontal and sphenoid sinus involvement. It may present as nodular or infiltrative enhancing mucosal masses, usually of iso- to-hypointense T2 signal compared to gray matter. Diffusion restriction may be identified. There is often disruption of the expected thin T2-bright mucosal signal and a hyperintense enhancement pattern by MRI. CT often reveals remodeling or erosion of the affected sinus wall.

50%恶性淋巴瘤患者临床表现为头部及颈部侵犯,主要表现为淋巴结病变。头颈部淋巴瘤约10%病例为淋巴结外侵犯,常发生于扁桃体,鼻前庭,甲状腺。鼻腔、鼻窦淋巴瘤最常发生于鼻前庭、上颌窦,而额窦及蝶窦罕见。常见表现为结节样或侵润性粘膜强化肿块,T2像与灰质比多呈等低信号。因扩散受限可以诊断。MRI上鼻窦的T2高信号及显著强化的薄层粘膜常被破坏。CT可发现受累窦壁骨质重建或侵蚀。

The cavernous sinus contains vital neurovascular structures that may be affected by vascular, neoplastic, infectious, or infiltrative processes. Patients with cavernous sinus syndrome usually present with paresis of one or more cranial nerves (III, VI), which may be associated with painful ophthalmoplegia. The cavernous sinus rarely represents a site of primary lymphoma involvement, with most cases arising as metastatic deposits from systemic disease or from direct infiltration, as in this case. CT or MR imaging typically reveals a homogenous enhancing cavernous sinus mass, which can mimic a meningioma. Evidence of direct extension from the paranasal sinuses, dural infiltration, or osseous permeation and remodeling may be helpful in differentiating this from other processes.

海绵窦内含重要的神经血管结构,可被血管、肿瘤、感染或浸润性病变累及。海绵窦综合征患者常表现为一条或多条颅神经麻痹(III, VI),这可能与痛性眼肌麻痹相关。海绵窦原发淋巴瘤侵犯罕见,大多为系统性病变转移,或如此病例所示的直接侵润。CTMR图像典型表现为海绵窦均质强化肿块,与脑膜瘤相似。副鼻窦病变直接侵犯,硬脑膜侵润,骨质穿凿样改变及重建等表现有助于此病与其它疾病鉴别。

Among extranodal forms of non-Hodgkin lymphoma, those involving the paranasal sinuses (most notably the sphenoid sinus) carry the poorest prognosis since the lack of specificity with regard to the presenting clinical symptoms leads to delays in diagnosis. Non-Hodgkin lymphomas are frequently treated with, and respond to, a combination of chemotherapy and radiotherapy. A review of several reports suggests that the best treatment outcomes are usually obtained with the CHOP [cyclophosphamide, Adriamycin, vincristine (Oncovin), and prednisone] regimen, given at 3-week intervals.

在结外的非何杰金氏淋巴瘤中,副鼻窦淋巴瘤(特别是蝶窦)因无特异性的临床症状而延误诊断,故预后最差。非何杰金氏淋巴瘤常行化疗、放疗的复合治疗。回顾多篇文献表明间歇期为3周的CHOP方案疗效最好。

References

Razek AAKA, Castillo M. Imaging lesions of the cavernous sinus. Am J Neuroradiol. 2009;30:444–52.

Demirkaya M, Sevinir B, Özdemir Ö, et al. Lymphoma of the cavernous sinus mimicking Tolosa-Hunt syndrome in a child. Pediatr Neurol.2010;42(5):351-4.

Park YM, Cho JH, Cho JY, et al. Non-Hodgkin’s lymphoma of the sphenoid sinus presenting as isolated oculomotor nerve palsy. World J Surg Oncol. 2007;5:86.

Haldorsen IS, Espeland A, Larsson EM. Central nervous system lymphoma: characteristic findings on traditional and advanced imaging. Am J Neuroradiol. 2011;32:984–92.

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