2. Glomus Tumours
They are the most common benign tumours that arise
within the temporal bone
Also known as
Paraganglioma or Chemodectoma
3. Glomus Tumours
Also known as
Paraganglioma or Chemodectoma
They are slowly growing , benign , nonencapsulated but
extremely vascular neoplasm.
4. Glomus Tumours
Also known as
Paraganglioma or Chemodectoma
They arise from the chemoreceptor tissue
along the parasympathetic nerves in
skull base, neck and thorax.
5. Familial distribution in 10% of cases (MEN syndrome).
10 %
Secrete catecholamines in approximately 10% of cases.
10% have multiple tumours.
10 % will show malignant transformation with rapid bony erosion,
metastases and short survival
7. Splays the carotid bifurcation.
Classification
Glomus tympanicum
Glomus jugulare
Glomus vagale
Carotid body tumour
The tumour is confined to the middle ear space.
Arises in the jugular foramen may extend upwards to fill
the middle ear.
Arises in the neck extending to the jugular foramen.
8. Tumour with intracranial extension.
Type A
Type B
Type C
Type D
The tumour is confined to the middle ear space.
Tumour in the middle ear and mastoid with no
destruction of bone in the infra-labyrinthine compartment
Tumour invading the bone in the infra-labyrinthine
compartment of the temporal bone.
Fisch classification of middle
ear glomus tumours
11. Vagal paragangliomas present similarly but can arise from any part of the
cervical vagus nerve so may additionally cause:
1. Dysphonia .
2. Dysphagia.
3. Apiration.
12. CBTs typically present as a slowly enlarging , painless, lateral neck
masses. As they enlarge , they will impinge on local structures causing
dysphagia, CN palsies and hornour`s syndrome.
16. Browne sign: it is blanching of the TM on pneumatic otoscopy.
Aquino sign: it is cessation of the pulsation of the tumour with ipsilateral
compression of the carotid artery.
17. CBTs typically present as a slowly enlarging , painless, lateral neck
masses. As they enlarge , they will impinge on local structures causing
dysphagia, CN palsies and hornour`s syndrome.
18. Examination will reveal a non tender neck mass at the anterior border of
SCM that is mobile in the horizontal and not the vertical plane (Fontaine
sign).
19. Differential diagnosis of a reddish
mass behind the TM:
1. Glomus Tympanicum
2. Haemotympanum
3. High Jugular bulb.
4. Aberrant carotid artery.
5. Aneurysm of carotid artery.
6. Cholesterol Granuloma.
7. Metastatic disease.
20. - History and clinical examination.
- Audiometric tests
- Urine catecholamines
Diagnosis
21. 1. CT scan to assess size tumour and bone erosion.
2. MRI scan to assess intracranial extent and exclude multiple tumours.
Typical ‘salt and pepper’ appearance due to areas of slow and fast blood flow
within the tumour.
3. Angiography for diagnostic purposes and pre-operative embolization.
A carotid balloon occlusion test can be performed during the
angiography to assess collateral circulation
Imaging
(CT , MRI , MRA)
27. If the tumour is secreting , what would you like to exclude?
Is there any special precaution to take before surgery?
Urine catecholamines (adrenaline, noradrenaline) and their
metabolites (vanillylmandelic acid, VMA) in a 24h urine
collection.
Questions
Diagnosis
28. Mainsaty of treatment is surgery with radiotherapy reserved
for frail patients and recurrent/residual disease.
Treatment
Depending on tumour and patient factors, the treatment intent can be
curative or palliative, and may involve surgery, radiotherapy, or
chemotherapy
29. - Glomus tympanicum tumours (or Fisch type A) can be excised via a
transcanal approach, using laser or diathermy for heamostasis.
- Type B glomus tumours need a post-auricular approach, as in mastoid
exploration, with extended facial-recess dissection to allow exposure of the
jugular bulb area.
- Larger glomus jugulare tumours (types C and D) are usually embolized pre-
operatively, the major vessels must be controlled in the neck and neurosurgical
input may be necessary to deal with any intracranial component. The
transtemporal skull-base or infratemporal approaches are used.
Treatment
It is the Most common benign tumour in the temporal bone.
It is the Most common benign tumour in the temporal bone.
It is the Most common benign tumour in the temporal bone.
- Large tumours can expand laterally to the tympanic membrane causing otalgia and otorrhoea (blood and/or suppuration), or medially invading the labyrinth (sensori-neural hearing loss and vertigo) and involving cranial nerves VII, IX, X, XI, XII, and VI.
- Large tumours can expand laterally to the tympanic membrane causing otalgia and otorrhoea (blood and/or suppuration), or medially invading the labyrinth (sensori-neural hearing loss and vertigo) and involving cranial nerves VII, IX, X, XI, XII, and VI.
Otoscopic examination reveals a red-blue mass behind the tympanic membrane. Glomus jugulare, will errode the floor of the middle ear giving the typical ‘rising sun’ appearance on otoscopy.
- Otoscopic examination reveals a red-blue mass behind the tympanic membrane. Glomus jugulare, will errode the floor of the middle ear giving the typical ‘rising sun’ appearance on otoscopy.
- The mass blanches when compressed using the pneumatic Siegle’s speculum.
Heterogenous hypervascular well defined strongly enhancing mass at carotid bifurcation splaying the ICA and the ECA
If the tumour is secreting, it is necessary to exclude an associated phaeocromocytoma (MEN syndrome) with an MRI of the adrenal glands.
Patients with secreting tumours need to have alpha- and beta-blockade prior to surgical treatment and intra-operative magnesium infusion to
protect against the potentially devastating cardiovascular effects of any catecholamines released during handling of the tumour