v

Col Anwar ul Haq

PAKISTAN

0301-8513303

• Paragangliomas (Glomus tumours) are tumours of paraganglionic tissue, which originally derive from the migration of neural crest cells during fetal development.

• Distributed predominantly throughout the middle ear, the jugular foramen, Vagus nerve, Carotid body, Upper mediastinum and retroperitoneum.

• Innervated by PS nervous system and function as chemoreceptors for circulatory regulation

PATHOLOGYPATHOLOGY• Sex M:F:: 1:6• More common in the middle age group• Sporadic ... Many reports of familial• Autosomal dominant• Non chromaffin paragangliomas with no endocrine

function• Multicentric

– presenting in both the ears– Carotid body being the 2nd most common site

PATHOLOGYPATHOLOGY......• Histologically similar appearance as glomus jugulare• Cytologically not very active with only rare mitotic figures,

have a well defined thin layer fibrous capsule• Metastases Low malignancy...cause problem because of its

location in the complex anatomy of the skull base• Others are malignant ones with both nodal and distant

metastasis.

Oldring and Fisch classificationOldring and Fisch classification

TypeA:Localized to middle ear cleft (Gl Tymp)

TypeB:Tympanomastoidtumours with no destruction of bone in the infralabyrinthine compartment of temporal bone

TypeC:Tumours invading the infralabyrinthine compartment of temporal bone

TypeD:Intracranial extension

Natural history of presentation• Slow growing so late diagnosis• Average delay 6 years• Pulsatile tinnitus Commonest• Conductive deafness• Red mass (rising sun behind the drum)• EUM...pulsation of mass, Cessation of tumour pulsation and

Blanching with +ve pressure using pneumatic otoscope (Brown sign) & blanching with ipsilat carotid art compression(aquino sign)

• Majority present at the time with cranial nerves palsies• Facial Palsy 30%• Otalgia and aural bleeding.....fairly common symptoms

D/D Red DrumD/D Red Drum

1. High jugular bulb

2. Aberrant carotid artery

3. Otitis media...obvious from hx..

4. Sq cell ca when extensive spread

5. Presenting to the neurologist cause a diagnostic problem as neuromas of last 4 cranial nerves have common symptomatology.

D/D PULSATILE TINNITUSD/D PULSATILE TINNITUS• VASCULAR ANOMALIES OF MIDDLE EAR

– Aberrant carotid art

– High Jugular bulb

– Intratympanic carotid art aneurysm..cong or aq.

• BENIGN INTRACRANIAL HYPERTENSION• Pulsatile tinnitus, Papilloedema, Raised I/cranial pressure (Turbulent flow in the Tr and

sigmoid sinus)

• Sigmoid sinus thrombosis (Tinnitus disappear on turning head to same side)

• Dural AV malformations ...depending on flow these can be life threatening so need complete investigation .(MRI with MRA)

• Jugulotympanic paragangliomas

INVESTIGATIONSINVESTIGATIONSPTAPTA: : Conductive hearing loss, often associated with a

low frequency hearing loss

TympanometryTympanometry: : May demonstrate pulsations

24 hrs urine for catecholamines, metanephrines and 24 hrs urine for catecholamines, metanephrines and VMAVMA:

Complete blood countComplete blood count

Thyroid function test..Thyroid function test..Hyperthyroidism

RadiologyRadiology

CT SCAN: CT SCAN:

--Bone erosion

-Relates extent of tumour to the bony anatomy of the ear ..vital in op planning

MRI: with gadolinium enhancementMRI: with gadolinium enhancement

-Better soft tissue involvement indication

-more difficult to correlate with the anatomy

RadiologyRadiology

4 vessel angiography 4 vessel angiography Obviated in smaller jugular tympanicus but useful in pre-op embolization

• A 20-year-old woman, June 1970 • Episodic hypertension, headaches, and palpitations. • Urine catecholamine levels were elevated• Pheochromocytoma. • Negative exploratory laparotomy. • Palsies of the IX, X, XI, and XII cranial nerves on the right

side.

• Norepinephrine-secreting glomus jugulare tumor with intracranial and cervical extension on radiologic and

arteriographic imaging.

