Collet-Sicard syndrome is a rare neurological disorder caused by injury to the cranial nerve pairs IX, X, X, and XII. The author reports on a previously fit 27-year-old man who presented with dysphagia, choking on drinking water, hoarseness, weakness when turning the neck and shrugging the shoulders, and unexplained weight loss. Enhanced magnetic resonance imaging indicated a space-occupying lesion at the right jugular foramen. After surgical resection, the pathologic findings suggested a paraganglioma of the right jugular foramen and confirmed the diagnosis of Collet-Sicard syndrome. After postoperative treatment with a combination of acupuncture and modern medicine, the patient\'s symptoms significantly improved. This article analyzes previous literature regarding Collet-Sicard syndrome etiology and reports the case of a patient with a rare etiology, whose prognosis improved significantly after treatment with a combination of acupuncture and modern medicine.

BACKGROUND: Jugular foramen dural arteriovenous fistulas (DAVFs) are rare and challenging lesions. Described methods of treatment include embolization and microsurgical disconnection through a far lateral transcondylar approach. The authors present the case of a Borden type III jugular foramen DAVF, which was treated with a novel, less invasive retrosigmoid approach with intradural skeletonization and packing of the sigmoid sinus.
METHODS: The patient presented with headache and visual field deficit. Neuroimaging demonstrated a right temporal intracerebral hematoma with mass effect. This was due to a Borden type III jugular foramen DAVF with cortical venous reflux into the vein of Labbe secondary to recanalization of a previously thrombosed sigmoid sinus. Microsurgical disconnection was performed via a retrosigmoid approach, in which the sigmoid sinus was identified intradurally at the jugular foramen. The sigmoid sinus was isolated by drilling at the pre- and retrosigmoid spaces to permit packing and clip ligation. Postoperative angiography revealed complete occlusion of the DAVF.
CONCLUSIONS: Jugular foramen DAVFs are rare entities, which have been traditionally treated through a far lateral transcondylar approach. An intradural retrosigmoid approach is a safe, less invasive alternative, which involves less soft tissue and bony dissection and does not have the associated morbidity of craniocervical instability and hypoglossal neuropathy.

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.

Jugular foramen tumours are uncommon, deeply located, and eloquently situated, making their diagnosis and management challenging. Paragangliomas and other benign tumours comprise the large majority of lesions in this region, but malignant tumours are occasionally identified. We report a unique case of a solitary plasmacytoma of the jugular foramen resembling a jugulotympanic paraganglioma. A solitary plasmacytoma of the jugular foramen is both rare in location and in disease presentation, as most plasma cell neoplasms are diagnosed as multiple myeloma. Our 75-year-old patient presented with symptoms typical for a jugular foramen tumour. Although there are radiographic features which help differentiate paragangliomas from other benign and malignant tumours, plasmacytomas are highly vascular and can demonstrate a local infiltrative spread which can mimic the radiographic appearance of a paraganglioma. Clinicians should consider plasma cell neoplasms in the differential when faced with an unusual presentation of a jugular foramen lesion. Our patient was treated with definitive radiotherapy to 45 Gy, which was very effective local treatment for the solitary plasmacytoma.

Objective  We describe the first jugular foramen angiomatoid fibrous histiocytoma (AFH) case and the first treatment with preoperative endovascular embolization. AFH is a rare intracranial neoplasm, primarily found in pediatric patient extremities. With an increase in AFH awareness and a well-described genetic profile, intracranial prevalence has also subsequently increased. Study Design  We compare this case to previously reported cases using PubMed/Medline literature search, which was performed using the algorithm [\"intracranial\" AND \"angiomatoid fibrous histiocytoma\"] through December 2020 (23 manuscripts with 46 unique cases). Patient  An 8-year-old female presented with failure to thrive and right-sided hearing loss. Work-up revealed an absence of right-sided serviceable hearing and a large jugular foramen mass. Angiogram revealed primary arterial supply from the posterior branch of the ascending pharyngeal artery, which was preoperatively embolized. Intervention  Gross total resection was performed via a translabyrinthine approach. Conclusion  The case presented is unique; the first reported AFH at the jugular foramen and the first reported case utilizing preoperative embolization. Preoperative embolization is a relatively safe technique that can improve the surgeon\'s ability to perform a maximally safe resection, which may decrease the need for adjuvant radiation in rare skull base tumors in young patients.

