Glomus Tumour and its Approaches

glomus tumour

1. GLOMUS TUMOUR & IT’S SURGICAL APPROACHES .

2. Glomus tumour also known as chemodectoma / non chromaffin paragangloima / fibroangioma is a rare slow growing , hypervascular tumour. GLOMUS BODIES – described by Guild in 1941 as “small highly vascular mass of epitheloid cells located in the region of adventitia of the jugular bulb” . Valentine in 1840 first described it as ganglia tympanica . Misnomer. WHAT IS GLOMUS TUMOUR?

3. Paraganglions – clusters of chief cells supported by sustentacular cells and small blood vessels. Distibuted along the course of autonomic nerves in skull base , thorax & neck. Chemoreceptor tissues. Derived – cells of neural crest. Compensatory physiological response to hypoxia , hypercapnia & acidity. Secrete – catecholamines & neuropeptides

4. Paraganglion cells.

5. Largest concentration of these paraganglion cells – adrenal medulla. Temporal bone - adventitia of jugular bulb. tympanic canaliculus . retrofacial air cells. promontory & geniculate ganglion. Neck – vagal body , carotid body , along laryngeal nerve & in the mediastinum . Rosenwasser first reported origin of tumour glomus juglare in 1945.

6. Distribution of paraganglion cells .

7. Most common bening tumour of middel ear. 2 nd most common tumour of cerebropontine angle & temporal bone. Estimated annual incidence – 1/ 1.3 million ppl . 5 th & 6 th decade of life. Male to female ratio 4-6: 1. No ethnic or racial prediliction . 2 to 4 % malignant. 5 % secrete neuroactive peptide ( functional ). 10 % multicentric . MEN 2A & 2B. 10 % familial - autosomal dominant ( gene 11q23). EPIDEMIOLOGY

8. Encapsulated , slowly growing tumour , highly vascular and locally invasive. Expand within temporal bone via pathways of least resistance – air cells , vascular lumens , skull Base foramina & the eustachian tube. Invade & erode bone in lobular fashion. Intially erodes in region of jugular fossa & posteroinferior petrous bone with subsequent extension to the mastoid & adjacent occipital bone. Intracranial & extracranial extension occur. Metastasis – lung , lymph node , liver , spleen . PATHOPHYSIOLOGY

9. Glomus tympanicum – confined to middle ear space. Glomus jugulare – arise in region of jugular Glomus vagale – arise in neck , extending towards jugular foramen. Carotid body tumour – area of carotid bifurcation.

10. Most of jugulotympanic paraganglia located in the adventitia of jugular bulb. Blood supply – ascending pharyngeal artery & branches from petrous portion of ICA. ANATOMY

11. Jugular foramen :- walls are formed anterolaterally by petrous bone. Posteromedially by the occipital bone. Canal – anterior , inferior & lateral direction to exit the skull. Posterolateral portion( pars venosa ) – jugular bulb , posterior meningeal artery & cranial nerve X & XI. Anteromedial portion ( pars nervosa) – inferior petrosal sinus & IX. Jugular bulb situated between sigmoid sinus & IJV. Lower cranial nerve are situated medial to medial wall of jugular bulb . IAC , PSCC, middle ear , EAC, facial nerve & ICA within the carotid canal. Extracranial end - ICA , IJV , nerves VII, X , XI , XII within 2cm area.

12. Glomus tympanicum . I – small Tm confined to promontary . II – Tm completely filling middle ear space. III – Tm filling middle ear & extending into mastoid process. IV – Tm filling middle ear , extending into mastoid process or through TM to fill EAC , may extend anterior to IAC. I – Tm involving jugular bulb , middle ear & mastoid process. II – Tm extending under IAC ; may have intracranial extension. III – Tm extending into petrous apex ; may have intracranial extension. IV – Tm extending beyond petrous apex into clivus or infratemporal fossa ; may have intracranial extension. CLASSIFICATION GLASSCOCK – JACKSON CLASSIFICATION Glomus jugulare .

