Glomus jugulare in a pediatric patient: case report and literature review.

PURPOSE: The jugular and tympanic glomus are rare neoplasms in the general population, being even more uncommon in the pediatric population. There is considerable morbidity associated with both disease and treatment. Treatment is essentially surgical, carried out in recent years in a multidisciplinary manner using preoperative embolization associated with microsurgery and eventually adjuvant radiotherapy. The outcome depends on the location of the lesion and its proximity to noble structures in addition to multidisciplinary monitoring in the postoperative period. METHODS: In this article, a literature review was carried out in the PubMed database, finding reports from 17 patients diagnosed with the disease. Only articles in English were considered. RESULTS: Moreover, we reported a case of a 14-year-old patient diagnosed with jugulotympanic glomus who underwent radical surgical treatment of the lesion. CONCLUSION: This is a rare case of jugulotympanic glomus in a pediatric patient, who underwent surgical treatment associated with multidisciplinary therapy, with a favorable postoperative outcome.

Treatment of patients with glomus jugulare tumours (GJT) and its subjective effect on quality of life (QoL) measures.

Cerebellopontine angle (CPA) tumours account for 6-10% of intracranial tumours. The most common CPA tumours are vestibular schwannomas (VS), also known as acoustic neuromas, benign tumours of the vestibulocochlear nerve. Less common but symptomatic skull base lesions are glomus jugulare tumours (GJT), of which approximately 40% are identified as CPA tumours. Initial symptoms for GJT may include hearing loss and tinnitus and progress to various cranial nerve dysfunctions. Three well-accepted treatment modalities for such tumours include surgical resection, radiotherapy and/or conservative management employing serial MR or CT imaging. Patients' quality of life may be impacted by different treatment methods, so treatment decisions should be client centered.

[Endovascular treatment of complex vascular pathology in neck and brain region. Case report]. / Komplex nyaki és agyi érpatológia endovascularis kezelése.

Authors present complex multistage endovascular treatment of female patient with an intradural infiltrative, inoperable tumor of the glomus jugulare, a stenosis of the internal carotid artery and an aneurysm of middle cerebri artery. In the literature our case report is the first announcement in which three pathology coexist ipsilateral in one patient and the patient has been treated step by step by endovascular procedures. Beside the endovascular treatment steps they display glomus jugulare tumor's symptoms, staging, treatment options and also the current treatment guidelines of internal carotid artery stenosis and intracranial aneurysms. Orv Hetil. 2017; 158(18): 706-710.

Papilledema and Vision Loss Caused by Jugular Paragangliomas.

Paragangliomas that arise from the jugular bulb are known to present as masses in the neck or with hearing loss, pulsatile tinnitus, and lower cranial nerve palsies. Much less recognized is their tendency to cause increased intracranial pressure and papilledema by obstructing jugular venous outflow. Only 7 such cases have been reported and with minimal ophthalmic documentation. We describe 3 more cases to provide additional documentation and to emphasize that paragangliomas are a potential cause of the pseudotumor cerebri syndrome, and that papilledema may be overlooked when a jugular paraganglioma is diagnosed or after it has been treated. Such lapses have led to disabling vision loss from damage to the optic nerves in long-standing papilledema.

Glomus jugulare: a rare cause of facial nerve palsy.

PURPOSE: To report a case of an unusual presentation of a glomus jugulare tumour. DESIGN: Case report. RESULTS: Glomus jugulare is a very rare brain tumour that usually presents with tinnitus, hearing loss, dysphagia and hoarseness. We report a case where this extremely rare diagnosis presented quite differently, with ipsilateral proptosis and subsequent facial nerve palsy. Furthermore, the sibling of the presented case had also been diagnosed with the same tumour. CONCLUSIONS: There have only been a few case reports in the literature describing facial nerve palsy in the context of a glomus jugulare tumour. This case highlights that although paragangliomas are exceedingly rare causes of facial palsy, they should be included in the differential diagnosis.

[A case of hemifacial spasm in a juglar foramen tumor patient treated by microvascular decompression].

Hemifacial spasm is a movement disorder characterized by involuntary paroxysmal chronic contractions of the facial musculature. The usual cause is simple vascular compression of the facial nerve, at its root exit zone of the brain stem. Previously only a case of hemifacial spasm associated with a juglar foramen tumor has been reported in the literature. In this article, we report a case in which hemifacial spasm accompanied an ipsilateral juglar foramen tumor in a 62-year-old woman. The sole use of arterial decompression of the facial nerve at the root exit zone resulted in complete resolution of the patient's symptoms.

