Glomus Jugulare

Finger [Ref: CSDT I3/e p1148 (12/c p1266); Anderson 10/e p2559; http://emedicine.medscape.com/article/1083405-overview; http://wwwpubmedcentraLnih.gov/articlerenderjegi?artid=1888624%5D

• The glomus tumor is a rare and benign vascular neoplasm that arises from the neuroarterial structure called a glomus body. The normal glomus body is located in the dermis throughout the body, but is more concentrated in the digits. They are believed to function in thermal regulation.
• The most common site of glomus tumors is subungual and 75% of the lesions occur in the hand.
• The lesions present most frequently during the fourth and fifth decade of life although they can be found in any age and at any site.
• The subungual tumors affect women three times more commonly than men.
• The lesions are usually solitay but mulifocal tumors are also seen.
• Clinically, glomus tumors are characterized by a triad of

– sensitivity to cold,

– localized tenderness and

– severe intermittent pain.

The pain can be excruciating and is described as a burning or bursting.

• Grossly, the tumors are usually less than one cm. in size, and appear as small red-blue nodules. The lesion appears as a localized dark red or blue lesion beneath the finger nail, but the subungual lesions can be difficult to detect on clinical examination.
• Two useful tests for diagnosing glomus tumors, particularly solitary painful glomus tumors (especially those under a nail) are the following:

– disappearance of pain after application of a tourniquet proximally on the arm

– eliciting pain by applying pressure to a precise area with the tip of a pencil

• Treatment of glomus tumors consists of surgical excision.

Glomus tumor is a benign soft-tissue extremity tumors, these lesions arise from dermal neuro myo arterial apparatus (glomus bodies).

Glomus tumor more commonly affects the hand.

Glomus jugulare  arise from juglar bulb and extend upward with erosion into the hypotympanum.

• They arise either from the middle ear (glomus tympanicum) or arise from juglar bulb with upward erosion into the hypotympanum (glomus jugulare).
• Patients clinically presents with pulsatile tinnitus and hearing loss.
• On examination a vascular mass is visible behind an intact tympanic membrane
  Rising sun sign is seen when the tumor arise from floor of middle ear cavity. 
• Pulsation sign (Browns sign) is positive i.e when ear canal pressure is raised using Siegel’s speculum tumor pulsates vigorously and then blanches, reverse happens with release of pressure.
• Large glomus jugulare tumors are associated with multiple cranial neuropathies, especially involving nerves VII, IX, X, XI, and XII. 
• Treatment modalities includes surgery, radiotherapy, or both.

Glomus tumour originates from the glomus bodies. It found in the dome of jugular bulb or on the promontory along the course of tympanic branch of IXth cranial nerve.

More than 200 syndromes are associated with hearing loss. Common syndromic forms of hearing loss, among others, include the following: (1) Usher syndrome (retinitis pigmentosa and hearing loss), (2) Waardenburg syndrome (pigmentary abnormality and hearing loss), (3) Pendred syndrome (thyroid organification defect and hearing loss), (4) Alport syndrome (renal disease and hearing loss), and (5) Jervell and Lange-Nielsen syndromes (prolonged QT interval and hearing loss).

The two grading systems which has been developed to classify glomus tumors are Fisch classification and Glasscock – Jackson classification.

Fisch classification of glomus tumors:

Class A: Tumors limited to the middle ear cleft.
Class B: Tumors limited to the tympanomastoid area without destruction of bone in the infralabrynthine compartment.
Class C: Tumors extending into and destroying the bone of the infralabrynthine and the apical compartments of the temporal bone.
Class D: Tumors with intracranial intradural extension.

Ans. is ‘a’ i.e., Carotid body tumor

o Non-chromaffin (neuroendocrine) cells of paraganglioma are also called glomus cells.

Ans. is ‘a’ i.e., CD-57

o Glomus tumor is a benign mesenchymal neoplasm of the subcutaneous tissue of the distal extremities and head & neck region.

o Immunohistochemistry shows smooth muscle actin, vimentin, collagen IV and CD-57, with little to no expression of neuroendocrine, endothelial or epithelial markers.

o Markers that have been proved consistently negative include cytokeratin, synaptophysin, chromogranin A, CD-31 and S-100 protein.

Ans. is ‘c’ i.e., Distal portion of digits

o It is a benign but painful tumor that arises from the modified smooth muscle cells of the glomus body, a specialized arteriovenous anastomosis that is involved in thermoregulation.

o They are most commonly found in the distal portion of the digits, especially under the fingernails.

Ans. is ‘b’ i.e., Glomus tumor ‘c’ i.e., Hemangiopericytoma

o The pericytes are a special variety of cells placed at intervals along certain capillaries. Pericytes are also known as

Rouget cells, mural cells, or adventitial cells.

o The tumors of pericytic differention are :‑

i)        Hemangiopericytoma      iii) Myopericytoma                v) Myofibroma

ii)       Glomus tumor               iv) Glomangiomyoma          vi) Angioleiomyoma

Note :- For exams like AI and AIIMS, hemangiopericytoma is best answer because It is related most definitly to pericytic differentiation.