13. Type A – Tm localized to middle ear cleft. Type B – Tympanomastoid Tm with no destruction of bone in the infralabrynthine compartment of the temporal bone. Type C – Tm invading the bone of the infralabrynthine compartment of temporal bone. C1 – Tm with limited involvement of vertical portion of carotid canal. C2- Tm invading vertical portion. C3- Tm invading the horizontal portion. Type D – Tm with intracranial extension. FISCH classification of glomus tumour.

14. Type A Type B

15. Type C1 Type C2

16. Type C3 Type D

17. CLINICAL PRESENTATION Tm of slow growth with average delay of 6 years. When Tm is intratympanic – earliest & most common symptom is deafness & pulsatile tinnitus ( swishing character , synchronous with pulse ). Deafness – mainly conductive & mixed. When Tm present as polyp – h/o profuse bleeding. Earache( uncommon) , aural fullness. Otorrhoea – 2 nd infection with polyp. Dizziness , vertigo & facial palsy.

18. Cranial nerve palsies – Tm invade jugular foramen & base of skull. late feature appear several years after aural symptoms. Cranial nerve IX- XI with or without XII.( jugular foramen / Vernet’s syndrome). Dysphagia & hoarseness with u/l paralysis of soft palate , loss of gag reflex , parotid glan d secretions with weakness of trapezius & scm m uscle.

19. May present as mass over mastoid or through ET as mass in nasopharynx . Intracranial extension – headache , hydrocephalus & raised ICT. Metastasis – lymph node mass. Functional Tm - headache , palpitations , tachycardia, sweating , hypertension & anxiety.

20. Otoscopic examination – characterstic , pulsatile , reddish – blue mass behind the TM . Rising sun appearance – Tm arise from floor of middle ear cavity. Polyp can be seen. Brown’s sign – when EAC pressure is raised with siegle’s speculum Tm pulsates vigourously & then blanches ; reverse happens with release of pressure. EXAMINATION

21. Aquino sign – blanching of mass with ipsilateral carotid artery compression. Cranial nerve palsies. Auscultation – systolic bruit over mastoid. Audiological examination – conductive & or mixed hearing loss. Other features according to extension

22. Determination of Tm size , type & extent. Evaluation of histochemical or multicentric associated lesion. Identification & assessment of ICE. Assessment of major vasculature involvement. Assessment of intracranial collateral circulation. TREATMENT PLANNING

23. H/o & physical examination. Routine investigations. Audiological investigation. Serum catecholamine levels. 24 hr urine for catecholamine , metanephrine & VMA. DIAGNOSIS

24. Mainstay of diagnosis is radiological imaging. CT scan MRI Angiography. Radionuclide scintigraphy . CT scan – Both axial & coronal plane extent of lesion bony erosion. Caroticojugular plate. Phelp’s sign

25. CT scan

26. MRI with gadolinium enhancement – Tm extent Intracranial extension Relationship of Tm to neural & vascular structures . Salt and pepper pattern ( vascular flow voids). Angiography – vascular supply of Tm . Evaluating ICA involvement. Preop embolization of Tm. Large Tm involving ICA , cross compression or trial of balloon occlusion is recommended.

27. MRI

28. ANGIOGRAPHY

29. Definitive or palliative. Surgery , irradiation , embolization & chemotherapy. Definitive T/t is surgery. Palliative – elderly , medically unfit or those with multicentric lesion, possible postoperative weakness of vagus or hypoglossal , or both nerves. Asymptomatic patients – GT observed with serial imaging. MANAGEMENT

30. Relative effectiveness is controversial. Primarily, palliatively & as surgical salvage. Fibrosis of arterioles rather then direct affect on Tm cells. Tm shrinks , bleeding ceases. Tinnitus & vertigo improve. Deafness & cranial nerve palsy persist. Radiotherapy with or without intensity modification Stereotactic radiotherapy , achieve Tm control rate of 80 – 90 %. Contraindicated – ICE & osteomyelitis . S/E – ICA thrombosis, CSF leak , necrosis of bone, brain & dura , pitutary – hypothalamic insufficency . Radiation therapy