Microsurgery via modified far-lateral approach for giant dumbbell-shaped jugular foramen tumors.

BACKGROUND AND OBJECTIVE: During the resection of jugular foramen tumors via the basic far lateral approach, the jugular foramen tumor area as well as its adjacent structures, especially the intracranial part, can be better exposed, which avoids stripping of the petrous part of temporal bone and displacement of facial nerve, and protects the patient's hearing from damage. However, when applied in tumors developed from ventral to the brain stem and middle fossa, with extracranial tumors, this surgical approach seems to be inadequate and limited. This study was to explore the microsurgical technique and clinical value for treating giant dumbbell-shaped tumors at jugular foramen (JF) via a modified far lateral approach. METHODS: A retrospective analysis was performed in 16 patients with huge dumbbell-shaped tumors at JF which were removed through the modified far lateral approach (suboccipital transjugular-jugular tubercle-jugular process) between January 2001 and December 2008. The process of operation, and pre-and postoperative clinical data were included in the analysis. RESULTS: Gross total tumor removal was achieved in 14 cases, subtotal removal in 1 case, and partial removal in 1 case. Follow-up examinations in most patients demonstrated that the patient with an obvious preoperative deficit had a good recovery. During the follow-up from three months to seven years, 10 (76.9%) cases with lower cranial nerve involvement showed obvious improvement of symptom after operation, 8 (80.0%) cases with facial palsy obtained various degrees of alleviation, and 7 (77.8%) cases with hearing impairment at different levels restored hearing. Two patients developed new lower cranial nerve palsies after operation, and underwent functional rehabilitation in the three-month follow-up. CONCLUSIONS: Modified far lateral approach is helpful for removing the huge tumors at JF, especially for tumors extending to the petroclival region ventral part of pontomedullary junction. It has a higher rate of total resection, preoperative cranial nerve function impairment is expected to restore, and also has the advantage of protecting the facial nerve, labyrinth and vertebral artery structure from unnecessary damage.

[Glomus tumor with diplopia]. / Diplopie prin tumora glomica--caz clinic.

This paper highlights the case of a 46-year-old female patient who seeks ophthalmic medical advice for her complaint about double vision on right gaze. Patient's medical history reveals several symptoms like headaches, progressive hearing loss on right side and dysphonia for which all investigations proved irrelevant. Thourough clinical examination, lab exams and imaging techniques revealed the cause for this symptoms i.e. glomic tumour with middle ear extention. We find this case to be exceptional as ocular symptoms led to the diagnose of a non-ocular serious, possible life threatening condition, bearing in mind that previously occured non-ocular symptoms couldn't elucidate the cause.

Involvement of the Cochlear Aqueduct by Jugular Paraganglioma Is Associated With Sensorineural Hearing Loss.

OBJECTIVE: The etiology of sensorineural hearing loss (SNHL) in patients with jugular paraganglioma (JP) whose tumors lack inner ear fistulae or vestibulocochlear nerve involvement is unknown. Recent literature has proposed that occlusion of the inferior cochlear vein may be causative. Herein, we assess the association between radiologic involvement of the cochlear aqueduct (CA) and the development of SNHL. STUDY DESIGN: Blinded, retrospective review of imaging and audiometry. SETTING: Tertiary center. PATIENTS: Adults with JP. INTERVENTION(S): None. MAIN OUTCOME MEASURES: Asymmetric SNHL was assessed continuously as the difference in bone conduction pure-tone average (BCPTA) between ears and as a categorical variable (≥15 dB difference at two consecutive frequencies, or a difference in speech discrimination score of ≥15%). Involvement of the CA was considered present if there was evidence of medial T2 fluid signal loss, contrast enhancement, or bony erosion/expansion. RESULTS: Of 30 patients meeting inclusion criteria, 15 (50%) had asymmetric SNHL. CA involvement was observed in 87% of patients with asymmetric SNHL compared with 13% in those with symmetric hearing (p = 0.0001). Univariate analysis demonstrated that age, sex, and tumor volume were not associated with asymmetric SNHL. The median difference in BCPTA between ears in patients with CA involvement was 21.3 dB HL compared to 1.2 dB HL in those without CA involvement (p < 0.0001). Regression analysis demonstrates that enhancement within the CA is associated with a BCPTA difference of 19.4 dB HL (p = 0.0006). CONCLUSIONS: Cochlear aqueduct involvement by JP is associated with SNHL in the absence of inner ear fistula, vestibulocochlear nerve involvement, or brainstem compression. Correlation with operative findings or histopathologic evidence of tumor involvement may validate this intriguing imaging finding.