CASE REPORTCASE REPORT

INITIAL CAROTID ARTERIOGRAMINITIAL CAROTID ARTERIOGRAM

POST EMBOLIZATIONPOST EMBOLIZATION

Bony Destruction

Normal

22 years after radiotherapy

CORRESPONDING MRI NO EVIDENCE OF TUMOUR GROWTH OVER TIME

RISING SUN BEHIND THE DRUM

AXIAL CT

JUGULAR FOSSA ENLARGED

JUGULAR FOSSA NORMAL

CORTEX ERODED

GLOMUS JUGULARE

CORTEX NORMAL

HIGH JUGULAR BULB

CORONAL CT

NORMAL CCLATERAL CC

GLOMUS TYMPAB CA

Pathologic evaluation of the tissuePathologic evaluation of the tissue

H&E stained .... Clusters of chief cellssurrounded by connective tissue stroma

Immunostaining Immunostaining for a variety of catecholamines and S100???

TREATMENT OPTIONSTREATMENT OPTIONS

No active treatment and continuous observation

Primary radiotherapy...Visible tumour shrinks and bleeding ceases, Tinnitus and vertigo improves.

Deafness and cranial nerve palsies persist• Elderly

• Infirm

Surgical resectionSurgical resection• Aim.... To remove tumour totally without increasing the pt’s

neurological deficit• Type a: external auditory approach• Type B: Combined approach (intact canal wall)• Type C: Tumour needs some skull base approach utilizing

upper cervical dissection and transmastoid approach• Type D: Skull base approach and posterior fossa craniotomy

– Some prefer to perform in 2 stages

SURGICAL TECHNIQUESSURGICAL TECHNIQUES

To Reduce Tumour VascularityTo Reduce Tumour Vascularity

• Pre op Irradiation

• Pre op Embolization (1% chance of stroke)– Gelfoam– Polyvinyl alcohol sponge

• 4-8 DAYS PRIOR TO OP

• Light GA is used

GAGA

• Highest quality of Anaesthetic help is required

• Profound hypotension is controlled by intra-arterial monitoring

• Use of Adrenaline soaked swabs reduces bleeding to an acceptable level

• TRANSMEATAL APPROACHTRANSMEATAL APPROACH

Very small tumour....Tympanotomy, if all the borders can be visualized and be cleared

• EXTENDED FACIAL RECESS APPROACHEXTENDED FACIAL RECESS APPROACH– For large Type A and small Type B tumoursCombined approach mastoidectomy– Intact canal wall instead of traditional radical cavity – By extending facial recess inferiorly reasonably good

access is obtained to the hypo tympanum, particularly if the chorda tympani is sacrificed

Even better exposure by skeletonizing the – facial nerve– Post semicircular canal – Sigmoid sinus

• INFRATEMPORAL FOSSA APPPROACHINFRATEMPORAL FOSSA APPPROACH

• Essential Features – Resection of the jugular bulb after ligating the internal

jugular vein in neck– Packing off sigmoid sinus superiorly– Anterior transposition of the facial nerve to allow

direct access to the jugular bulb region

• POSTEROLATERAL APPROACHPOSTEROLATERAL APPROACH– Modification of Fisch Lateral approach method– For many Type C and D tumours– No transposition of VII nerve– Combined posterior fossa craniotomy approach with

infratemporal fossa approach– Transposition of VII N is required in those cases with

extensive tumours around internal carotid artery– Increase chances of post op neurological deficits so

better to have restricted approach and have post op irradiation

MANAGEMENT OF SECRETORY GLOMUS JUGULARE MANAGEMENT OF SECRETORY GLOMUS JUGULARE TUMOURSTUMOURS

• Functionally active para gangliomas are on increase.• Many as phaeochromocytomas• Localization often creates diagnostic problem• Blood pressure is managed by both alpha and beta blockers

and when these tumours are removed there is a circulatory collapse. Needs massive I/V infusions and use of antigravity suits.

• 24 Hrs urine collection for VMA preoperatively

POST OP NEUROLOGICAL COMPLICATIONSPOST OP NEUROLOGICAL COMPLICATIONS• IX and X N damage at surgery Ac. Swallowing problem• Normal function by adaptation in 1-2 weeks• PEG• Temp Tracheostomy• Hoarseness Thyroplasty (If persists for more than 6

months )

Surgical resection with planned adjunctive Surgical resection with planned adjunctive radiotherapyradiotherapy

• 40% tumours continue to grow even after R.T.

• Partial resection with irradiation post-op method of choice in skull-base surgery

• I125 (long half life) newer interstitial radioactive implants