Glomus jugulare tumor (GJT) is uncommon and has been shown to develop primarily between the fourth and seventh decades of life [1] [2]. It has a higher prevalence in females than males and is sometimes inherited familially [1].

Slow growing and non-malignant nature of GJT results in a negative outcome, as the presentation is delayed with gradual onset of symptoms [3]. Symptoms are due to the direct encroachment of the tumor into neighboring structures, and both the size and location of GJT is important for classification purposes [4]. As these tumors arise from the temporal bone, the middle ear is impinged on, causing tinnitus (pulsatile), sensorineural and conductive hearing loss, vertigo, ear discharge, pain, and a reddish-bluish discoloration of the eardrum by an inconspicuous mass on otoscopy. Due to their extensive vasculature, a bruit may be heard upon auscultation of the temporal bone.

The jugular foramen is the aperture through which the internal jugular vein, and cranial nerves IX, X, and XI exit the skull. Compression of the cranial nerves results in dysphagia, hoarseness, change in voice, weakness in the sternocleidomastoid and trapezius muscles leading to the jugular foramen syndrome [2] [5].

The spread of a glomus jugulare tumor into the cranial vault consequently manifests as headaches, accumulation of cerebrospinal fluid and other features of raised intracranial pressure [6]. The brain stem may be affected, giving rise to signs such as loss of coordination, ataxia, loss of balance, and involvement of multiple cranial nerves [6]. Unilateral facial weakness is unusual but has been reported [5].

Most GJTs do not release any hormones. In some instances, however, they secrete adrenalin, noradrenalin, and dopamine, and have been referred to as complex GJTs [7]. Ensuing sympathetic hyperstimulation can induce tachycardia, tachypnea, diaphoresis, and elevated blood pressure; similar to the presentation of adrenal gland tumors. Malignant change is uncharacteristic for these tumors, noted in only a minority of cases [8].

• Respiratory Distress

  Postoperatively, 2 patients with acute respiratory distress syndrome required a temporary tracheotomy. Three patients developed partial ischemia of the wound, pinna, or both. [ncbi.nlm.nih.gov]

• Tinnitus

  Glomus jugulare tumor should be considered in the differential diagnosis of adult chronic otitis, deafness, tinnitus, and dysfunction of the lower cranial nerves. [ncbi.nlm.nih.gov]

  […] predominance of norepinephrine suggests that phenylethanolamine-N-methyltransferase (PNMT), the enzyme that converts norepinephrine to epinephrine, was absent.4 We describe a 37-year-old man with severe hypertension (blood pressure, 180/150 mm Hg), right-sided tinnitus [nejm.org]

  Forty had pulsatile tinnitus at the time of GKS. The median dose to the tumor margin was 15 Gy. The median of follow-up period was 50.5 months. [wjso.biomedcentral.com]

• Pulsatile Tinnitus

  A 33-year-old woman presented a chronic headache and sore throat on the right side of her body, continuous pulsatile tinnitus with decreasing hearing in the right ear, and recurrent bleeding from the right ear. [ncbi.nlm.nih.gov]

  May be incidental but symptomatic masses produce pulsatile tinnitus, otalgia, or conductive hearing loss 1,3. Glomus tympanicum paragangliomas arise from the Jacobson nerve at the cochlear promontory. [radiopaedia.org]

  Symptoms of these tumors usually include pulsatile tinnitus, hearing loss, facial nerve paralysis, vertigo, otalgia, dysphagia, hoarseness, throat sore, episodic hypertension with headaches, and tachycardia (3). [journals.lww.com]

  It is pathognomonic for this tumour but it usually follows a year after the initial symptoms of hearing loss and pulsatile tinnitus. [patient.info]

  In the middle ear, most glomus tympanicum tumors present with hearing loss and pulsatile tinnitus and are found during a physical exam, appearing as a reddish-blue mass behind the eardrum. [upmc.com]

• Hearing Problem

  Symptoms may include: Difficulty swallowing (dysphagia) Dizziness Hearing problems or loss Hearing pulsations in the ear Hoarseness Pain Weakness or loss of movement in the face (facial nerve palsy) Glomus jugulare tumors are rarely cancerous and do not [medlineplus.gov]

  Symptoms Symptoms may include: Difficulty swallowing (dysphagia) Dizziness Hearing problems or loss Hearing pulsations in the ear Hoarseness Pain Weakness or loss of movement in the face (facial nerve palsy) Exams and Tests Glomus jugulare tumors are [ufhealth.org]

  Symptoms Symptoms may include: Difficulty swallowing (dysphagia) Dizziness Hearing problems or loss Hearing pulsations in the ear Hoarseness Pain Weakness or loss of movement in the face (facial nerve palsy) Signs and tests Glomus jugulare tumors are [coordinatedhealth.com]

  Symptoms Symptoms may include: Difficulty swallowing (dysphagia) Dizziness Hearing problems or loss Hearing pulsations in the ear Hoarseness Pain Weakness or loss of movement in the face (facial nerve palsy) Cases [dallasear.com]

• Otalgia

  May be incidental but symptomatic masses produce pulsatile tinnitus, otalgia, or conductive hearing loss 1,3. Glomus tympanicum paragangliomas arise from the Jacobson nerve at the cochlear promontory. [radiopaedia.org]

  Symptoms of these tumors usually include pulsatile tinnitus, hearing loss, facial nerve paralysis, vertigo, otalgia, dysphagia, hoarseness, throat sore, episodic hypertension with headaches, and tachycardia (3). [journals.lww.com]