Jugular foramen tumour resulting in hypoglossal denervation pseudohypertrophy: a rare and significant cause for tongue asymmetry.

Paragangliomas of the jugular foramen are rare. They may present with symptoms of compression of the glossopharyngeal or vagus nerves, or due to secretion of catecholamines from chromaffin cells within the tumour. This case describes a rare presentation of glomus tumour. A 67-year-old patient presented with a 2-month history of right-sided tongue swelling. She was found to have an obvious swelling on the right side of the tongue but no obvious weakness or fasciculation on initial examination. Ultrasound confirmed diffuse muscle swelling, but no lesion within the tongue. Magnetic resonance imaging of the neck revealed an ipsilateral glomus jugulare tumour that extended to the hypoglossal canal, and had resulted in ipsilateral denervation pseudohypertrophy of the lingual muscles. This paper reviews presentation of glomus jugulare tumours and contributes a novel presentation of a rare entity.

Pathophysiology of sensorineural hearing loss in jugular foramen paraganglioma.

OBJECTIVES/HYPOTHESIS: Pathologic involvement of the inferior cochlear vein is a mechanism of sensorineural hearing loss in patients with jugular foramen paraganglioma. STUDY DESIGN: Retrospective case-control study. METHODS: The presenting audiograms, magnetic resonance imaging, and computed tomography were reviewed in 46 subjects with jugular foramen paragangliomas. Four-frequency bone conduction average was compared between the tumor and nontumor ears in each subject to establish the presence of sensorineural hearing loss. Imaging findings for each subject were recorded. Univariate and multivariate statistical analyses were performed to determine which radiographic features were associated with sensorineural hearing loss. Hearing data were analyzed as a continuous variable and as a categorical variable. RESULTS: Twenty subjects (43.4%) had a bone-conduction pure-tone asymmetry of greater than 15 dB. Inferior cochlear vein involvement was identified in 19 of the 20 (95%) subjects with sensorineural hearing loss. Inferior cochlear vein involvement was found to be a statistically significant predictor of sensorineural hearing loss using univariate and multivariate analyses. Other imaging findings that were statistically significant predictors of sensorineural hearing loss include Glasscock-Jackson stage, Fisch-Mattox stage, hypoglossal canal involvement, jugulo-carotid spin erosion, and petrous carotid canal erosion. CONCLUSIONS: Involvement of the inferior cochlear vein appears to be a plausible mechanism for sensorineural hearing loss in patients with jugular foramen paraganglioma. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:67-75, 2019.

Dural arteriovenous fistula associated with a glomus jugulare tumour presenting with only pulsatile tinnitus.

We present the second known case of a dural arteriovenous fistula (DAVF) associated with a glomus jugulare tumour in a 66-year-old man and the first with a presenting symptom of pulsatile tinnitus. The tumour occluded the left internal jugular vein at the bulb. Our patient opted for monitoring, but the tinnitus progressed and became debilitating, prompting him to proceed with embolisation of the tumour. Angiography revealed a DAVF of the left transverse sinus with retrograde flow. Embolisation of 80% of the tumour did not relieve symptoms. The patient returned for embolisation of the DAVF. Occlusion of the DAVF achieved symptomatic relief. A quandary develops during a procedure when the surgeon discovers that another intervention could satisfy the patient, while the patient is under anaesthesia. The higher flow in the DAVF likely causes the tinnitus in those with a patent sigmoid sinus, and embolisation of the DAVF alone could achieve relief.

Facial paralysis associated with glomus jugulare tumors.

OBJECTIVE: To review the intraoperative findings and facial nerve management in nine patients who presented with facial paralysis associated with glomus jugulare tumors. STUDY DESIGN: A retrospective analysis of patient medical records. SETTING: Tertiary care academic medical center. PATIENTS: All patients who presented with facial paralysis and a glomus jugulare tumor who underwent surgical resection of their tumors at our institution. INTERVENTION: A postauricular infratemporal fossa approach for tumor removal and greater auricular interposition neural repair. MAIN OUTCOME MEASURE: Intraoperative facial nerve findings and long-term facial recovery. RESULTS: One hundred two patients underwent a postauricular infratemporal approach for resection of glomus jugulare tumor from July 1988 through July 2005. Nine of these patients presented with ipsilateral facial paralysis. The medial surface of the vertical segment was invaded by tumor in all nine cases. Facial recovery at 2 years was House-Brackmann Grade III in eight patients and Grade IV in one individual. Facial recovery did not significantly change after 2 years (mean follow-up of 7.4 years). DISCUSSION: Facial nerve invasion of the vertical segment occurred in 9 (9%) of 101 patients in our series. Facial nerve resection with interposition grafting resulted in House-Brackmann Grade III in eight (89%) of nine patients. Facial nerve dissection and preservation was not possible when preoperative facial paralysis was evident.

