【双语病例】颈静脉孔神经鞘瘤

病史

A 34-year-old woman with headache, impaired hearing in right ear, and imbalance for 3 months

女性34岁,近3个月出现头痛、右耳听力下降、身体不平衡。

【双语病例】颈静脉孔神经鞘瘤

【双语病例】颈静脉孔神经鞘瘤

【双语病例】颈静脉孔神经鞘瘤

【双语病例】颈静脉孔神经鞘瘤

【双语病例】颈静脉孔神经鞘瘤

【双语病例】颈静脉孔神经鞘瘤

图例Axial T1WI (A), postcontrast axial T1WI (B), postcontrast coronal T1WI (C), and axial T2WI (D) show a well defined T1-hypointense, T2-hyperintense, and strongly enhancing lesion in the right cerebellopontine angle extending from the right jugular foramen, causing widening of the jugular foramen and deformity of the brainstem, fourth ventricle, and cerebellum. The right internal auditory canal (D) is normal. There is no extracranial extension. Axial T2*-weighted gradient echo image (E) shows multiple intratumoral microhemorrhages.

横断位T1WI平扫(A)、横断位及冠状位增强扫描(B、C)、横断位T2WI平扫示右侧桥小脑角区至颈静脉孔区肿块,边界清楚,T1WI呈低信号,T2WI呈高信号,增强扫描明显强化。颈静脉孔增宽,脑干、第四脑室、小脑受压变形。右侧内听道正常(D)。脑内无明显肿瘤侵犯。横断位GRE T2*WI(E)示瘤内可见多个微出血灶。

【双语病例】颈静脉孔神经鞘瘤

Photomicrograph (F) shows fascicles of spindle cells with buckled nuclei (inset) of neural origin.

镜下可见起源于神经的梭形细胞呈束状排列。

【双语病例】颈静脉孔神经鞘瘤

Jugular Schwannoma

颈静脉孔神经鞘瘤

背景Jugular schwannomas are very rare tumors, accounting for about 2–4% of intracranial schwannomas

颈静脉孔神经鞘瘤是一种少见肿瘤,约占全部颅内神经鞘瘤的2-4%

临床表现

  • They occur commonly between the 3rd and 6th decades, with female predilection.好发于30-60岁,女性多于男性。
  • Hearing loss, tinnitus, ataxia, and vertigo are the most common initial symptoms.最常见的首发症状包括听力下降、耳鸣、共济失调、眩晕。
  • A constellation of symptoms localizing to the jugular foramen (9th, 10th, and 11th cranial nerve palsy) can sometimes be seen — called Vernet syndrome or jugular foramen syndrome — comprised of hoarseness; nasal regurgitation; uvula deviation to the contralateral side and dysphagia with loss of gag reflex; sternomastoid and trapezius palsy; and loss of sensation of the posterior third of tongue.有时可见颈静脉孔区受压引起的第9、10、11对颅神经麻痹,称为Vernet综合征或颈静脉孔综合征,其症状包括声音嘶哑、鼻涕返流、悬雍垂向对侧移位、吞咽困难、呕吐反射丧失;胸锁乳突肌及斜方肌麻痹;舌后1/3感觉减退。
影像诊断要点

  • Well defined lesion arising from jugular foramen, expanding it and further extending intracranially into the posterior fossa, below the skull base, or both (dumbbell shaped if both)颈静脉孔区占位,边界清楚,呈膨胀性生长,可向颅内生长至后颅凹、或生长至颅底下方,也可向两侧生长,呈哑铃状。
  • Hypointense on T1WI, hyperintense on T2WI, and moderate-to-marked contrast enhancementT1WI低信号,T2WI高信号,增强扫描中至重度强化。
  • Larger lesions may be heterogeneous due to necrosis or cystic formation. Intratumoral microhemorrhages can be appreciated in T2*-weighted gradient echo sequences.较大病灶内可出现坏死囊变,表现为信号不均匀。T2*WI GRE序列可以很好地显示瘤内的微出血。
  • 3D CISS and 3D postcontrast sequences might help in further localizing the lesion within the jugular foramen, whether it arises from pars nervosa (indicating IX nerve origin) or pars vascularis (indicating X or XI nerve origin).3D CISS序列及3D增强序列有助于颈静脉孔内病灶的进一步定位,来源于神经部(起源于舌咽神经)或静脉部(起源于迷走神经或副神经)。
  • On CT the lesion appears well defined and iso-to-hypodense compared to brain parenchyma, and the affected jugular foramen will show expansion and remodeling.CT上病灶表现为等或略低密度,边界清楚。病灶累及颈静脉孔可表现为颈静脉孔增大、变形。
鉴别诊断

  • Meningioma: Dural tail, hyperostosis, calcification, homogeneous isointense T1 and T2 signal, and intense homogeneous enhancement favor diagnosis of meningioma over schwannoma脑膜瘤:可见硬膜尾征,邻近骨质增厚,瘤内钙化,MRI呈等T1、等T2信号,增强扫描均匀强化。
  • Schwannoma from other cranial nerves (vestibular, hypoglossal): The diagnosis will depend on which cranial foramen or canal is widened其他颅神经(前突神经、舌下神经)起源的神经鞘瘤:鉴别诊断需要靠病变所在的颅神经孔增大来判断。
  • Glomus jugulare or jugulotympanicum: Bone destruction instead of bone remodeling, intratumoral flow voids, and dilated feeding arteries and draining veins, due to the hypervascular nature of the lesion颈静脉球瘤或鼓室球瘤:一般以骨质破坏为主,肿瘤为富血供,瘤内可见流空信号、扩张的滋养动脉和引流静脉。
  • Metastatic skull base lesions: Bone destruction and symptoms appear with smaller-sized lesions. Clinical history is fundamental for the DDx.颅底转移瘤:可见骨质破坏,很小的病灶即可出现症状。临床病史是主要的鉴别点。
治疗Excision. Small tumors can be followed up with imaging or treated with stereotactic radiosurgery.

手术切除。较小的病灶可影像学定期复查,或立体定向放疗。

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