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GLOMUS TUMOUR
MODERATOR : DR PRASHANTH BABU
PRESENTER : DR SREEJITH BOBAN
Learning Obectives
• Definition
• Etiology
• Epidemiology
• Pathophysiolgy
• Clinical features
• Signs and symptoms
• Differential diagnosis
WHAT IS GLOMUS
TUMOUR?
Glomus tumour
Glomus tumour / paraganglioma was coined by Kohn
1840 – Valentine described it first as Ganglia Tympanica
1924 - Lattes and Waltner termed glomus jugulare which was originally termed
as glomus jugularis
1945 – Rosenwasser was first to diagnose a patient with glomus tumour and its
surgical excision
Glomus tumour
Latin word - ball of thread or yarn
Arise from glomus bodies
Component of the dermis layer of the skin
Synonyms:
Chemodectoma
Paraganglioma
Ganglia tympanica
Vascular tumuor of middle ear
Glomus tumour
They are highly vascular, slow growing, benign tumour arising from
paraganglionic glomus tissue
These cells are derivatives of non chromaffin ganglionic of neuroectodermal
origin and have secretory capacity
Glomus tumour
The paraganglionic cells are distributed as :
1. Carotid body
2. Ciliary and vagal bodies along the aorta
3. Bladder
4. Adrenal gland
Glomus tumour
In temporal bone, glomus bodies are close
related with :
1. Tympanic branch of glossopharyngeal
nerve
2. Auricular branch of vagus nerve
3. Adventitia of jugular bulb
4. Promontory
Glomus tumour
Classification:
Primary tumour
◦ Benign - Glomus tumour
◦ Malignant - carcinoma, sarcoma
Secondary tumour
Glomus tumour
Classification of glomus tumour
1. Glomus tympanicus
2. Glomus jugular
3. Glomus vagale
Glomus tumour
Aetiology
• Sporadic
• Familial : 10-50 % Autosomal dominant with paternal imprinting
• Genetic
gene responsible is located in chromosome 11q13, 11q23
Glomus tumour
Epidemiology:
• More common in Caucasians
• Female dominant (M:F - 1:6)
• 40- 50 years
•Autosomal Dominant mode of transmission
PATHOLOGY
Glomus tumour
Adrenal Paraganglioma :
◦ Nor epinephrine is secreted
Non Adrenal Paraganglioma :
◦ Rarely secrete epinephrine
◦ Phenyl etholamine–N-Methyl Tranferase deficiency
Glomus tumour
Pathology:
Hypervascular tumours (non encapsulated) arising from the glomus bodies
Initially erodes in region of jugular fossa and posteroinferior petrous bone with
subsequent extension to the mastoid and adjacent occipital bone.
Few cases may go for malignant changes and metastasis
Glomus tumour
Histology:
▪ It shows masses and sheets of epithelial cells
▪ Large nuclei
▪ Granular cytoplasm
▪ Abundance of thin wall blood sinusoids with no
contractile muscle coat
Glomus tumour
Histology:
• Chief cells - catecholemine secreting cells
• Sustentacular cells - supporting cells
• Surrounded by fibromusclar stroma/ vessels
• The pattern these cells arranged are called as
Zallballen
SYMPTOMS
Glomus tumour
Symptoms:
Intratympanic tumour
1. Hearing loss
2. Tinnitus
Glomus tumour
Symptoms:
Ear polyp
• Profuse ear bleeding
• Otorrhea
• Dizziness or vertigo
Glomus tumour
Symptoms:
9th - 12th cranial nerve palsies (late features)
• Dysarthria, Dysphasia, Hoarseness of voice, Paralysis of soft palate, pharynx
• Vocal cord involved
• Weakness of trapezius, sternocleidomastoid muscle
• Atrophy of tongue
Glomus tumour
Symptoms:
Catecholemine features
• Headache
• Sweating
• Palpitations
• Hypertension
• Anxiety
Glomus tumour
Symptoms:
Rule of 10s.
• 10% of the tumours are familial
• 10% multicentric (occurring in more than one site)
• Up to 10% functional, i.e. they secrete catecholamines.
