Glomus Jugulare Tumors Research Articles

Report of Four Cases of Misdiagnosis About Glomus Jugular Tumor

Aim To raise awareness of glomus jugular tumor and decrease misdiagnosis and mistreatment rate. Method Reviewing, summarizing and analyzing the clinical data of four misdiagnosed patients visiting our hospital from July, 2008 to September, 2012. Outcome The four misdiagnoses are respectively and successively secretory otitis media, cerebral infarction and cholesteatoma of external auditory meatus. Conclusion As the symptom and signs of glomus jugular tumor apt to be confused with other diseases, clinician’s lack of knowledge would cause misdiagnosis and mistreatment.

Glomus jugulare tumours: A 15 year radiotherapy experience in South Australia

Glomus jugulare tumours (GJT) are one of a family of benign hypervascular neoplasms that arise from chief cells of the paraganglionic tissue from the dome of the jugular bulb. Historically, these tumours have primarily been managed surgically but radiation is an alternative treatment modality. The purpose of this retrospective review was to determine the patient survival, tumour control, clinical control rates and long term toxicity of GJT treated with radiation in South Australia. Between 1996 and 30 June 2012, 15 patients with GJT (16 tumours) were managed with radiotherapy. Twelve patients were female and the median age was 62years. Thirteen patients (87%) were treated with conventional external beam radiotherapy and two patients (13%) with stereotactic radiosurgery. The mean duration of follow-up was 4years (range 1month–15years 4months). The close-out date for survival analyses was 31 October 2012. Crude overall survival, tumour control, clinical control and long term grade >2 toxicity rates were 100%, 94% (15/16), 94% and 7% (1/16), respectively. The Kaplan-Meier 5 and 10year clinical and tumour control rates were both 93% (95% confidence interval: 61–99%). Radiation is an effective treatment modality for GJT. With comparable patient survival, arguably improved tumour and clinical control rates relative to surgery, minimal toxicity and ongoing advances in radiotherapy technologies, radiation should be strongly considered for the primary management of GJT.

Less invasive transjugular approach with Fallopian bridge technique for facial nerve protection and hearing preservation in surgery of glomus jugulare tumors

For the past three decades, surgery of glomus jugulare tumors (GJTs) has been characterized by extensive combined head and neck, neuro-otologic, and neurosurgical approaches. In recent years, the authors have modified the operative technique to a less invasive approach for preservation of cranial nerves while achieving satisfactory tumor resection. We evaluated and compared the clinical outcomes of our current less invasive approach with our previous more extensive procedures. The clinical records of 39 cases of GJT surgically treated between 1992 and 2011 were retrospectively reviewed. The less invasive transjugular approach with Fallopian bridge technique (LI-TJ) was used for the most recent five cases. The combined transmastoid-transjugular and high cervical (TM-HC) approach was performed in 30 cases, while four cases were treated with a transmastoid-transsigmoid approach with facial nerve translocation. Operative technique, extent of tumor resection, operating time, hospital stay, and morbidity were examined through the operative records, and a comparison was made between the LI-TJ cases and the more invasive cases. No facial nerve palsy was seen in the LI-TJ group while the TM-HC group demonstrated six cases (17.6%) of facial palsy (House-Brackmann facial nerve function grading scale grade II and III). The complication rate was 0 % in the LI-TJ group and 16.7% in the more invasive group. The mean operative time and hospital stay were shorter in the LI-TJ group (6.4 h and 4.3 days, respectively) compared with the more invasive group (10.7 h and 8.0 days, respectively). The LI-TJ approach with Fallopian bridge technique provided adequate tumor resection with cranial preservation and definitive advantage over the more extensive approach.

Successful Management of a Catecholamine-Secreting Glomus Jugulare Tumor with Radiosurgery Alone

Secretory glomus jugulare tumors are often resected surgically to control the systemic effects of the catecholamines they produce. This involves complex skull base surgery, which carries significant risks and frequent morbidity. Stereotactic radiosurgery (SRS) is a recently recognized treatment for glomus jugulare tumors, though little is known about its use in secretory tumors. Case Report A young fit patient with a catecholamine-secreting glomus jugulare tumor was treated with SRS alone and over the following 37 months her urinary catecholamine excretion fell to near normal levels, and serial magnetic resonance imaging (MRI) confirmed a reduction in tumor volume. Discussion Radiosurgery is an accepted treatment for glomus jugulare tumors and is now readily available to skull base surgeons. In this case a catecholamine-secreting tumor was successfully controlled with radiosurgery alone. Further research and long-term follow-up will determine the role of this treatment in the nonsurgical management of secreting glomus jugulare tumors.