Ans is d i.e. Finger 

• The glomus tumor is a rare and benign vascular neoplasm that arises from the neuroarterial structure called a glomus body. The normal glomus body is located in the dermis throughout the body, but is more concentrated in the digits. They are believed to function in thermal regulation.
• The most common site of glomus tumors is subungual and 75% of the lesions occur in the hand.
• The lesions present most frequently during the fourth and fifth decade of life although they can be found in any age and at any site.
• The subungual tumors affect women three times more commonly than men.
• The lesions are usually solitay but mulifocal tumors are also seen.
• Clinically, glomus tumors are characterized by a triad of

– sensitivity to cold,

–  localized tenderness and

– severe intermittent pain.

The pain can be excruciating and is described as a burning or bursting.

• Grossly, the tumors are usually less than one cm. in size, and appear as small red-blue nodules. The lesion appears as a localized dark red or blue lesion beneath the finger nail, but the subungual lesions can be difficult to detect on clinical examination.
• Two useful tests for diagnosing glomus tumors, particularly solitary painful glomus tumors (especially those under a nail) are the following:

–  disappearance of pain after application of a tourniquet proximally on the arm

–  eliciting pain by applying pressure to a precise area with the tip of a pencil

– Treatment of glomus tumors consists of surgical excision.

Pulsatile tinnitus   – Seen in Glomus tumor

Pulsatile otorrhea  – Seen in ASOM

Explanation

• Glomus tumor is more common in females.
• Glomus tumor is also referred to as chemodectomy or nonchromaffin paraganglion.
• Glomus tumor is a benign tumor, therefore lymph node metastats is not present.
• Multicentric tumors are found in 3-10% of sporadic cases and in 25-50% of familial cases.
• Fluctuating (Pulsatile) tinnitus and conductive hearing loss are the earliest symptoms of glomus tumor.

Phelp’s sign

Rising sun sign                           are all seen in Glomus tumor

Aquino sign

Pulsation sign/brown sign

Phelp sign This sign is seen on CT scan

In CT- in case of glomus jugulare tumor the normal crest between the carotid canal and jugulare tumor is absent whereas it is not so in case of glomus tympanicum

MRI – gives soft tissue extent of tumor; Magnetic Resonance Angiography (MRA) shows compression of the carotid artery whereas magnetic resonance venography shows invasion of jugular bulb by the tumor.

Ans. C i.e. Mesoderm

They are included in a group of tumors referred to as paragangliomas, which occur at various sites and include carotid body, glomus vagale, and glomus tympanicum tumors

Glomus jugulare tumors occur predominantly in women in the fifth and sixth decades of life. Because of the insidious onset of symptoms, these tumors often go unnoticed, and delay in diagnosis is frequent.

The most common symptoms are conductive hearing loss and pulsatile tinnitus. Other aural signs and symptoms are ear fullness, otorrhea, hemorrhage, bruit, and the presence of a middle ear mass. Significant ear pain is uncommon.

Glomus cells are derived from neural crest which itself is derivative of neuroectoderm.

• Other derivates of neural crest are‑

a) Neural derivatives

• Sensory neurons of spinal dorsal root ganglia.
• Sympathetic chain ganglia and plexus (celiac/preaortic/renal ganglia, enteric plexus in GIT, i.e. Auerbachs and Meissner’s)
• Parasympathetic ganglia and plexus of GIT.
• Schwann cells of peripheral nerves, satellite cells of all ganglia.
• Adrenal medulla, chromaffin cells, para follicular C-cells of thyroid gland.
• Melanocytes and melanoblasts.

b) Mesenchymal derivatives

• Dermal bones of skull : Frontal, parietal, temporal, nasal, vomer, palatine, mandible, maxillae.
• Leptomeninges : arachnoid and pia mater (Durameter is mesodermal).
• Dentine of teeth (odontoblasts).

Eye : choroid, sclera, iris epithelium, pupillary muscles (sphincter and dilator pupillae, ciliary muscles).

• Pharyngeal arch cartilages.
• Retinal pigmented epithelium.
• Connective tissues of head including dermis, tendon, ligaments.
• Bulbar and conal ridges of heart.

Neural crest

Ans. is ‘d’ i.e., Presbyacusis

Tinnitus

• Tinnitus is ringing sound or noise in the ear.
• The characteristic feature is that the origin of this sound is within the patient.

Ans. is ‘a’ i.e., Hypotympanum

There are two types of glomus tumors:-

i) Glomus jugulare

These glomus tumors arise from the dome of the internal jugular vein in the hypotympanum and jugular foramen. In jugular foramen they can invade IX to XII cranial nerves.

ii) Glomus tympanicum

They arise from the promontory of the middle ear along the course of the tympanic branch of the IXth cranial nerve.

Ans. is ‘a’ i.e., Pulsatile tinnitus

Clinical features of glomus tumor

• The earliest symptoms of glomus tumour is pulsatile tinnitus (earliest) and hearing loss. Hearing loss is conductive and slowly progressive. These are followed by blood stained otorrhoea and earache.
• 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. This results in a red reflex through the intact tympanic membrane. Sometimes, eardrum may be bluish and bulging.
• 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.
• 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.
• Cranial nerve palsies is a late feature appearing several years after aural symptoms. IX’ to XII^th 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.
• Ausculation with stethoscope over the mastoid may reveal audible systolic bruit.
• 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.