[Glomus tumours of temporal bone origin. Study of 17 cases]. / Tumores glómicos del hueso temporal. Estudio de 17 casos.

OBJECTIVE: The objective of this paper is to make a retrospective analysis in patients with glomus tumours of temporal bone origin. We present the results according to the surgical approach applied in each case. PATIENTS AND METHOD: This retrospective study presents the findings in 17 patients with diagnosis of glomus jugulare of the temporal bone, who were observed and treated in our department over a 5-year period (1999 to 2004). We performed a general otolaryngology exam, systemic evaluation and radiological exam. Surgical treatment was performed in 16 cases out of 17. In 1 case treatment with stereotaxic surgery was performed. RESULTS: The surgical approaches were: retroauricular transcanal approach, radical or modified mastoidectomy through facial recess, and infratemporal fossa approach. Pre-operative embolization was used in 11 of our cases. In all cases the diagnosis of glomus tumour was confirmed. The most frequent post-operative complications found were: transitory paralysis of the facial nerve, sensorineural hearing loss, imbalance, paralysis of the IXth and XIth cranial nerves, and salivary fistula. No recurrences were found after 8 years of follow-up. One case of persistence was found in the case treated with radiosurgery. CONCLUSIONS: In our series surgery was found as the elective therapy for patients with glomus tumour of the temporal bone with no recurrences after 8 years of follow-up. Pre-operative embolization diminishes surgery time and intraoperative bleeding. Stereotaxic therapy cannot provide tumour growth control. Complications are discussed and compared with the bibliography.

Intracranial glomus jugulare tumors: volume reduction with Gamma Knife surgery.

OBJECT: Glomus jugulare tumors (GJTs) are slow-growing benign tumors arising from paraganglion cells of the superior vagal ganglion. Involvement of cranial nerves and extensive erosion of the jugular foramen and petrous bone are typically seen in patients with GJTs. Advances in microsurgical techniques have improved patient outcomes, but tumors involving the petrous bone remain difficult to treat effectively. The aim of our study was to further evaluate the role of Gamma Knife surgery (GKS) in the management of intracranial GJTs. METHODS: Twelve consecutive patients (mean age 51.7 years) with intracranial GJTs were included in this study. The treatment strategy was either multimodal, with microsurgical tumor volume reduction followed by GKS in patients suffering from brainstem compression, or GKS as the only treatment. Follow-up examinations included thorough neurological examinations and neuroradiological quantitative volumetric tumor analysis. Five patients (41.6%) underwent microsurgery before GKS. Tumor volumes ranging from 1.6 to 24.8 cm3 were treated using prescription doses of 14 to 20 Gy (nine-28 isocenters). The achieved overall tumor control rate after GKS was 100% (33 months mean follow up) with only mild side effects observed. A tumor volume reduction (mean 41.1%; 3.2 cm3) was achieved in all patients. CONCLUSIONS: Gamma Knife surgery is a safe and effective treatment for intracranial GJTs. The tumor volume reductions achieved are comparable to those achieved using microsurgery but with a much lower rate of side effects. More studies with longer follow-up times are necessary to confirm these very promising results.

Radiosurgery for glomus jugulare tumors: experience treating 16 patients in Iran.