Robotic Stereotactic Radiosurgery in Patients with Unresectable Glomus Jugulare Tumors

We evaluated the treatment results of robotic stereotactic radiosurgery (SRS) in our patients with unresectable glomus jugulare tumors (GJTs). The medical charts of fourteen patients with GJT, who were treated with robotic SRS, were retrospectively evaluated. The gross tumor volume was described as the clinical target volume. The median dose to the tumor was 25 Gy in median 5 fractions. The dose was normalized to 80% isodose line. All patients were evaluated for tumor growth and clinical outcome every 6 months in the first 2 years and then annually. Median follow-up was 39 months (range, 7-60 months). Lesions were stable in 8 patients, and tumor regression was observed in 6 patients. We did not observe any treatment related toxicity in our patients. In conclusion, according to our early experience, robotic SRS seems to be successful treatment option in the management of unresectable GJTs.

Stereotactic radiosurgery for the treatment of Glomus Jugulare Tumors

Background:The glomus jugulare tumor is a slowly growing benign neoplasm originating from neural crest. There is a high morbidity associated with surgical resection of glomus jugulare. Radiosurgery play a relevant role as a therapeutic option in these tumors and its use has grown in popularity. The authors describe a retrospective series of 15 patients and reviewed the literature about the glomus jugulare tumors.Methods:We reviewed retrospectively the data of 15 patients treated with stereotactic linear accelerator stereotactic radiosurgery (LINAC) radiosurgery between 2006 and 2011.Results:The average tumor volume was 18.5 cm3. The radiation dose to the tumor margin ranged between 12 and 20 Gy. The neurological status improved in three patients and remained unchanged in 12 patients. One patient developed a transient 7th nerve palsy that improved after clinical treatment. All tumors remained stable in size on follow-up with resonance magnetic images.Conclusions:The radiosurgery is a safe and effective therapy for patients with glomus jugulare tumor. Despite the short follow-up period and the limited number of patients analyzed, we can infer that radiosurgery produce a tumor growth control with low morbidity, and may be used as a good option to surgical resection in selected cases.

Prospective Study of Glomus Jugulare and Tympanicum Tumors: Clinical Findings, Surgical Approach and Outcomes, Short-Term Follow-Up: A Clinical Perspective

Prospective Study of Glomus Jugulare and Tympanicum Tumors: Clinical Findings, Surgical Approach and Outcomes, Short-Term Follow-Up: A Clinical Perspective A glomus tumor is the most common primary tumor of the middle ear, typically presenting with pulsatile tinnitus and unilateral hearing loss. This study provides a clinical perspective of glomus tumors. Methods: A prospective study was performed on 26 patients (10 with glomus tympanicum and 16 with glomus jugulare) from January 2009 to January 2011 in Amir Alam Hospital (tertiary referral center). All patients underwent neurological examination, audiometry, and radiological survey before and after surgery. A questionnaire was completed by patients to evaluate their postoperative quality of life. The Fisch classification was used in this study.

A Facial Canal Paraganglioma with Intradural Extension via the Internal Auditory Canal

Prospective Study of Glomus Jugulare and Tympanicum Tumors: Clinical Findings, Surgical Approach and Outcomes, Short-Term Follow-Up: A Clinical Perspective Facial canal paragangliomas are rare and by definition they arise from the paraganglia along the vertical portion of facial canal. We report a case of a facial canal paragangliomas with extension into the internal auditory canal. This report describes for the first time this avenue of extension of facial canal paragangliomas.