BACKGROUND: Paragangliomas (PGLs) are rare neoplasms that may be associated with hereditary PGL syndromes and variable risk of metastasis. Middle ear adenomas are extremely rare tumors with no known hereditary predisposition and extremely low risk of metastasis. Although often easily differentiated, they may share clinical and pathological features that misdirect and confuse the diagnosis.
METHODS: The authors discussed a 35-year-old woman with left-sided hearing loss and bleeding from the external ear canal who presented to an outside hospital. She underwent resection of a middle ear and mastoid mass, initially diagnosed as a middle ear adenoma with neuroendocrine features, with later mastoidectomy and ligation of the sigmoid sinus with microsurgical excision of persistent tumor in the jugular foramen and temporal bone. Histopathologically, her tumor was vascular, composed of benign-appearing epithelioid cells with \"salt and pepper\" neuroendocrine chromatin arranged in vague nests. Lesional cells were GATA3-immunopositive, glucagon-negative, and succinate dehydrogenase-immunonegative, consistent with PGL rather than middle ear adenoma, and required further workup for hereditary PGL syndromes.
CONCLUSIONS: This case demonstrates potential challenges in differentiating a PGL from a middle ear adenoma. The authors offer clinical, histopathological, and imaging principles to aid in diagnosis and workup.

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: The jugular foramen (JF) can be the site of several tumours. Paragangliomas, schwannomas and meningiomas are the most commonly reported. We describe a case of melanocytoma originating from the JF and presenting with an accessory nerve palsy.
UNASSIGNED: A 48-year-old woman presented with a 6-month history of cervical and left shoulder pain with wasting and weakness of the left trapezius. A Magnetic Resonance Imaging (MRI) showed a T1-hyperintense, T2-isointense, heterogeneously enhancing lesion involving the left JF and extending into the cerebello-medullary and cerebello-pontine cisterns. A retrosigmoid craniotomy was performed and a near-total removal achieved. The accessory nerve was involved by tumour and could not be preserved. Given the diagnostic uncertainty between melanotic schwannoma, metastatic melanoma and meningeal melanocytoma, next generation sequencing and genome-wide DNA methylation arrays were performed, documenting a mutation in GNA11 (c.6226A>T, p. Gln209Leu) and a methylation profile consistent with melanocytoma. The patient underwent adjuvant fractionated radiotherapy of the tumour remnant. A follow-up MRI 4 years after surgery did not show any tumour recurrence.
CONCLUSIONS: The differential diagnosis of skull base pigmented tumours can be challenging, particularly when they occur in unusual locations such as the JF. They can be misdiagnosed given their similar clinical, neuroradiological and pathological features if anatomy of the site of origin is not carefully considered and molecular tests are not performed, leading to erroneous treatment and follow-up planning.

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.

The posterior inferior cerebellar artery (PICA) rarely arises from the cavernous segment of the internal carotid artery (ICA) and is called persistent trigeminal artery variant. The PICA also can arise from the cervical segment of the ICA, and it enters the posterior fossa via the hypoglossal canal, where it is called persistent hypoglossal artery variant. Using magnetic resonance angiography (MRA), we diagnosed a 79-year-old man with a PICA arising from the ascending pharyngeal artery and passing through the medial side of the jugular foremen pars vascularis. Only six cases of this variation have been reported previously in the English language literature. To identify this variation on MRA, the careful observation of source images is useful. Recognizing this variation is important in order to avoid ischemic cerebellar complications during neck surgery and endovascular therapy.