Glomus tumours pakistan
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  • 1.  
  • 2. v
    • Col Anwar ul Haq
    • PAKISTAN
    • 0301-8513303
    GLOMUS TUMOUR
  • 3.
    • 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
    GLOMUS TUMOURS
  • 4.
    • PATHOLOGY
    • 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 2 nd most common site
    GLOMUS TUMOURS
  • 5.
    • PATHOLOGY ...
    • 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.
    GLOMUS TUMOURS
  • 6.
    • Oldring 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
    GLOMUS TUMOURS
  • 7.  
  • 8.
    • 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
    GLOMUS TUMOURS
  • 9.
    • D/D Red Drum
    • High jugular bulb
    • Aberrant carotid artery
    • Otitis media...obvious from h x. .
    • Sq cell ca when extensive spread
    • Presenting to the neurologist cause a diagnostic problem as neuromas of last 4 cranial nerves have common symptomatology.
    GLOMUS TUMOURS
  • 10.
    • D/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
    GLOMUS TUMOURS
  • 11.
    • INVESTIGATIONS
    • PTA : Conductive hearing loss, often associated with a low frequency hearing loss
    • Tympanometry : May demonstrate pulsations
    • 24 hrs urine for catecholamines, metanephrines and VMA :
    • Complete blood count
    • Thyroid function test.. Hyperthyroidism
    GLOMUS TUMOURS
  • 12.
    • Radiology
    • CT SCAN:
    • - Bone erosion
    • -Relates extent of tumour to the bony anatomy of the ear ..vital in op planning
    • MRI: with gadolinium enhancement
    • -Better soft tissue involvement indication
    • -more difficult to correlate with the anatomy
    GLOMUS TUMOURS
  • 13.
    • Radiology
    • 4 vessel angiography Obviated in smaller jugular tympanicus but useful in pre-op embolization
    GLOMUS TUMOURS
  • 14.
    • 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 REPORT
  • 15. INITIAL CAROTID ARTERIOGRAM
  • 16. POST EMBOLIZATION
  • 17. Bony Destruction Normal
  • 18. 22 years after radiotherapy
  • 19. CORRESPONDING MRI NO EVIDENCE OF TUMOUR GROWTH OVER TIME
  • 20. GLOMUS TUMOURS RISING SUN BEHIND THE DRUM AXIAL CT JUGULAR FOSSA ENLARGED JUGULAR FOSSA NORMAL CORTEX ERODED GLOMUS JUGULARE CORTEX NORMAL HIGH JUGULAR BULB Radiology CORONAL CT NORMAL CC LATERAL CC GLOMUS TYMP AB CA
  • 21.
    • Pathologic evaluation of the tissue
    • H&E stained .... Clusters of chief cellssurrounded by connective tissue stroma
    • Immunostaining for a variety of catecholamines and S100???
    GLOMUS TUMOURS
  • 22.
    • TREATMENT 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
    GLOMUS TUMOURS
  • 23.
    • Surgical 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
    GLOMUS TUMOURS
  • 24.
    • SURGICAL TECHNIQUES
    • To 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
    GLOMUS TUMOURS
  • 25.
    • GA
    • 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 APPROACH
    • Very small tumour....Tympanotomy, if all the borders can be visualized and be cleared
    GLOMUS TUMOURS
  • 26.
    • EXTENDED FACIAL RECESS APPROACH
      • For large Type A and small Type B tumours
      • Combined 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
    GLOMUS TUMOURS
  • 27.
    • INFRATEMPORAL 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
    GLOMUS TUMOURS
  • 28. GLOMUS TUMOURS
  • 29.
    • POSTEROLATERAL 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
    GLOMUS TUMOURS
  • 30.
    • MANAGEMENT OF SECRETORY GLOMUS JUGULARE TUMOURS
    • 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
    GLOMUS TUMOURS
  • 31.
    • POST 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 )
    GLOMUS TUMOURS
  • 32.
    • Surgical resection with planned adjunctive radiotherapy
    • 40% tumours continue to grow even after R.T.
    • Partial resection with irradiation post-op method of choice in skull-base surgery
    • I 125 (long half life) newer interstitial radioactive implants
    GLOMUS TUMOURS
  • 33. THANK YOU