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].

• Pathologist

  The book was designed to be a comprehensive textbook crossing all disciplines and representing a wide spectrum of specialists throughout the country, including surgical, radiation, and medical oncologists, as well as dentists, pathologists, radiologists [books.google.com]

• Aspiration

  Three patients with preoperative Xth nerve dysfunction showed an adequate compensation of the opposite vocal fold in the postoperative period without dysphonia or aspiration. [ncbi.nlm.nih.gov]

  Ambulate with assistance (QD) (unless at bed rest for lumbar drainage) Postoperative Day 2 Remove abdominal Penrose drain Change mastoid dressing Advance diet if no NG Begin tube feeds if NG present Postoperative Day 3 Swallow study if NG or aspiration [medicine.uiowa.edu]

  […] submitted to surgical treatment, the appearance of deficit in one or more cranial nerves occurred in 22-59% of the cases after surgery.[ 11 ] The same review showed the following incidence of other postoperative complications: Liquoric fistula (8.3%), aspiration [surgicalneurologyint.com]

• Thrombosis

  Involvement of the dural sinuses mimics sinus thrombosis. A small number of patients present primarily with a phaeochromocytoma-like picture - eg, perspiration, pallor, nausea, hypertension and tachycardia. [patient.info]

  […] negative 3 bony metastases hypervascular tumors may be very similar in appearances (e.g. renal cell carcinoma, thyroid cancer) meningioma normal anatomical variation asymmetry of jugular foraminal size high riding or dehiscent jugular bulb jugular bulb thrombosis [radiopaedia.org]

  Dural sinuses may be involved may mimic sinus thrombosis Clinical features: Otoscopic examination reveals a characteristic, pulsatile, reddish-blue tumor behind the tympanic membrane that often is the beginning of more extensive findings (ie, the tip [drtbalu.co.in]

  Consent for Surgery Risks inherent to any surgical procedure Bleeding, infection, scar formation, skin anesthesia, or dysesthesia Subtotal tumor removal Risks of anesthesia Death, pneumonia, pulmonary embolism, deep vein thrombosis Risks of jugular foramen [medicine.uiowa.edu]

  Involvement of the dural sinuses may mimic sinus thrombosis [ 11, 12 ]. [wjso.biomedcentral.com]

• Flushing

  A patient with a long history of explosive diarrhea, facial flushing, and violent headaches, the symptom of carcinoid syndrome, was cured after removal of his large glomus jugulare tumor. [ncbi.nlm.nih.gov]

  Patients with active GJ tumors have vasomotor symptoms like headaches, excess lacrimation and episodic flushing. Dysphagia is an uncommon presentation of GJ tumor (6%). [n.neurology.org]

  […] include: weakness in facial muscles facial paralysis difficulty swallowing hoarseness drooping shoulders tongue weakness In a small percentage of glomus jugulare tumors, the mass may produce hormones and cause additional symptoms: headaches tremors flushing [healthline.com]

  Examination Progression of deficits Cranial nerve dysfunction and compensation Brainstem and long tract signs Decreased color vision, visual acuity, or papilledema caused by hydrocephalus Signs and symptoms of a catecholamine secreting tumor Palpitations Flushing [medicine.uiowa.edu]

• Neck Pain

  Shoulder pain, neck pain, and trigeminal symptoms also detected. [wjso.biomedcentral.com]

• Esotropia

  There was a right relative afferent pupillary defect, a right esotropia, and limited abduction of the right eye consistent with a right sixth nerve palsy. Fundus examination revealed bilateral optic disc edema (Fig. 1). [journals.lww.com]

• 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]

• 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]

  Pulsatile tinnitus, conductive hearing loss and aural fullness are the most common presenting symptoms. Moreover, it may cause significant lower cranial nerve deficits due to mass effect. [tjn.org.tr]

  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]

  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. [n.neurology.org]

  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]

• Hearing Problem

  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 [indiatoday.in]

  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 [sites.magellanhealth.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) Signs and tests Glomus jugulare tumors are [coordinatedhealth.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]

• Seizure

  […] grafting using either the greater auricular nerve or sural nerve Swallowing dysfunction with potential for aspiration Hearing loss Vestibular dysfunction CSF leak with the potential for meningitis Need for lumbar drain Need for revision surgery Stoke Seizure [medicine.uiowa.edu]

  […] forms; the infantile form presents at 4-5 months of age with anemia, loss of cognitive gains, neck retraction, dysphagia, and hepatosplenomegaly; the juvenile form features a slowly progressive loss of intellect, hepatosplenomegaly, ataxia, myoclonic seizures [medindex.am]

  Seizures from temporal lobe penetration by a paraganglioma have been reported, and these tumors have been cited as the cause of cerebral ischemic events, congestive heart failure, and subarachnoid hemorrhage. [neurosurgery.tv]

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].

Stereotactic radiosurgery for the treatment of Glomus Jugulare Tumors. [surgicalneurologyint.com]

A 47-year-old woman with left ear pain and hearing loss was diagnosed with a glomus jugulare tumor for which she received radiation therapy as the primary treatment. [ncbi.nlm.nih.gov]

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]

Complex tumors of the glomus jugulare: criteria, treatment, and outcome. J Neurosurg 2002 ; 97: 1356 – 1366. Google Scholar | Medline 18. Ramina, R, Maniglia, JJ, Fernandes, YB. Jugular foramen tumors: diagnosis and treatment. [journals.sagepub.com]

However, treatment may be needed to relieve symptoms. The main treatment is surgery. Surgery is complex and is usually done by both a neurosurgeon and a head and neck surgeon (neurotologist). [sites.magellanhealth.com]

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

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

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

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]

Conductive hearing loss can also occur as the tumor grows into the middle ear space and prevents transmission of sound through the eardrum and ossicular (hearing bone) chain. [neurotucson.com]

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. [indiatoday.in]

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2. Woods CI, Strasnick B, Jackson CG. Surgery for glomus tumors: the Otology Group experience. Laryngoscope. 1993;103(11 Pt 2 Suppl 60):65–70.
3. Kuhweide R, Lanser MJ, Fisch U. Catecholamine secreting paragangliomas at the skull base. Skull Base Surg. 1996;6(1):35–45.
4. Offergeld C, Brase C, Yaremchuk S, et al. Head and neck paragangliomas: clinical and molecular genetic classification. Clinics (Sao Paulo). 2012;67(Suppl 1):19-28.
5. Leonetti JP, Anderson DE, Marzo SJ, et al. Facial paralysis associated with glomus jugulare tumors. Otol Neurotol. 2007;28(1):104-106.
6. Patel SJ, Sekhar LN, Cass SP, Hirsch BE. Combined approaches for resection of extensive glomus jugulare tumors. A review of 12 cases. J Neurosurg. 1994;80(6):1026–1038.
7. Al-Mefty O, Teixeira A. Complex tumors of the glomus jugulare: criteria, treatment, and outcome. J Neurosurg. 2002;97(6):1356–1366.
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