  Patients (%) Pulsatile tinnitus 36 (86) Hearing loss 29 (69) Otalgia 8 (19) Otorrhea 3 (7) Vertigo 9 (21) Twenty–nine patients (70 %) had no preoperative cranial nerve deficit. [ncbi.nlm.nih.gov]

  Other complications such as otalgia, vertigo, cofosis and facial palsy occurred only once. One patient eventually presented a basal cell carcinoma in the face and neck approximately 30 years after radiotherapy on the same irradiated side. [scielo.br]

• Unilateral Facial Weakness

  Unilateral facial weakness is unusual but has been reported. Most GJTs do not release any hormones. In some instances, however, they secrete adrenalin, noradrenalin, and dopamine, and have been referred to as complex GJTs. [symptoma.com]

Imaging is the mainstay of diagnosis and monitoring in this type of tumor. Computerized tomography (CT) and magnetic resonance imaging (MRI) scans are most frequently used. CT scans and MRI scans help to visualize tumor extension into the bone, as well as spread to adjacent intracranial structures respectively [6]. X-rays of the skull can also be done, and will show signs of abnormal growth around the jugular fossa, but are not as helpful as CT and MRI scans. The aforementioned rich blood supply of glomus jugulare tumors necessitates arteriography and is especially important if tumor resection is planned. Radionuclide scan using meta-iodobenzyl-guanidine (MIBG) has also been used with success, in assessing these masses [9]. If there are indications of sympathetic predominance, urine catecholamine levels can be measured to determine whether tumors are secretory or not [10].

At UPMC, the preferred treatment is a transmastoid surgical approach to remove the tumor. In addition, Gamma Knife® radiosurgery may be used as the first treatment option or as an additional treatment after surgery. [upmc.com]

One patient developed a new permanent facial paresis grade II according to House & Brackmann six months after treatment (D2-tumor, tumor surface dose 20 Gy, treatment in 1991). [journals.plos.org]

Treatment may be essential for advanced tumors which may be bleeding through the ear. [ncbi.nlm.nih.gov]

Common surgical treatments for glomus jugulare tumors are craniotomy and endoscopic endonasal surgery, and nonsurgical treatment is stereotactic radiosurgery. [neurosurgery.columbia.edu]

Treatment and prognosis Surgical resection is the treatment of choice 4. [radiopaedia.org]

Outlook (Prognosis) People who have surgery or radiation tend to do well. More than 90% of those with glomus jugulare tumors are cured. [ufhealth.org]

Expectations (prognosis) Patients who have surgery or radiation tend to do well. More than 90% of those with glomus jugulare tumors are cured. [coordinatedhealth.com]

However, males are more commonly affected in the familial type. [1], [2] The peak incidence of paragangliomas is in the 4 th or 5 th decade of life; however, subjects with familial etiology present at a younger age. [3], [4] It has now been recognized [neurologyindia.com]

[…] carotid canal Type C3 tumor - Tumor invasion of the horizontal portion of the carotid canal Type D1 tumor - Tumor with an intracranial extension less than 2 cm in diameter Type D2 tumor - Tumor with an intracranial extension greater than 2 cm in diameter Etiology [emedicine.medscape.com]

Epidemiology[1] The annual incidence is about 1 in 1.3 million people per year. However, it is the most common tumour of the middle ear and the second most common tumour of the temporal bone. [patient.info]

--------- the second most common temporal bone tumors The first is the acoustic neurinomas. 8 Epidemiology The incidence of glomus jugulare tumors is 11,300,000 population. the predominant incidence in females. [powershow.com]

Surgical excision is the preferred treatment for benign glomus tumors.[11] Epidemiology[edit] The exact rate of glomus tumors is unknown. The multiple variant is rare, accounting for less than 10% of all cases. [en.wikipedia.org]

Epidemiology Glomus tumors occur with an estimated annual incidence of 1 case per 1.3 million people. [25] Although rare, glomus tumors are the most common tumor of the middle ear and are second to vestibular schwannoma as the most common tumor of the [emedicine.medscape.com]

PATHOPHYSIOLOGY  Benign, Encapsulated, Slow growing, Highly vascular, Locally invasive tumour that erodes bone.  Expand within temporal bone via pathways of least resistance – air cells, vascular lumens, skull Base foramina & the eustachian tube.  [de.slideshare.net]

Pathophysiology: Glomus tumors are encapsulated, highly vascular, and locally invasive tumors. [drtbalu.co.in]

Pathophysiology The glomera jugulare, or glomus bodies, are small collections of paraganglionic tissue. [emedicine.medscape.com]

Tumors may present a decrease in size, but rarely disappear as observed in pathophysiological analysis of follow-up and radiological tests. [scielo.br]

In some cases, a procedure called embolization is performed before surgery to prevent the tumor from bleeding too much during surgery. After surgery, radiation therapy may be used to treat any part of the tumor that could not be removed completely. [medlineplus.gov]

Gamma Knife radiosurgery is used to prevent further growth of the glomus tumor and maintain current neurological and hearing function. [upmc.com]

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8. Sanna M, Jain Y, De Donato G, et al. Management of jugular paragangliomas: the Gruppo Otologico experience. Otol Neurotol. 2004;25(5):797–804
9. Hoefnagel CA, Voute PA, de Kraker J, Marcuse HR. Radionuclide diagnosis and therapy of neural crest tumours using iodine-131 metaiodobenzylguanidine. J Nucl Med. 1987;28(3):308–314.
10. Schwaber MK, Glasscock ME, Nissen AJ, et al. Diagnosis and management of catecholamine secreting glomus tumors. Laryngoscope. 1984;94(8):1008–1015