|Year : 2012 | Volume
| Issue : 4 | Page : 214-216
Glomus jugulare (Type A): A case review
Vinod T Kandakure, Girish V Thakur, Ayesha Kausar, Amit Thote
Department of Otorhinolaryngology, Government Medical College, Latur, Maharashtra, India
|Date of Web Publication||19-Dec-2012|
Vinod T Kandakure
Opp. Mamata Hospital, Mitra Nagar, Latur, Maharashtra
Source of Support: None, Conflict of Interest: None
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.
Keywords: Benign paraganglioma, Glomus jugulare, Hypotympanotomy
|How to cite this article:|
Kandakure VT, Thakur GV, Kausar A, Thote A. Glomus jugulare (Type A): A case review. Indian J Otol 2012;18:214-6
| Introduction|| |
Glomus jugulare tumors are rare, slow growing, hypervascular tumors that arise within jugular foramen of the temporal bone and may extend to involve the middle ear. These originate from the chief cells of the paraganglia or glomus bodies, located within the wall of adventitia of the jugular bulb and may involve either the auricular branch of vagus nerve (Arnold nerve) or the tympanic branch of the glossopharyngeal nerve (Jacobson nerve). ,,
Estimated incidence is 1 per 1.3 million populations of which 1-5% is malignant. The benign tumors are locally aggressive. Growth rate is about 0.8 mm per year. Glomus secreting catecholamines occur symptomatically in about 1-3% of cases. ,,,
| Case Report|| |
A 60 year old lady, reported with chief complaints of continuous pulsatile hissing tinnitus in right ear and progressively increasing impairment of hearing from last two years, with heaviness in right mastoid region. There was no history of ear discharge, giddiness/vertigo or facial weakness. There was no history of difficulty in swallowing and hoarseness of voice, palpitation or inability to lift shoulder.
On Otoscopy pulsations were seen in the posterior half of tympanic membrane with bulging and congestion. Handle of malleus was ill defined because of bulging and congested tympanic membrane [Figure 1]. Tuning fork tests showed Rinne's negative on right side and Weber's lateralized to right ear. On pure tone audiometry, there was right moderate conductive hearing loss.
HRCT temporal bone showed dehiscent jugular bulb in right middle ear with soft tissue mass in middle ear without intracranial extension [Figure 2]. All routine hematological investigations were normal. 24 hr urine VMA level was in normal range (3-4 mg/day). On this basis non-secretory glomus jugulare was diagnosed. As per the classification devised by Oldring and Fish, the tumor was type A (tumor limited to middle ear cleft).
Patient was treated successfully by surgical excision of tumor. Postaural Wilde's incision for better exposure was taken. The skin and periosteum of the anteroinferior and posterior osseous meatal wall elevated and pushed upward for exposing the tympanic bone from the tympanomastoid suture behind to the tympanosquamous suture in front. Bony meatus enlarged by thinning down the anteroinferior to posterior meatal wall with cutting burr and curette. The annulus was elevated from its sulcus posteriorly and anteroinferiorly confined to the lower half of tympanic cavity. Tympanic membrane folded upward upon itself and the tumor came into view. Resection of the bony sulcus inferiorly and posteriorly was done to expose the hypotympanum and tumor in it. The extent of the tumor in all directions delineated by curetting the bone. The tumor was rapidly dissected away from the promontory and floor of hypotympanum with side knife and cotton balls. Base of the tumor was cauterized and tumor was excised with crocodile forcep. Bleeding was controlled with gelfoam pack. Dehiscent jugular bulb was sealed with bone wax [Figure 3]. Tympanic membrane and tympanomeatal flap reposited back and postaural wound was sutured. Tumor size was 1 cm.
The Postoperative histopathology report was an encapsulated mass consisting of convolutes of capillary sized vessels; surrounded by zallballen of glomus cells, diagnosed to be glomus tumor.
| Discussion|| |
Glomus jugulare tumor, once thought to be one of the most difficult surgically unapproachable ones, is now becoming safely manageable with reasonable morbidity and mortality rates. This recent achievement has been accomplished by the extraordinary efforts put forth in the understanding of the microsurgical techniques and instrumentations and by the most exciting and promising innovations in cranial base surgery Because of the insidious onset of symptoms, these tumors often go unnoticed, and delay in diagnosis is frequent. Due to its location and extent of involvement, glomus jugulare tumors present difficulty in early diagnosis and management as well as social challenge. 
