Jugular Foramen Paragangliomas.

Paragangliomas are the most common tumors at jugular foramen and pose a great surgical challenge. Careful clinical history and physical examination must be performed to adequately evaluate neurological deficits and its chronologic evolution, also to delineate an overview of the patient performance status. Complete imaging evaluation including MRI and CT scans should be performed, and angiography is a must to depict tumor blood supply and sigmoid sinus/internal jugular vein patency. Screening for multifocal paragangliomas is advisable, with a whole-body imaging. Laboratory investigation of endocrine function of the tumor is necessary, and adrenergic tumors may be associated with synchronous lesions. Preoperative prepare with alpha-blockage is advisable in norepinephrine/epinephrine-secreting tumors; however, it is not advisable in exclusively dopamine-secreting neoplasms. Best surgical candidates are young otherwise healthy patients with smaller lesions; however, treatment should be individualized each case. Variations of infratemporal fossa approach are employed depending on extensions of the mass. Regarding facial nerve management, we avoid to expose or reroute it if there is preoperative function preservation and prefer to work around facial canal in way of a fallopian bridge technique. If there is preoperative facial nerve compromise, the mastoid segment of the nerve is exposed, and it may be grafted if invaded or just decompressed. A key point is to preserve the anteromedial wall of internal jugular vein if there is preoperative preservation of lower cranial nerves. Careful multilayer closure is essential to avoid at most cerebrospinal fluid leakage. Residual tumors may be reoperated if growing and presenting mass effect or be candidate for adjuvant stereotactic radiosurgery.

Extraskeletal Myxoid Chondrosarcoma of the Jugular Foramen in a Pediatric Patient: A Case Report and Comprehensive Review of the Literature.

INTRODUCTION: 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. CASE PRESENTATION: 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. CONCLUSION: 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.

Utility of dynamic susceptibility contrast MRI for differentiation between paragangliomas and meningiomas in the cerebellopontine angle and jugular foramen region.

PURPOSE: Differentiation of paragangliomas and meningiomas can be a challenge. This study aimed to assess the utility of dynamic susceptibility contrast perfusion MRI (DSC-MRI) to distinguish paragangliomas from meningiomas. METHODS: This retrospective study included 40 patients with paragangliomas and meningiomas in the cerebellopontine angle and jugular foramen region between March 2015 and February 2022 in a single institution. Pretreatment DSC-MRI and conventional MRI were performed in all cases. Normalized relative cerebral blood volume (nrCBV), relative cerebral blood flow (nrCBF), relative mean transit time (nrMTT), and time to peak (nTTP) as well as conventional MRI features were compared between the 2 tumor types and between meningioma subtypes as appropriate. Receiver operating characteristic curve and multivariate logistic regression analysis were performed. RESULTS: Twenty-eight meningiomas including 8 WHO grade II meningiomas (12 males, 16 females; median age 55 years) and 12 paragangliomas (5 males, 7 females; median age 35 years) were included in this study. Paragangliomas had a higher rate of cystic/necrotic changes (10/12 vs 10/28; P = 0.014), a higher rate of internal flow voids (9/12 vs 8/28; P = 0.013), higher nrCBV (median 9.78 vs 6.64; P = 0.04), and shorter nTTP (median 0.78 vs 1.06; P < 0.001) than meningiomas. There was no difference in conventional imaging features and DSC-MRI parameters between meningioma subtypes. nTTP was identified as the most significant parameter for the 2 tumor types in the multivariate logistic regression analysis (P = 0.009). CONCLUSIONS: In this small retrospective study, DSC-MRI perfusion differences were observed between paragangliomas and meningiomas, but not between grade I and II meningiomas.

Precise differentiation between jugular foramen paragangliomas and metastases: utility of diffusion-weighted and dynamic contrast-enhanced magnetic resonance imaging.

