BACKGROUND: Extraskeletal myxoid chondrosarcoma of the jugular foramen is a rare clinical entity, especially in the pediatric population. Thus, it can be confused with other pathologies.
METHODS: We report an extremely rare case of a 14-year-old female patient with jugular foramen myxoid chondrosarcoma that was completely removed through microsurgical resection.
CONCLUSIONS: The primary purpose of the treatment is gross total resection of the chondrosarcomas. However, adjuvant methods such as radiotherapy should additionally be applied in patients who have high-grade diseases or cannot undergo gross total resection because of anatomic localization.

The anterolateral approach (ALA) enables access to the mid and lower clivus, jugular foramen (JF), craniocervical junction, and cervical spine with added anterior and lateral exposure than the extreme lateral and endoscopic endonasal approach, respectively. We describe the microsurgical anatomy of ALA with cadaveric specimens and report our clinical experience for benign JF tumors with predominant extracranial extension.
A stepwise and detailed microsurgical neurovascular anatomy of ALA was explored with cadaveric specimens. Then, the clinical results of 7 consecutive patients who underwent ALA for benign JF tumors with predominant extracranial extension were analyzed.
A hockey stick skin incision is made along the superior nuchal line to the anterior edge of the sternocleidomastoid muscle (SCM). ALA involves layer-by-layer muscle dissection of SCM, splenius capitis, digastric, longissimus capitis, and superior oblique muscles. The accessory nerve runs beneath SCM and is found at the posterior edge of the digastric muscle. The internal jugular vein (IJV) is lateral to and at the level of the accessory nerve. The occipital artery passes over the longissimus capitis muscle and IJV and into the external carotid artery, which is lateral and superficial to IJV. The internal carotid artery (ICA) is more medial and deeper than external carotid artery and is in the carotid sheath with the vagus nerve and IJV. The hypoglossal and vagus nerves run along the lateral and medial side of ICA, respectively. Prehigh cervical carotid, prejugular, and retrojugular surgical corridors allow deep and extracranial access around JF. In the case series, gross and near-total resections were achieved in 6 (85.7%) patients without newly developed cranial nerve deficits.
ALA is a traditional and invaluable neurosurgical approach for benign JF tumors with predominant extracranial extension. The anatomic knowledge of ALA increases competency in adding anterior and lateral exposure of extracranial JF.

暂无摘要。

本文报道一例源于颈静脉孔区并同时向颅内外侵犯的舌咽神经鞘瘤病例，患者为青年男性，以耳闷伴耳鸣及听力下降为主诉，术前行颞骨CT、颅脑增强MRI及血管造影（DSA）检查，采用乙状窦前-面后-迷路下入路手术完整切除肿瘤，同时保留了患者的听力和面神经功能，术后随访15个月，未见复发。.

Schwannoma, a tumor originating from the peripheral nervous system, may arise from the vagus nerve, although it is not very often. Injury of the vagus nerve by surgical attempts may have consequences that will seriously affect the patient\'s quality of life. In recent years, continuous monitoring of the laryngeal adductor reflex (LAR) has become a promising methodology for evaluating vagus nerve function intraoperatively. We refer to our experience changing our surgical strategy due to concurrent deterioration in LAR and CoMEPs intraoperatively. We also provide a literature review and summarize the current knowledge of this technique.
The LAR was elicited and recorded by an electromyographic endotracheal tube in a 36-year-old man diagnosed with vagal nerve schwannoma. Subdermal needle electrodes were placed in both cricothyroid (CTHY) muscles for corticobulbar motor evoked potentials (CoMEPs) recording.
Recordings of ipsilateral LAR and CTHY CoMEPs were obtained despite preoperative ipsilateral cord vocalis weakness. The surgical strategy was altered after the simultaneous decrease of CTHY CoMEPs and LAR amplitudes, and the surgery was completed with subtotal resection. No additional neurological deficit was observed in the patient except dysphonia, which resolved within a few weeks after the surgery.
We conclude that LAR with vagal nerve CoMEPs are two complementary methods and provide reliable information about the functional status of the vagus nerve during surgery.

Inflammatory pseudotumor (IP) is a nonneoplastic, reactive inflammatory process, of unknown etiology, characterized by a proliferation of connective tissue with an inflammatory infiltrate, most commonly involving the lungs and orbits. Primary intracranial IP is an extremely rare entity often arising from the meningeal structures of the skull base. We reported an extremely rare case of a primary intracranial IP located in the cerebellopontine angle, mimicking a jugular foramen meningioma. We further illustrated our microsurgical technique through a surgical video and performed a review of the pertinent scientific literature. The patient underwent gross total microsurgical resection of the tumor mass through a left retrosigmoid approach. Intraoperative neuromonitoring of the VII-VIII cranial nerve complex and lower cranial nerve was performed, and thulium laser fibers were used as a tool for tumor debulking. Postoperatively, the patient\'s neurologic symptoms recovered. Histopathologic studies showed dense infiltrate of T- and B-cell lymphocytes and epithelioid granulomas, compatible with the diagnosis of IP. Postoperatively, magnetic resonance imaging scans showed complete tumor resection. The patient underwent a 3-month oral corticosteroid therapy showing no signs of recurrence at the radiologic follow-up. Primary intracranial IPs are rare pathologic entities that can mimic extraaxial tumors and should be taken into consideration as a potential differential diagnosis. Complete microsurgical resection in combination with other treatments (steroids therapy, radiotherapy) is the most common treatment of choice and is associated with good outcomes and low rates of recurrence.

