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 predominantlythroughoutthe middle ear, the jugular foramen, Vagus nerve, Carotid body, Upper mediastinum and retroperitoneum.
  • Innervated byPS nervous system and function as chemoreceptors for circulatory regulation

GLOMUS TUMOURS 4.

  • PATHOLOGY
  • SexM: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 ndmost 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
  • MetastasesLow 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 nodestruction of bone in the infralabyrinthinecompartment of temporal bone
  • TypeC: Tumours invading the infralabyrinthinecompartment of temporal bone
  • TypeD: Intracranial extension

GLOMUS TUMOURS 7. 8.

  • Natural history of presentation
  • Slow growing so late diagnosis
  • Average delay 6 years
  • Pulsatile tinnitusCommonest
  • Conductive deafness
  • Red mass (rising sunbehind 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 needcomplete 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 angiographyObviated 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 extensionon radiologic and arteriographic imaging.

CASE REPORT 15. INITIAL CAROTID ARTERIOGRAM 16. POST EMBOLIZATION 17. Bony Destruction Normal 18. 22 years after radiotherapy 19. CORRESPONDINGMRI NO EVIDENCE OF TUMOURGROWTHOVER 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
  • Immunostainingfor a variety of catecholamines and S100???

GLOMUS TUMOURS 22.

  • TREATMENT OPTIONS
  • No active treatmentand 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 pts 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 i