OBJECT: Glomus jugulare tumors (GJT) have traditionally been treated by surgery or fractionated external-beam radiotherapy. The aim of this retrospective study was to determine the tumor control rate, clinical outcome, and short-term complications of stereotactic radiosurgery in subsets of patients who are poor candidates for these procedures, based on age, medical problems, tumor size, or prior treatment failure. METHODS: The Leksell Gamma Knife was used to treat 16 patients harboring symptomatic, residual, recurrent, or unresectable GJTs. The age of the patients ranged from 12 to 77 years (median 46.5 years). Gamma Knife surgery (GKS) was performed as primary treatment in five patients (31.3%). Microsurgery preceded radiosurgery in 10 patients (62.5%) and fractionated radiotherapy in three patients (18.8%). The median tumor volume was 9.8 cm3 (range 1.7-20.6 cm3). The median marginal dose applied to a mean isodose volume of 50% (range 37-70%) was 18 Gy (range 14-20 Gy). Neurological follow-up examinations revealed improved clinical status in 10 patients (62.5%), a stable neurological status in six (37.5%), and no complications. After radiosurgery, follow-up imaging was conducted in 14 patients; the median interval from GKS to the last follow up was 18.5 months (range 4-28 months). Tumor size had decreased in six patients (42.9%), and the volume remained unchanged in the remaining eight (57.1%). None of the tumors increased in volume during the observation period. CONCLUSIONS: According to the authors' experience, GKS represents a useful therapeutic option to control symptoms and may be safely conducted in patients with primary or recurrent GJTs with no death and no acute morbidity. Because of the tumor's naturally slow growth rate, however, long-term follow-up data are needed to establish a cure rate after radiosurgery.

[Paralysis of vocal fold as the first symptom of Vernet's syndrome in the course of jugular chemodectoma]. / Porazenie faldu glosowego jako pierwszy objaw zespolu Verneta w nastepstwie klbczaka otworu zyly szyjnej.

INTRODUCTION: Chemodectomas are relatively frequent tumors of the head and neck but their diagnosis in consideration of the slow growth is difficult. The aim of this study is pointing out of the attention on: (1) non-typical beginning of ill and diagnostic difficulties leading to delaying of putting the proper diagnosis, (2) symptoms which are cause of notifying the patient to the doctors of different specializations e.g. the laryngologist and the neurologist. MATERIAL AND METHODS: The case of 72-year-old man with the paraganglioma situated near to the foramen of the jugular vein is discribed. Clinical symptoms, giving at last typical picture of Vernet's syndrome, the diagnostic procedure as well as treatment of the entity are discussed in the report. CONCLUSIONS: (1) In cases of paralysis of any cranial nerve is necessary close cooperation among a laryngologist and a neurologist. (2) In idiopathical paralysis of the vocal fold, changes runing near by the internal jugular vein's foramen should be taken into account. (3) The computer scanning of skull's bases and angiograpy are the most useful in the differential diagnostics. (4) The radiotherapy allow to obtain good therapeutic's results especially in persons stricken in years.

Systemic Inflammatory Syndrome Associated with a Case of Jugular Paraganglioma.

Jugular paraganlioma is a benign, slow-growing tumor originating from the paraganglion cells and it is associated with catecholamine secretion. Paragangliomas can secrete Interleukin-6 (IL-6) and present as a systemic inflammatory syndrome; these characteristics have not been previously associated with jugular paragangliomas. A 63-year-old man with a jugular tumor in the skull base was referred to our hospital for an evaluation of pyrexia, back pain, and acute inflammation. His serum IL-6 level was elevated on admission and it decreased after radiotherapy. This is the first known case of a jugular paraganglioma exhibiting systemic inflammatory syndrome.

Jugulotympanic paraganglioma: a rare cause of vertigo.

BACKGROUND: Jugulotympanic paraganglioma generally presents in the 5th or 6th decades of life with tinnitus and hearing loss. In this manuscript, we present a rare case of jugulotympanic paraganglioma presenting in the 9th decade with vertigo as the most bothersome symptom. CASE REPORT: An 83-year-old woman presented with worsening episodes of dizziness of a few months duration. She also complained of tinnitus and hearing loss, more severe on the left side. Examination revealed a red bulging left-sided tympanic membrane, conductive hearing loss, and a bruit at the base of the skull. Dix-Hallpike test was negative. CT head and MRI brain revealed findings consistent with a large left-sided jugulotympanic paraganglioma, which was found to be hormonally inactive on laboratory tests. The patient underwent treatment with radiotherapy, which resulted in partial improvement of symptoms. CONCLUSIONS: Jugulotympanic paraganglioma may manifest in the elderly with the chief complaint of intermittent vertigo, as in our case. A red bulging mass on otoscopy raises the suspicion, necessitating further investigations, including CT and MRI.

Radiation induced brainstem glioblastoma in a patient treated for glomus jugulare tumour.

The intensive utilisation of cranial irradiation in young individuals with benign intracranial pathologies are of particular concern because of the potential for delayed development of radiation-induced neoplasms. We present a 48-year-old man who developed a second metachronous brainstem glioblastoma 10 years following adjuvant radiotherapy for a partially resected glomus jugulare tumour. The current patient highlights the importance of judicious and individualised consideration for irradiation treatment in benign pathologies that are associated with long-term survival.