Lower Cranial Nerves Compromise in Glomus Jugulare Tumors

Objective: Describe lower cranial nerve compromise (IX to XII) in preoperative and postoperative time of patients with glomus jugulare tumors trying to correlate the relationship with functional outcome with epidemiological variables (age, sex) or variables related to the tumor (extent, location, and relationship to the facial nerve and internal carotid artery). Method: Retrospective chart review of 53 patients with glomus jugulare submitted to surgical treatment. We searched for lower cranial nerve compromise (LCNC) in preoperative time and at initial PO (until 7 days), PO of 6 months and 1 year. Results of LCNF were compared with tumor aspects and epidemiological features. Results: The LCNC in preoperative time observed was IX (7/13, 21%), X (11/30, 19%), XI (7/13, 21%), and XII (11/20, 75%). All showed decreased function postoperatively as expected but also recovery with 1 year of PO. Vagus nerve (X) was the most affected at 12 months, showing some degree of symptoms in 23 patients (43.39%). There was no epidemiological evidence of relationship of age and gender with the prevalence of cranial nerve compromise. Patients classified as Fisch-Matto C3/C4 had a higher prevalence of CN involvement preoperatively compared to patients C1/C2 ( P < .05). There was statistical significance between the presences of deficits in lower cranial nerves with the degree of tumor extension in the internal carotid artery. Conclusion: Functional impairment of the lower cranial nerves was observed in the pre- and the postoperative time of patients undergoing surgery for resection of the glomus jugulare tumors and that its frequency was directly proportional to the extent of the tumor and its relationship with internal carotid artery.

Facial Nerve Function in Postoperative of Glomus Jugulare

Objective: Describe the facial nerve function (FNF) in preoperative and postoperative time of patients with glomus jugulare tumors trying to correlate the relationship with functional outcome with epidemiological variables (age and sex) or variables related to the tumor (extent, location, and relationship to the facial nerve and internal carotid artery). Method: Retrospective review of 53 patients with glomus jugulare submitted to surgical treatment (1987 to 2010) in Edmundo Vasconcelos Hospital. The FNF was analyzed preoperatively and at initial PO (until 7 days), PO of 6 months, and PO of 1 year. Results of FNF were compared with tumor aspects and epidemiological features. Results: In a preoperative time we observed FNF decreased in 11 patients (20.75%) in which the average of facial function by House Brackmann scale degree was 2.54. Most of those (7%-63.64%) scaled as HB II. Intraoperative findings demonstrated anatomic invasion in 5 patients. In the immediate PO time (1 to 7 days) a decrease of facial nerve function in 83.02% of the patients (avgHB = 3.32) was noted. In PO of 6 and 12 months, some degree of FNF was observed in 32 (60.37%/avgHB 2.09) and 25 (47.17%/avgHB 1.86). There was no observed relation ( P < .05) between kinds of facial nerve manipulation or epidemiological features with FNF with in 1 year postoperatively. Conclusion: FNF is a common feature in PO of patients with jugulare foramen tumors, and its recovery is expected for the majority after initial PO time. For the group of patients with preoperative compromise of VII nerve, poorer outcomes were were observed.

E-032 Imaging characteristics of tumors of the head and neck

PurposeTumors of the head and neck have characteristic findings on MRI and cerebral angiography which help with proper diagnosis and localization. This presentation will provide a review of the characteristic...

Surgical Management of Giant Transdural Glomus Jugulare Tumors with Cerebellar and Brainstem Compression

Objective The objective of this study is to discuss the management of advanced glomus jugulare tumors (GJTs) presenting with intradural disease and concurrent brainstem compression. Study Design This is a retrospective case series. Results Over the last decade, four patients presented to our institution with large (Fisch D2; Glasscock-Jackson 4) primary or recurrent GJTs resulting in brainstem compression of varying severities. All patients underwent surgical resection through a transtemporal, transcervical approach resulting in adequate brainstem decompression; the average operative time was 12.75 hours and the estimated blood loss was 2.7 L. All four patients received postoperative adjuvant radiotherapy in the form of intensity-modulated radiation therapy or stereotactic radiosurgery. Combined modality treatment permitted tumor control in all patients (range of follow-up 5 to 9 years). Conclusion A small subset of GJTs may present with intracranial transdural extension with aggressive brainstem compression mandating surgical intervention. Surgical resection is extremely challenging; the surgical team must be prepared for extensive operating time and the patient for prolonged aggressive rehabilitation. Newly diagnosed and recurrent large GJTs involving the brainstem may be controlled with a combination of aggressive surgical resection and postoperative radiation.