Glomus tumour
Otoscopic stages:
1. Hyper vascular stage
2. Tympanic stage
3. Polypoidal stage/ hemorrhagic stage
SIGNS
Glomus tumour
Signs:
Red reflex
Glomus tumour
Signs:
Rising sun appearance
Glomus tumour
Signs:
Pulsation sign (Browns sign)
- Siegel’s speculum is used here.
Glomus tumour
Signs:
Aquino sign :
Cessation of pulsations with compression of the ipsilateral carotid artery
Glomus tumour
1. Fisch classification
2. Lundgrens classification
3. Bickers and Howell classification
4. Jacksons and glascock classification
Glomus tumour
Fisch classification
Type A - tumour located in the middle ear
Type B - tumour located in the tympanomastoid area with no involvement of
infralabyrinthine compartment
Type C - tumour originates in the dome of the jugular bulb destroying the overlying
bone.
Type D - De1 – extradural, extension <2cm
De2 - extradural, extension >2cm
Di1 - intradural, extension <2cm
Di2 - intradural, extension >2cm
Di3 – intradural, unresectable
Glomus tumour
Jacksons and glascock classification
Tympanicum
Type I - Tumour involves only promontory
Type II - Tumour involves middle ear space
Type III - Tumour involves the middle ear mastoid
Type IV - Tumour involves middle ear, mastoid and external auditory canal
Glomus tumour
Jacksons and glascock classification
Jugulare
Type I - Tumour involves jugular bulb, middle ear and mastoid
Type II - Tumour extends beneath the internal acoustic meatus
Type III - Tumour involves the petrous apex
Type IV - Tumour involves the infratemporal fossa
Glomus tumour
Spread of tumour:
• The tumour always prefers the
pathway that offers minimal
resistance.
• Air cell tracts, vascular channels,
naturally occurring fissures and
foramina.
• The pneumatized air cells tracts
of the temporal bone are the most
important route of spread
Glomus tumour
Differential diagnosis :
1. Otitis media
2. Idiopathic haemotympanum
3. Primary brain tumour
4. Cholesterol granuloma
5. Aberrant intrapetrous internal carotid artery
6. Aneurysm
7. Arteriovenous malformation
8. Prominent jugular bulb
9. Persistent stapedial artery
Glomus tumour
SUMMARY-
References
1. Scott-Brown's Otorhinolaryngology and Head and Neck Surgery, Eighth
Edition
2. Glasscock-Shambaugh Surgery of the Ear
3. Otolaryngologic Clinics of North America - Journal - Elsevier
THANKYOU

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Glomus tumour

  • 1. GLOMUS TUMOUR MODERATOR : DR PRASHANTH BABU PRESENTER : DR SREEJITH BOBAN
  • 2. Learning Obectives • Definition • Etiology • Epidemiology • Pathophysiolgy • Clinical features • Signs and symptoms • Differential diagnosis
  • 4. Glomus tumour Glomus tumour / paraganglioma was coined by Kohn 1840 – Valentine described it first as Ganglia Tympanica 1924 - Lattes and Waltner termed glomus jugulare which was originally termed as glomus jugularis 1945 – Rosenwasser was first to diagnose a patient with glomus tumour and its surgical excision
  • 5. Glomus tumour Latin word - ball of thread or yarn Arise from glomus bodies Component of the dermis layer of the skin Synonyms: Chemodectoma Paraganglioma Ganglia tympanica Vascular tumuor of middle ear
  • 6. Glomus tumour They are highly vascular, slow growing, benign tumour arising from paraganglionic glomus tissue These cells are derivatives of non chromaffin ganglionic of neuroectodermal origin and have secretory capacity
  • 7. Glomus tumour The paraganglionic cells are distributed as : 1. Carotid body 2. Ciliary and vagal bodies along the aorta 3. Bladder 4. Adrenal gland
  • 8. Glomus tumour In temporal bone, glomus bodies are close related with : 1. Tympanic branch of glossopharyngeal nerve 2. Auricular branch of vagus nerve 3. Adventitia of jugular bulb 4. Promontory
  • 9. Glomus tumour Classification: Primary tumour ◦ Benign - Glomus tumour ◦ Malignant - carcinoma, sarcoma Secondary tumour