PURPOSE: This study tested the utility of diffusion-weighted imaging (DWI) and dynamic contrast-enhanced imaging (DCE-MRI) in differentiating paragangliomas and metastases in the jugular foramen in combination with conventional imaging. METHODS: Forty-nine consecutive patients with paragangliomas or metastases between January 2015 and April 2022 were included in this retrospective study. All patients had pretreatment DWI and DCE-MRI. Between paragangliomas and metastases, normalized apparent diffusion coefficient (nADCmean) and DCE-MRI parameters were compared along with conventional imaging features (enhancement pattern, presence of flow voids, cystic/necrotic change, and bone erosion). The diagnostic performance was tested using receiver operating characteristic (ROC) analysis. RESULTS: Thirty-five paragangliomas (5 male; median 49 years) and 14 metastases (9 male; median 61 years) were analyzed. The most common 3 primary cancers included 4 lung cancers, 3 breast cancers, and 3 melanomas. The presence of flow void was significantly different between paragangliomas and metastases (21/35 vs 2/14; P = 0.0047) in conventional imaging features, while fractional plasma volume (Vp) was significantly different between the two tumor types (median 0.46 vs 0.19; P < 0.001) in DWI and DCE-MRI parameters. The areas under the ROC curves (AUCs) of the presence of flow void and Vp were 0.72 and 0.93, respectively. The AUC of the combination of the presence of flow void and Vp was 0.95 and significantly improved compared to that of the presence of flow void (P < 0.001). CONCLUSION: Adding DCE-MRI to the head and neck protocol can aid in the precise differentiation between jugular foramen paragangliomas and metastases.

Facial Nerve Schwannoma Extending to Jugular Foramen: A Case Report.

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.

Anaplastic Hemangiopericytoma of the Jugular Foramen: Case Report and Systematic Review.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Jugular Bulb Anomalies Involving the Internal Auditory Canal: A Case Series.

OBJECTIVE: Jugular bulb abnormalities (JBA) such as high riding jugular bulb and jugular bulb diverticulum can extend or erode into the middle and inner ear. In this report, we report on a series of 6 patients with jugular bulb anomalies involving the internal auditory canal (IAC). METHODS: A retrospective case series. RESULTS: Six females, ages 6 to 63 presenting with myriad of otologic symptoms including hearing loss, tinnitus, balance disturbance, fullness, and otalgia were discovered to have JB eroding into IAC. Computerized tomography, but not Magnetic Resonance Imaging, was able to identify IAC erosion by a significantly enlarged JB. CONCLUSION: A significantly enlarged JB eroding into the IAC maybe congenital or acquired. It can present with a variety of common otologic symptoms. Long term follow-up is needed to determine the natural history of JB anomalies involving the IAC and need for intervention.

Intraforaminal Dural Septations of the Jugular Foramen: A Cadaveric Study.

OBJECTIVE: The aim of this study was to define the types, prevalences, and diameters of dural septations (DSs) on the inner surface of the jugular foramen (JF) and to describe the distances between the JF, the glossopharyngeal nerve (cranial nerve [CN] IX), vagus nerve (CN X), and accessory nerve (CN XI), the internal acoustic meatus, and nearby surgical landmarks on cadaveric heads. METHODS: Seventeen adult (9 men and 8 women) formalin-fixed cadaveric heads were used to analyze the types and prevalence of DS bilaterally. Diameters and distances between the DS and the adjacent CNs (CN IX-XI) were measured by digital microcaliper. The multiple t test (SPSS version 25) was used to analyze the comparison between both sides via diameters, numbers, distance, length, and thickness of DS. RESULTS: The most frequent type of DS was type I (62.5%, right; 56.3%, left), followed by type II (18.8%, right; 25%, left), type III (12.5%, right; 6.3%, left), and type IV (6.3%, right; 12.5%, left). The mean diameter of the septum was 0.6-1 mm, and the mean length of the dural septa was 4.01 mm (right) and (3.83 mm) left. The difference in the length and thickness of the DS between the genders was statistically significant on both sides (P < 0.05). The DS-CN X and DS-JF distances of women were greater than those of men on the right side (P < 0.05). CONCLUSIONS: The significant differences between dural septum types on the 2 sides of the body may indicate asymmetric location or a variant emerging site of CNs in the same individual.

Intraoperative Monitoring of Auditory Function During Lateral Skull Base Surgery.

OBJECTIVE: We present a unique case of a patient with a jugular foramen tumor with serviceable hearing. This study discusses the audiometric results and intraoperative electrocochleographic (ECochG) findings recorded during tumor removal to illustrate the potential utility of this technique in skull base surgery. PATIENTS: A 22-year-old female patient presented with a jugular foramen schwannoma and associated symptoms of right-sided otalgia, mild hearing loss, and blurry vision. INTERVENTIONS: Intraoperative ECochG responses during an infratemporal fossa approach: click and tone burst (1, 2, 4 kHz) stimuli were used and presented at 90 dB nHL. MAIN OUTCOME MEASURES: Intraoperative ECochG testing using frequency-specific tone bursts and clicks before and after tumor resection. RESULTS: The compound action potential magnitudes, cochlear microphonic, and summation potential were recorded pre- and post-tumor removal. For statistical analysis, a paired t test with significance set at p < 0.05 was used. The compound action potential magnitudes increased at all test frequencies (p < 0.01) while the summation potential and cochlear microphonic remained relatively stable (p > 0.05). Audiometric testing demonstrated an improvement of the preoperative mild right-sided hearing loss after tumor resection (pure-tone average for 0.5, 1, 2, and 4 kHz of 30 dB HL preoperation and 7.5 dB HL after tumor resection). CONCLUSIONS: Intraoperative ECochG may allow for real-time monitoring during complex skull base surgery.