BACKGROUND: Most infants and children with achondroplasia show delayed motor skill development; however, some patients may have clinical consequences related to cranio-cervical junction stenosis and compression.
OBJECTIVE: To assess, using brain magnetic resonance imaging (MRI), quantitative variables linked to neuromotor impairment in achondroplasic children.
METHODS: In total, 24 achondroplasic children underwent pediatric neurological assessment and were grouped in two cohorts according to relevant motor skill impairment. Achondroplasic children with (n=12) and without (n=12) motor symptoms were identified, and brain MRI scans were quantitatively evaluated. 3D fast spoiled gradient echo T1-weighted images were used to assess: supratentorial intracranial volumes (SICV); supratentorial intracranial brain volume (SICBV); SICV/SICBV ratio; posterior cranial fossa volume (PCFV); posterior cranial fossa brain volume (PCBFV); PCFV/PCFBV ratio; ventricular and extra-ventricular cerebrospinal fluid (CSF) volumes; foramen magnum (FM) area; and jugular foramina (JF) areas.
RESULTS: In both groups, SICV/SICBV ratio, supratentorial ventricular and extra-ventricular space volumes were increased while SICBV was increased only in the asymptomatic group (P < 0.05). PCFV/PCFBV ratio, IV ventricle, infratentorial extra-ventricular spaces volumes were reduced (P < 0.05) in the symptomatic group while PCFBV was increased only in the asymptomatic group (P < 0.05). Foramen magnum (FM) area was more reduced in the symptomatic group than the asymptomatic group (P < 0.05) but no correlation between FM area and ventriculomegaly was found (P > 0.05).
CONCLUSIONS: Evaluation of the FM area together with infratentorial ventricular and extra-ventricular space volume reduction may be helpful in differentiating patients at risk of developing motor skill impairment. Further investigation is needed to better understand the temporal profile between imaging and motor function in order to propose possible personalized surgical treatment.

Carney综合征是一种罕见的常染色体显性遗传病，其特征性表现包括皮肤及黏膜色素沉着，心脏、皮肤及其他部位黏液瘤以及多发性内分泌或非内分泌肿瘤。本文报道1例累及颈静脉孔区的黏液瘤最终诊断为Carney综合征的患者，经二次手术切除，结合术后病理及全身检查得以确诊，术后定期复查未见肿瘤复发。.

Hemangiopericytomas (HPCs) and solitary fibrous tumors (SFTs) are rare tumors of mesenchymal origin. Here, the authors present a rare case of anaplastic HPC in the jugular foramen (JF). The authors also conduct a systematic review of the literature to examine the current fund of knowledge on JF HPC/SFTs.
A systematic MEDLINE search was conducted using key words \"hemangiopericytoma\" OR \"solitary fibrous tumor\" AND \"jugular foramen\" OR \"extracranial\" OR \"skull base.\" Clinicopathologic characteristics and outcomes of the present case were reviewed and compared with those in the literature.
A 41-year-old male, who had undergone stereotactic radiation therapy 6 years ago for a presumed glomus jugulare tumor, presented to our institution with worsening dysphagia, hoarseness, persistent tongue weakness, and radiographic evidence of tumor progression. The patient underwent uncomplicated gross total resection with sacrifice of the infiltrated hypoglossal nerve. Histopathologic evaluation revealed anaplastic HPC/SFT (World Health Organization grade III). Review of the literature yielded 9 additional cases of JF HPC/SFT in 5 males (56%) and 4 females (44%), with a mean age of 49.6 years old. Patients commonly presented with pain (37.5%) and lower cranial nerve deficits (100%). Preoperative diagnoses included glomus jugulare (n = 2) or JF schwannomas (n = 3). All patients underwent microsurgical resection of the lesion, except for 1 who refused all treatment after diagnostic biopsy.
The authors present the only reported case of anaplastic HPC of the JF. The illustrative case and those found on systematic review of the literature highlight the importance of tissue diagnosis and appropriate management.

Facial nerve schwannoma (FNS) is a benign, slow-growing schwannoma that originates from Schwann cells. Facial nerve schwannoma is the most common tumor of the facial nerve but rare and only accounts for 0.15% to 0.8% of intracranial neurinomas. It may be manifested as asymmetric hearing loss, facial palsy, and hemifacial spasm. A 56-year-old woman was transferred to our department, because of pain behind the right ear and spasm of the right lateral muscle for more than 2 years and pulsatile tinnitus for half a year. Based on the preoperative medical history, physical signs, and auxiliary examination, it was diagnosed with jugular foramen (JF) space-occupying lesion. We removed the tumor through the infratemporal fossa type A approach and found that the tumor originated from the facial nerve. After the tumor resection, sural nerve transplantation was performed. The patient demonstrated postoperative facial palsy (House-Brackman grade VI) and was smoothly discharged after good recovery. Facial nerve schwannoma rarely invades the JF, and the most common tumor in the JF is the glomus jugular tumor, followed by the posterior cranial schwannoma. They have common symptoms, making it difficult to obtain a correct diagnosis. Clinical data, medical history, and auxiliary examinations should be carefully analyzed to avoid misdiagnosis or mistreatment. Infratemporal fossa type A approach is an effective method for treating FNS of JF.