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.

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.

[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.

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.

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.

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.

Lesions involving the jugular foramen: clinical characteristics and surgical management.

CONCLUSION: Lesions involving the jugular foramen (JF) present as various diagnoses. Pulsatile tinnitus is more common in glomus jugulare (GJ) tumors, whereas otalgia and facial nerve paresis are more prevalent in temporal bone malignancies (TBMs). Preoperative facial nerve electroneurography (ENoG) was significantly correlated with postoperative facial nerve function. OBJECTIVE: To describe the diagnosis and surgical management of lesions involving the JF. METHODS: The charts were reviewed for 38 patients who had lesions involving the JF, including 14 patients with TBMs, 11 with GJ tumors, 7 with cholesteatomas, 2 with facial nerve schwannomas, 2 with JF schwannomas, and 2 with cholesterol granulomas. The follow-up data were recorded. RESULTS: The most frequent symptoms included hearing loss (89.47%), followed by otorrhea (47.37%) and pulsatile tinnitus (39.47%). With respect to TBMs, 57.14% of patients complained of otorrhea and otalgia, and 50.00% presented with facial nerve paresis. Among the 13 patients with facial nerve paresis, 53.85% were diagnosed with TBMs. Regarding GJ tumors, 81.82% had pulsatile tinnitus and hearing loss. Among the 25 patients with normal preoperative facial nerve function, the mean facial nerve ENoG reduction was 29.48 ± 29.15%, and the mean postoperative facial nerve score was 77.48 ± 33.13. The correlation coefficient was -0.973 (p = 0.000).

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.

Hypoglossal canal invasion by glomus jugulare tumors: clinico-radiological correlation.

The aim of this retrospective study is to assess the rate at which glomus jugulare tumors invade the hypoglossal canal (HC) and to correlate computed tomography (CT) and magnetic resonance imaging (MRI) findings with the clinical evidence of cranial nerve (CN) XII dysfunction. CT and MRI imaging modalities of 31 patients were blindly reviewed by an attending neuroradiologist. Imaging studies identified involvement in 22 tumors (22/31, 71.0%). Thirteen of 22 patients (59.1%) had clinically evident CN XII symptoms. Accuracy rate was 76.7% (23/30) for MRI and 78.6% (11/14) for CT. MRI showed 100% sensitivity but had only 59% specificity and the specificity for CT was 66.7%. When radiologists elucidate HC involvement, it may alter the surgical approach and may lead to more focused/accurate clinical evaluation of tongue function.

Jugulotympanic paragangliomas treated with Gamma Knife radiosurgery: a single-center review of 58 cases.

OBJECT: Jugulotympanic paragangliomas (JTPs) are rare benign tumors whose surgical treatment is usually associated with partial resection of the lesion, high morbidity, and even death. Gamma Knife radiosurgery (GKRS) has been reported as a useful treatment option. The goal of this retrospective study is to analyze the role of GKRS in tumor volume control and clinical outcomes of these patients. METHODS: A total of 75 patients with JTPs were treated with GKRS at the authors' center from 1995 to 2012. The authors analyzed those treated during this period to allow for a minimal observation time of 2 years. The MR images and clinical reports of these patients were reviewed to assess clinical and volumetric outcomes of the tumors. The radiological and clinical assessments, along with a group of prognostic factors measured, were analyzed using descriptive methods. The time to volumetric and clinical progression was analyzed using the Kaplan-Meier method. Prognostic factors were identified using log-rank statistics and multivariate Cox regression models. RESULTS: The mean follow-up was 86.4 months. The authors observed volumetric tumor control in 94.8% of cases. In 67.2% of cases, tumor volume decreased by a mean of 40.1% from the original size. Of patients with previous tinnitus, 54% reported complete recovery. Improvement of other symptoms was observed in 34.5% of cases. Overall, clinical control was achieved in 91.4% of cases. Previous embolization and familial history of paraganglioma were selected as significant prognostic factors for volumetric response to GKRS treatment in the univariate analysis. In multivariate analysis, no factors were significantly correlated with progression-free survival. No patient died of side effects related to GKRS treatment or tumor progression. CONCLUSIONS: Gamma Knife radiosurgery is an effective, safe, and efficient therapeutic option for the treatment of these tumors as a first-line treatment or in conjunction with traditional surgery, endovascular treatment, or conventional fractionated radiotherapy.

Evaluation of linear accelerator-based stereotactic radiosurgery in the management of glomus jugulare tumors.

AIMS AND BACKGROUND: Although mostly benign and slow-growing, glomus jugulare tumors have a high propensity for local invasion of adjacent vascular structures, lower cranial nerves and the inner ear, which may result in substantial morbidity and even mortality. Treatment strategies for glomus jugulare tumors include surgery, preoperative embolization followed by surgical resection, conventionally fractionated external beam radiotherapy, radiosurgery in the form of stereotactic radiosurgery or fractionated stereotactic radiation therapy, and combinations of these modalities. In the present study, we evaluate the use of linear accelerator (LINAC)-based stereotactic radiosurgery in the management of glomus jugulare tumors and report our 15-year single center experience. METHODS AND STUDY DESIGN: Between May 1998 and May 2013, 21 patients (15 females, 6 males) with glomus jugulare tumors were treated using LINAC-based stereotactic radiosurgery at the Department of Radiation Oncology, Gulhane Military Medical Academy. The indication for stereotactic radiosurgery was the presence of residual or recurrent tumor after surgery for 5 patients, whereas 16 patients having growing tumors with symptoms received stereotactic radiosurgery as the primary treatment. RESULTS: Median follow-up was 49 months (range, 3-98). Median age was 55 years (range, 24-77). Of the 21 lesions treated, 13 (61.9%) were left-sided and 8 (38.1%) were right-sided. Median dose was 15 Gy (range, 10-20) prescribed to the 85%-100% isodose line encompassing the target volume. Local control defined as either tumor shrinkage or the absence of tumor growth on periodical follow-up neuroimaging was 100%. CONCLUSIONS: LINAC-based stereotactic radiosurgery offers a safe and efficacious management strategy for glomus jugulare tumors by providing excellent tumor growth control with few complications.