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。
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。
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。
颈静脉孔神经鞘瘤
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%
- 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。
- 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。
- 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。
手术切除。较小的病灶可影像学定期复查，或立体定向放疗。