Glomus Jugulare Tumors. A Series of 24 Cases

Glomus Jugulare Tumors. A Series of 24 Cases

Dysphagia: The Common Thread in Two Uncommon Coexistent Conditions, Glomus Jugulare Tumor and Dysphagia Lusoria (P03.146)

Objective: To report a case of glomus jugulare tumor that was initially diagnosed as dysphagia lusoria. Background Glomus jugulare (GJ) tumors are very rare with an estimated incidence around one per 1.3 million people. Patients with GJ tumor usually present with conductive hearing loss and pulsatile tinnitus. Growth of the tumor can cause facial nerve paralysis, vertigo, hoarseness, and paralysis of lower cranial nerves. Active GJ tumors secrete catecholamines into the circulation. Patients with active GJ tumors have vasomotor symptoms like headaches, excess lacrimation and episodic flushing. Dysphagia is an uncommon presentation of GJ tumor (6%). Dysphagia lusoria due to compression of the esophagus by an aberrant subclavian artery is also uncommon with a prevalence of 0.5- 1.8%. Design/Methods: This is a case report from Yale-New Haven Hospital. Results: A 46-year-old female presented with progressive dysphagia with headaches and unilateral cranial nerve palsies demonstrating facial droop, tongue deviation and weakness of neck and shoulder muscles. Initial work up for her dysphagia revealed an aberrant right subclavian artery compressing the esophagus, so her symptoms were thought to be secondary to dysphagia lusoria. However, there was no relief of her dysphagia after the corrective surgery, and her headaches persisted. MRI of the brain was then done and revealed a GJ tumor. Octreotide scan depicted an octreotide-avid mass at the left jugular foramen consistent with a paraganglioma. Catecholamine assessments showed increased levels of epinephrine and normetanephrine in a 24 hour urine study. She underwent radiosurgical treatment with 16 Gy in a single fraction with improvement in her dysphagia. Conclusions: This case illustrates an unusual presentation of a rare catecholamine secreting GJ tumor (active type) causing dysphagia which initially mimicked dysphagia lusoria. This highlights the need to be vigilant in keeping a broad differential diagnosis in patients presenting with dysphagia. Disclosure: Dr. Mittal has nothing to disclose. Dr. Jabbari has nothing to disclose. Dr. Machado has nothing to disclose.

A common symptom in two uncommon coexistent conditions: Glomus jugulare tumor and dysphagia lusoria

Glomus jugulare (GJ) tumors are paragangliomas originating rom chromaffin cells. GJ tumors are very rare with an estimated ncidence around one per 1.3 million people [1]. These neoplasms re highly vascularized and histologically benign in most cases but ay invade bone, blood vessels, dura mater, and cranial nerves. atients with GJ tumor usually present with conductive hearing oss and pulsatile tinnitus. Growth of the tumor can cause facial erve (FN) paralysis, vertigo, hoarseness, and paralysis of lower ranial nerves [2]. Active GJ tumors secrete catecholamine into the irculation presenting with vasomotor symptoms like headaches, xcess lacrimation, diarrhea and episodic flushing [3]. Dysphagia is n uncommon presentation of glomus jugulare tumor (6%) [1]. Dyshagia lusoria due to compression of the esophagus by an aberrant ubclavian artery is also uncommon with a prevalence of 0.5–1.8% 4]. We present a rare catecholamine secreting GJ tumor which coxisted with an aberrant subclavian artery. Although dysphagia was nitially attributed to the aberrant subclavian artery, later work up ndicated a GJ tumor as the main culprit. To our knowledge, coxistence of GJ tumor and aberrant subclavian artery has not been eported before in the literature. Moreover active catecholamine

Cochlear Radiation Dose Does Not Predict Hearing Loss after SRS for Glomus Jugulare Tumors

Cochlear Radiation Dose Does Not Predict Hearing Loss after SRS for Glomus Jugulare Tumors

Glomus jugulare (Type A): A case review

We report a glomus jugulare tumor in a 60 yrs old female; non diabetic, non hypertensive who presented with continuous pulsatile hissing tinnitus in right ear with progressive hearing impairment and fullness in the ear. Detailed history and examination with aid of investigations it turned out to be glomus jugulare, treated by surgical excision by post auricular hypotympanotomy approach.