The candidate gene mutation for familial and some sporadic paragangliomas is SDHD (succinate dehyrogenase enzyme subunit D) has been mapped to PGL1 locus in 11 q 23, 3. 
Glomus tumors are encapsulated, highly vascular and locally invasive tumors. Inside the temporal bone they tend to expand along the pathway of least resistance such as air cells, vascular lumen, skull base foramina, and Eustachian tube. They also invade and erode bone in lobular fashion. Middle ear ossicles are spared. Initially skull base erosions occur in the region of jugular fossa and posteroinferior part of petrous bone. Later on extension occurs to the mastoid and adjacent occipital bone. Cochleovestibular destruction is caused by ischemic necrosis. 
Glomus tumors occur with an estimated annual incidence of 1 case per 1.3 million people. Although rare, glomus tumors are the most common tumor of the middle ear and are second to vestibular schwannoma as the most common benign tumor of the temporal bone. The female-to-male ratio is 3-6:1. Most tumors occur in patients aged 40-70 years, but cases have been reported in patients as young as 6 months and as old as 88 years. Multicentric tumors are found in 3-10% of sporadic cases and in 25-50% of familial cases. ,,
The most common symptoms are pulsatile hissing tinnitus, conductive hearing loss. Other aural signs and symptoms are ear fullness, otorrhea, hemorrhage, bruit and the presence of middle ear mass. Significant ear pain is uncommon. Involvement of inner ear produces vertigo and sensorineural hearing loss. Cranial nerve involvement produces hoarseness and dysphagia. The presence of jugular foramen syndrome (paresis of cranial nerves ix to xi) is pathognomic for this tumor, but it usually follows 1 yr after the initial symptoms of hearing loss and pulsatile tinnitus. Less commonly glomus tumor produces facial nerve palsy, hypoglossal nerve palsy or Horner's syndrome. Headache, hydrocephalus and elevated intra cranial pressure may be produced by intra cranial extension of tumor.
Otoscopic examination reveals a characteristic pulsatile, reddish-blue tumor behind the tympanic membrane. Audiologic examination reveals mixed conductive and sensorineural hearing loss. The sensorineural component tends to be more significant in tumors with labyrinthine extension. Plain skull radiography may show enlargement of the lateral jugular foramen and fossa. Axial and coronal computed tomography (CT) scanning with thin sections are superior at demonstrating the extent of bone destruction. Magnetic resonance imaging (MRI) with gadolinium-diethylenetriamine pentaacetic acid (DTPA) contrast is best for delineating tumor limits. Glomus tumors on T1- and T2-weighted MRI have characteristic soft tissue mixed intensity with intermixed high-intensity signals and signal voids (i.e. salt and pepper appearance) representing fast flowing blood. A combination of CT scanning and contrast MRI is the imaging regimen of choice for glomus jugulare tumors. ,,,,,,
Alpha-blockers and beta-blockers are useful for tumors secreting catecholamines. They are usually administered for 2-3 weeks before embolization and/or surgery to avoid potentially lethal blood pressure lability and arrhythmias. Successful treatment of pulmonary metastases with etoposide (VP-16) and cisplatin has been described.
Preoperative superselected embolisation done to reduce bleeding and complete resection with ease.
Surgical therapy is definitive treatment for glomus tumors.
For smaller tumors i.e. fish type a, post auricular hypotympanotomy or transcanal tympanotomy done for complete resection. The mass is avulsed from the promontory and the bleeding is controlled by microbipolar cautery or light packing. Bone wax sealing is done for the bone defect in promontory.
The tumor involving mastoid air cells system transmastoid resection is performed, which comprises of complete mastoidectomy with extended facial recess exposure.
Larger glomus jugular tumors removed by means of lateral skull base surgery and represent a multi disciplinary team effort.  A major advancement in the surgical treatment of glomus jugulare tumors occurred with the development of preoperative superselective embolization.
Because of slow growth rate of tumor, 10-15 yrs follow up period is required post surgery. Advances in diagnostic imaging like CT and MRI has certainly decreased the incidence of residual tumors in surgical therapy. 
The treatment of glomus jugulare tumors by radiation therapy is palliative, which is recommended for the elderly, medically infirm, or those selected multicentric lesions in which definitive treatment is otherwise contraindicated. 
| Conclusion|| |
High index of suspicion, with early diagnosis and appropriate treatment i.e. complete excision of glomus jugulare, can reduce the morbidity and mortality as has been seen in our case.
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[Figure 1], [Figure 2], [Figure 3]