GlomusJugulare

GLOMUS JUGULARE
  • Glomus jugulare tumors are rare, slow-growing, hypervascular tumors that arise within the jugular foramen of the temporal bone.
  • They are included in a group of tumors also referred to as chemodectomas or nonchromaffin paragangliomas.
  • Paragangloima occur at various sites and include carotid body, glomus vagale, and glomus tympanicum tumors
  • Paraganglia develop from the neural crest .They arise from Glomus cells(which is a kind of chemoreceptor).
  • Most common benign neoplasm of middle ear.
  • Glomus tumor is a benign tumor, therefore lymph node metastats is not present.
  • Hypotympanum is the usual location of Glomus Jugulare Tumor.
  • It may labyrinth, petrous pyramid and mastoid
  • Rule of 10: 10% familial; 10% multicentric; 10% functional (secrete catecholamines)
  • Glomus jugulare tumors occur predominantly in women in the fifth and sixth decades of life.
  • Benign encapsulated, extremely vascular, very slow growing, locally invasive
  • Abundant thin walled blood sinusoids with no contractile muscle coat – profuse bleeding
CLINICAL FEATURES OF GLOMUS JUGULARE
  • The earliest symptoms of glomus tumour is pulsatile tinnitus (earliest)- swishing character, temporarily stopped by carotid pressure and hearing loss.These are followed by blood 
  • stained otorrhoea and earache.
  • Hearing loss is conductive and slowly progressive.
  • Before the tympanic membrane (eardrum) is perforated a red swelling is seen to arise from the floor of middle ear, i.e. "Rising sun" appearance when the tumor arises from the floor of middle ear.Pulsation sign (Brown sign) is positive, i.e. when ear canal pressure is raised with Siegle's speculum, tumor pulsates vigorously and then blanches; reverse happens with release of pressure.
  • This results in a red reflex through the intact tympanic membrane. Sometimes, eardrum may be bluish and bulging.
  • Aquino sign is positive, i.e. blanching of mass with manual compression of ipsilateral carotid artery.
  • When the tumour perforates the eardrum a polypus will be seen in the meatus and this will bleed profusely if touched.
  • Ausculation with stethoscope over the mastoid may reveal audible systolic bruit.
  • Cranial nerve palsies is a late feature appearing several years after aural symptoms. IX' to XIIth cranial nerves may be paralysed
  • This can cause dysphagia and hoarseness, and weakness of trapezius and sternocleidomastoid muscles, unilateral paralysis of soft palate, pharynx and vocal cord.
  • Some glomus tumours secrete catecholamines and produce symptoms like tachycardia, arrhythmias, sweating, flushing and headache etc.
  • Facial palsy may be caused by glomus tympanicum type of glomus tumor.
  • Audiometry shows conductive deafness, However if inner ear is invaded, mixed conductive and sensorineural hearing loss is seen.
INVESTIGATION IN A CASE OF GLOMUS JUGULARE TUMOR
  • Plain skull radiography may show enlargement of the lateral jugular foramen and fossa.
  • Computed tomography (CT) scanning are superior at demonstrating the extent of bone destruction.
  • It shows an irregularly marginated, intensely enhancing destructive lesion centered in the jugular fossa.
  1. Phelp's sign – erosion of jugular plate.
  2. Magnetic resonance imaging (MRI) with gadolinium-diethylenetriamine pentaacetic acid (DTPA) contrast is best for delineating tumor limits.
  3. MRI shows a salt and pepper appearance which is diagnostic for paraganglioma. 
  • A combination of CT scanning and contrast MRI is the imaging regimen of choice for glomus jugulare tumors.
  • Carotid arteriography may be needed in case of larger tumors for preoperative evaluation and/or embolization
FISCH CLASSIFICATION OF GLOMUS JUGULARE TUMORS
  1. Class A: Tumors limited to the middle ear cleft.
  2. Class B: Tumors limited to the tympanomastoid area without destruction of bone in the infralabrynthine compartment.
  3. Class C: Tumors extending into and destroying the bone of the infralabrynthine and the apical compartments of the temporal bone.
  4. Class D: Tumors with intracranial intradural extension.
TREATMENT OF GLOMUS JUGULARE TUMOR
  • Surgery is the treatment of choice for glomus jugulare tumors.
  • Fisch type A tumors (see Pathophysiology) can be excised by a transmeatal or perimeatal approach.
  1. Type B tumors (see Pathophysiology) require an extended posterior tympanotomy.
  2. Type C tumors (see Pathophysiology) require radical resection via a standard combined transmastoid-infratemporal or transtemporal-infratemporal approach.
  3. Large type D tumors (see Pathophysiology) need to be treated with a combined otologic and neurosurgical approach. 
  • Surgical resection for type I tumors is relatively simple and complication free
  • Large tumors affecting the lower cranial nerves and extending beyond the petrous apex carry a significant risk of postoperative complications, especially in older patients. In such cases, embolization, radiation,gamma knife surgey or intratumoral injection of cyanoacrylate glue may be considered.
DIFFERENTIAL DIAGNOSIS OF GLOMUS JUGULARE TUMOR
  • Glomus tumor
  • Glomus Tumor arises from Glomus Bodies(a component of the dermis layer of the skin, involved in body temperature regulation)
  • Whereas Glomus Jugulare tumor is a paraganglioma which arises from glomus cell(which works as chemoreceptor)
  • Glomus tumor is a rare neoplasm arising mainly found under the nail, on the fingertip or in the foot.
  • Immunohistochemistry shows smooth muscle actin, vimentin, collagen IV and CD-57,
  • Pericyte formation may occur.
  • Sugical excision is the treatment of choice.
Exam Question
  • Most probable diagnosis in a patient presenting with Browns sign and a Mass in ear on touch bleeding heavily is Glomus jugulare tumour
  • Pulsatile Tinnitus in ear is due to Glomus jugulare tumour
  • Rising sun sign is seen in Glomus juglare
  • 9th and 10th cranial nerve may be involved in Glomus juglare.
  • Probable diagnosis in a middle aged female presenting with progressive deafness(conductive in nature) and tinnitus and Otoscopy shows a red reflex through intact tympanic membrane with Pulsation sign”positive is Glomus Jugulare Tumor.
  • Glomus Jugulare is the most common benign neoplasm of middle ear.
  • Glomus tumor is a benign tumor, therefore lymph node metastats is not present.
  • Glomus cells may be seen in Carotid body tumor.
  • Pulsation sign: When ear canal pressure is raised with Siegle’s speculum tumor pulsates vigorously and then blanches and the reverse happens when the pressure is released.
  • Probable diagnosis in a patient presenting with pulsatile tinnitus and hearing loss and a vascular mass is seen behind the tympanic membrane is Glomus Jugulare
  • Fisch classification is used for classifying -Glomus Jugulare tumor.
  • The usual location of Glomus jugular tumor is Hypotympanum.
  • Earliest symptom of glomus tumor is Pulsatile tinnitus
  • Glomus jugulare tumor arises from non-chromaffin cells.
  • Glomus jugulare tumor is more common in females.
  • Glomus jugulare tumor included in a group of tumors referred to as paragangliomas.
  • Sensorineural hearing loss may be seen in Glomus Jugulare tumor.
  • Glomus Jugulare invades labyrinth, petrous pyramid and mastoid.
  • Glomus Jugulare is not a disease of infancy.It is seen in patients with 5th-6th decade of life.
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