Preoperative Embolization of the Inferior Petrosal Sinus in Surgery for Glomus Jugulare Tumors

To compare the outcomes of surgery for glomus tumors involving the jugular foramen with and without preoperative venous embolization of the inferior petrosal sinus (IPS). Retrospective chart review. Tertiary referral center. Twenty-four patients with paragangliomas involving the jugular foramen treated between 1995 and 2008. All patients underwent surgical resection after receiving preoperative angioembolization with or without venous embolization of the IPS. Total operative duration, estimated blood loss, intensive care unit and total hospital days, and novel postoperative lower cranial nerve deficits were recorded, and appropriate statistical analysis was conducted. Twenty-four patients met inclusion criteria. Fourteen of these patients underwent preoperative embolization of the IPS in addition to angioembolization. The group that did not undergo embolization of the IPS was used as the control group (n = 10). These groups were compared with regard to the above outcome measures. Blood loss and new lower cranial nerve deficits were reduced in the venous embolization group, although neither measure reached statistical significance. Tumor size correlated with increased intraoperative hypotensive events and longer total hospital stay, and these correlations were statistically significant. Preoperative embolization of the IPS is possible in many patients undergoing surgery of the jugular foramen. The addition of venous embolization to the traditional arterial embolization of glomus jugulare tumors adds little additional time or expense to the procedure and facilitates control of bleeding once the jugular bulb has been opened.

Imaging carotid body chemodectomas with68Ga-DOTA-NOC PET-CT

The purpose of the present study was to evaluate the role of 68-gallium-1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acid-1-Nal3-octreotide ((68)Ga-DOTA-NOC) positron emission tomography CT (PET-CT) in carotid body chemodectomas (CBCs). 5 patients with known CBCs (conventional imaging and angiography) were evaluated retrospectively (3 males, 2 females; mean age 36.6 years). All underwent PET-CT after injection of 148 MBq (4 mCi) of (68)Ga-DOTA-NOC. At baseline there were four bilateral CBCs and one unilateral CBC with an additional glomus tympanicum and bilateral glomus jugulare tumour in these five patients. All these lesions showed intense uptake on (68)Ga-DOTA-NOC PET-CT. Apart from these known lesions, additional tumours were detected in three patients; glomus jugulare in one, aortic body tumour and multiple abdominal paragangliomas in another and unsuspected vertebral metastasis in the last. Overall it led to a change in management in 3 of the 5 patients (60%). (68)Ga-DOTA-NOC PET-CT is a promising non-invasive imaging modality in the detection and characterisation of CBCs.

Molecular Analysis of Pheochromocytoma after Maternal Transmission of SDHD Mutation Elucidates Mechanism of Parent-of-Origin Effect

Pheochromocytoma/paraganglioma occurs almost exclusively after paternal transmission of succinate dehydrogenase D (SDHD) mutations. This parent-of-origin effect has not been fully explained but is accompanied by obligate loss of the maternal copy of chromosome 11. Loss of wild-type SDHD and an additional imprinted gene (hypothesized to be H19) appears necessary for tumor formation. Two previous reports suggested tumor formation after maternal transmission of SDHD mutation, but histological and molecular characterization was unavailable. We report the first kindred in which histologically confirmed pheochromocytoma/paraganglioma occurred after maternal transmission of an SDHD mutation and investigate the molecular mechanism of tumor formation. The design of the investigation was the study of a three-generation family with SDHD c.242C>T (p.Pro81Leu) mutation. The index patient had a histologically confirmed pheochromocytoma and an identical SDHD germline mutation (p.Pro81Leu) to her mother (who had a glomus jugulare tumor) and paraganglioma tissue from her maternal grandfather. Tumor DNA from the index patient revealed loss of heterozygosity (LOH) at 11q23, causing loss of the wild-type paternal SDHD allele and LOH affecting maternal 11p15, including H19. These two regions of LOH were separated by a region exhibiting clearly retained heterozygosity, including SDHAF2, a recently reported paraganglioma susceptibility gene. Tumor formation can occur after maternal transmission of SDHD, a finding with important clinical implications for SDHD families. Tumor formation in SDHD mutation requires the loss of both the wild-type SDHD allele and maternal 11p15, leading to the predominant but now not exclusive pattern of disease inheritance after paternal SDHD transmission.