31. Aim is complete resection with minimal neurological deficit. Type A – Glomus tympanicus approached via transmeatal route. Type B – Combined approach ( extended facial recess ) Type C – Skull base approach utilizing upper cervical dissection and transmastoid approach. Type D – skull base approach with posterior fossa craniotomy. SURGICAL RESECTION

32. Reduce Tm vascularity Pre op irradiation. Pre op embolization . SURGICAL TECHNIQUES

33. CLASSIFICATION SURGICAL APPROACH Tympanic Transcanal Tympanomastoid Mastoid/extended facial re cess Jugular bulb mastoid/neck (facial rerouting) Carotid artery infratemporal fossa Transdural infratemporal fossa / intracranial Craniocervical transcondylar Vagale cervical Modified De La Cruz Glomus Tm classification

34. If the margins of the lesion are visible around the entire circumference of the lesion. Middle ear is exposed via tympanomeatal flap. Malleus may be seperated from the TM to gain additional exposure. Limits of Tm confirmed. Microbipolar cautery is applied to the feeding vessels & margins , and Tm is avulsed. Tm is seperated from its attachement to promontary mucosa. Nerve of tympanic plexus is also involved. TRANSMEATAL APPROACH (type A )

35. Tympanomastoid Glomus Tm. Postauricular incision made & complete mastoidectomy done. Facial recess is opened. Further bone is removed inferiorly with sacrifice of chorda tympani. Expose the middle ear & hypotympanum . EXTENDED FACIAL RECESS APPROACH

36. Tm may be removed from ossicular chain , facial nerve & IJV. Using bipolar cautery shrinking of Tm & controlling the blood supply. TM may be removed if it is involved. If necessary posterior wall of EAC is removed.

37. Tm involving jugular bulb but doesn’t extend into ICA or neck or PCF. Same exposure as extended facial recess approach. Additional amputation of mastoid tip done. Periosteum of diagastric groove is followed anteriorly till it turns abruptly at stylomastoid foramen. Drilling is done laterally along the ridge. Free the mastoid tip. Tip is removed. Incision is extended along anterior border of SCM. Mastoid neck approach.

38. Posterior belly of diagastric muscle is identified. Freed from diagastric groove. Retracted anteriorly . Neurovascualr structures are exposed. IJV is identified & ligated . Jugular vein followed over the transverse process of 1 st cervical vertebra into base of skull. Cranial nerve XI (lateral to vein ) identified. IX , X identified & preserved. Expose SS & JB.

39. Proximal SS controlled with extraluminal packing of surgicell . SS may be opened distal to the proximal packing. Tm is resected . Tm & ossicles if involved should be removed.

40. Periosteum of the facial nerve at the stylomastoid foramen is preseved . Fibrous attachement of nerve in its vertical part is transected. Nerve is transported with the periosteum at the attached belly of diagastric muscle. Less chances of injury to nerve. Mastoid neck with limited facial nerve rerouting.

41. Transdural extension of the Tm. Infratemporal approach.

42. Bleeding. Uncontrolled hyper/ hypotension Cranial nerve palsises . CSF leak. Meningitis. Hearing loss. TM perforation . EAC stenosis . Tumour regrowth . Death. Complications

43. Radiological & when indicated endocrinological monitoring of tumour regrowth is indicatedevery 6 months to 1 year for 2 year , then depending upon the findings every 2 year. Follow up

44. Glomus jugulare Tm grow slowly & may lead to cranial nerve palsy. Which is benign . However , recent studies have shown long term reduced quality of life. Mortality rate is6.2 % in patients treated with radiation. 2.5 % in surgically treated patients. Overall mortality is 8.7%. Outcome & prognosis.

45. Surgery is the treatment of choice. Effectiveness will improve with intraoperative imaging & guiding system. Recent genetic research on familial glomus tumour suggest future direction of tumour towards gene manipulation. FUTURE

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