Jugular Foramen Metastasis from Lung Cancer: A Case of "A Mass without His Syndrome".

Jugular foramen (JF) metastasis is rare and often presents as JF syndrome. A 73-year-old male complained of left-sided mastoid pain that irradiated to the neck since the past 3 months. Onset of facial nerve (FN) palsy and persistence of the symptomatology despite corticosteroid therapy demanded radiologic evaluation. Computed tomography and magnetic resonance imaging showed a wide osteolytic lesion of the left JF with involvement of the third segment of the FN. The patient underwent transmastoid incisional biopsy. Histopathological examination showed an adenocarcinoma that was suggested to be of respiratory origin. A primary pulmonary lesion and metastasis to other sits were detected. The patient died 1 month after the initiation of the chemotherapy. Persistent mastoid pain and progressive FN palsy must be considered indicative of JF malignant lesions. Despite early diagnosis, secondary lesions of the JF are characterized by a poor prognosis; however, accurate diagnosis may avoid unnecessary aggressive surgery.

Meningeal Architecture of the Jugular Foramen: An Anatomic Study Using Plastinated Histologic Sections.

OBJECTIVE: This 3-dimensional histologic study aimed to provide a precise description of the meningeal structures in the jugular foramen. METHODS: 22 posterior skull base tissue blocks containing the jugular foramen region were obtained from 11 human cadaveric heads. These blocks were plastinated and cut into serial sections. After staining, these sections were examined under an optical microscope and used to reconstruct a 3-dimensional visualization model. RESULTS: At the intracranial orifice of the jugular foramen, the meningeal dura formed 2 separate dural perforations: the glossopharyngeal meatus and the vagal meatus. The arachnoid extended into 2 dural meatuses and terminated at the inferior ganglion of the glossopharyngeal nerve in the glossopharyngeal meatus and the superior ganglion of the vagus nerve in the vagal meatus. At the intraforaminal part of the jugular foramen, the meningeal dura encased the glossopharyngeal nerve to form a dural sheath while encasing the vagus and accessory nerves to form a dural network. At the extracranial orifice of the jugular foramen, the dural wall of the jugular bulb extended downward to form a dense connective tissue sheath. The initial end of the internal jugular vein invaginated into this sheath and fused with the jugular bulb. CONCLUSIONS: Knowledge of the anatomy of the meningeal architecture of the jugular foramen can be helpful in avoiding surgical complications of the lower cranial nerves when this complex area is approached.

Retrosigmoid Intradural Suprajugular Approach to Jugular Foramen Tumors with Intraforaminal Extension: Surgical Series of 19 Cases.

OBJECTIVE: The intraforaminal component of jugular foramen tumors is difficult to access surgically, as it requires complex approaches for radical removal and leads to a high recurrence due to residual tumor. The retrosigmoid suprajugular approach, intradural drilling of the roof of the jugular foramen, has been recently proposed for removal of such intraforaminal component without sacrificing the sigmoid-jugular venous system or requiring additional approaches. This study presents our experience with this approach and introduces the use of intraoperative continuous vagus nerve monitoring. METHODS: Nineteen patients (14 with neuromas and 5 with meningiomas) were operated using this approach over a 12.5-year period. In the more recent 14 cases, continuous vagus nerve monitoring was performed with a ball-type electrode placed on the proximal vagus nerve. RESULTS: More than 95% of the tumor removal was achieved in all but the first neuroma case. Extubation immediately after surgery and oral feeding within a week postoperatively was achieved in all patients. Seven of 9 patients with preoperative hearing disturbance symptomatically improved after surgery. All but the first case had no signs of recurrence during the follow-up period (average of 58 months). CONCLUSIONS: The retrosigmoid suprajugular approach is safe and effective for removal of tumors extending into the jugular foramen, maintaining a chance of hearing improvement. Intraoperative continuous vagus nerve monitoring is useful to avoid postoperative complications in such surgeries.