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Glomus Jugulare Tumor

Glomus jugulare tumor is a rare, benign paraganglioma of the skull base that forms in the jugular fossa of the temporal bone and is made up of glomus cells which are neural crest cell derivatives.


Presentation

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]
Tachypnea
  • Ensuing sympathetic hyperstimulation can induce tachycardia, tachypnea, diaphoresis, and elevated blood pressure; similar to the presentation of adrenal gland tumors.[symptoma.com]
Sore Throat
  • 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]
Explosive Diarrhea
  • 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]
Regurgitation
  • Improvement was mainly in dysphonia, regurgitation and also shoulder pain. Seven cases showed stable clinical disease with no additional symptoms or signs. One patient developed transient partial 7th nerve palsy at 9 months post gamma knife surgery.[wjso.biomedcentral.com]
Glossalgia
  • Glossalgias Painful sensations in the tongue, including a sensation of burning. Glossectomy Partial or total surgical excision of the tongue.[healthdictionary.info]
Lacrimation
  • Patients with active GJ tumors have vasomotor symptoms like headaches, excess lacrimation and episodic flushing. Dysphagia is an uncommon presentation of GJ tumor (6%).[neurology.org]
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]
Skin Lesion
  • Pain is the main symptom of the skin lesions, which are therefore known as painful subcutaneous tumors. In glomus tumors of the central group pain is not an outstanding clinical feature.[pubs.rsna.org]
Tinnitus
  • The patient exhibited all the symptoms of primary malignant tumors of the ear, including facial paralysis, otorrhea, pain, hearing loss, tinnitus, dizziness, and vertigo.[ncbi.nlm.nih.gov]
  • 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]
  • 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]
  • Abstract A glomus jugulare tumor secreted a large quantity of noradrenaline (NA) and produced symptoms of tinnitus, palpitation, sweating, and labile hypertension. The NA content of the tumor was 0.75 mg/gm of tissue.[ncbi.nlm.nih.gov]
  • 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[symptoma.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]
  • 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.[neurology.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]
  • Pulsatile tinnitus, the most common symptom of glomus tumors, occurs due to the vascular nature of these tumors.[neurotucson.com]
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) Signs and tests Glomus jugulare tumors are[coordinatedhealth.com]
  • Difficulty in swallowing (dysphagia), dizziness, hearing problems or loss, hearing pulsations in the ear, hoarseness, Weakness or loss of movement in the face (facial nerve palsy) are the symptoms associated with this problem.[alwaysayurveda.net]
  • 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]
Otalgia
  • 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]
  • 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]
Progressive Hearing Loss
  • We present the case of a 31-year-old woman, operated by the senior author, with a 1-year history of tinnitus, vertigo, and progressive hearing loss, that evolved with facial nerve palsy (House-Brackmann IV) 2 months before surgery.[academic.oup.com]
Facial Numbness
  • Six patients (15%) experienced new deficits (hearing loss alone in three, facial numbness and hearing loss in one, vocal cord paralysis and hearing loss in one, and temporary imbalance and/or vertigo in one).[wjso.biomedcentral.com]
Neck Mass
  • Enhance your understanding with multiple-choice questions accompanying each case, emphasizing cranial nerves, skull base lesions, sinonasal, orbital, salivary gland, aerodigestive system mucosal lesions and deep space neck masses.[books.google.de]
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]

Workup

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].

Treatment

  • We recommend surgical excision as primary treatment for glomus tympanicum tumors and high voltage radiotherapy as primary treatment for glomus jugulare tumors.[ncbi.nlm.nih.gov]
  • 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]
  • Abstract Over the past few years, considerable advances have been made in the surgical treatment of glomus jugulare tumors. The long-term results of such surgery for cure of these tumors has yet to be established, however.[ncbi.nlm.nih.gov]
  • The mean follow-up period was 56 months (range 36-108 months); there were 3 males, 19 females; mean age was 43.6 years; 15 patients had GKS as the primary treatment; 2 patients had surgical residuals; 2 had previous radiation therapy; and 3 previously[ncbi.nlm.nih.gov]
  • Care must be taken to avoid inducing a hypertensive crisis during and following treatment, and longer follow-up will help determine whether and when pharmacologic blockade can be discontinued.[ncbi.nlm.nih.gov]

Prognosis

  • 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]
  • 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]
  • 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]

Epidemiology

  • 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]
Sex distribution
Age distribution

Pathophysiology

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

Prevention

  • It prevent the abnormal growth of the cancer cells and enhances the growth of health cells. Moreover blood detoxification properties of this herb, help to eliminate the toxins from the body.[alwaysayurveda.net]
  • 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.[coordinatedhealth.com]
  • 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]

References

Article

  1. Young WF Jr. Paragangliomas: clinical overview. Ann N Y Acad Sci. 2006;1073:21-29.
  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

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Last updated: 2019-06-28 10:53