  • 10. Glomus tumour Classification of glomus tumour 1. Glomus tympanicus 2. Glomus jugular 3. Glomus vagale
  • 11. Glomus tumour Aetiology • Sporadic • Familial : 10-50 % Autosomal dominant with paternal imprinting • Genetic gene responsible is located in chromosome 11q13, 11q23
  • 12. Glomus tumour Epidemiology: • More common in Caucasians • Female dominant (M:F - 1:6) • 40- 50 years •Autosomal Dominant mode of transmission
  • 14. Glomus tumour Adrenal Paraganglioma : ◦ Nor epinephrine is secreted Non Adrenal Paraganglioma : ◦ Rarely secrete epinephrine ◦ Phenyl etholamine–N-Methyl Tranferase deficiency
  • 15. Glomus tumour Pathology: Hypervascular tumours (non encapsulated) arising from the glomus bodies Initially erodes in region of jugular fossa and posteroinferior petrous bone with subsequent extension to the mastoid and adjacent occipital bone. Few cases may go for malignant changes and metastasis
  • 16. Glomus tumour Histology: ▪ It shows masses and sheets of epithelial cells ▪ Large nuclei ▪ Granular cytoplasm ▪ Abundance of thin wall blood sinusoids with no contractile muscle coat
  • 17. Glomus tumour Histology: • Chief cells - catecholemine secreting cells • Sustentacular cells - supporting cells • Surrounded by fibromusclar stroma/ vessels • The pattern these cells arranged are called as Zallballen
  • 20. Glomus tumour Symptoms: Ear polyp • Profuse ear bleeding • Otorrhea • Dizziness or vertigo
  • 21. Glomus tumour Symptoms: 9th - 12th cranial nerve palsies (late features) • Dysarthria, Dysphasia, Hoarseness of voice, Paralysis of soft palate, pharynx • Vocal cord involved • Weakness of trapezius, sternocleidomastoid muscle • Atrophy of tongue
  • 22. Glomus tumour Symptoms: Catecholemine features • Headache • Sweating • Palpitations • Hypertension • Anxiety
  • 23. Glomus tumour Symptoms: Rule of 10s. • 10% of the tumours are familial • 10% multicentric (occurring in more than one site) • Up to 10% functional, i.e. they secrete catecholamines.
  • 24. Glomus tumour Otoscopic stages: 1. Hyper vascular stage 2. Tympanic stage 3. Polypoidal stage/ hemorrhagic stage
  • 25. SIGNS
  • 28. Glomus tumour Signs: Pulsation sign (Browns sign) - Siegel’s speculum is used here.
  • 29. Glomus tumour Signs: Aquino sign : Cessation of pulsations with compression of the ipsilateral carotid artery
  • 30. Glomus tumour 1. Fisch classification 2. Lundgrens classification 3. Bickers and Howell classification 4. Jacksons and glascock classification
  • 31. Glomus tumour Fisch classification Type A - tumour located in the middle ear Type B - tumour located in the tympanomastoid area with no involvement of infralabyrinthine compartment Type C - tumour originates in the dome of the jugular bulb destroying the overlying bone. Type D - De1 – extradural, extension <2cm De2 - extradural, extension >2cm Di1 - intradural, extension <2cm Di2 - intradural, extension >2cm Di3 – intradural, unresectable
  • 32. Glomus tumour Jacksons and glascock classification Tympanicum Type I - Tumour involves only promontory Type II - Tumour involves middle ear space Type III - Tumour involves the middle ear mastoid Type IV - Tumour involves middle ear, mastoid and external auditory canal
  • 33. Glomus tumour Jacksons and glascock classification Jugulare Type I - Tumour involves jugular bulb, middle ear and mastoid Type II - Tumour extends beneath the internal acoustic meatus Type III - Tumour involves the petrous apex Type IV - Tumour involves the infratemporal fossa
  • 34. Glomus tumour Spread of tumour: • The tumour always prefers the pathway that offers minimal resistance. • Air cell tracts, vascular channels, naturally occurring fissures and foramina. • The pneumatized air cells tracts of the temporal bone are the most important route of spread
  • 35. Glomus tumour Differential diagnosis : 1. Otitis media 2. Idiopathic haemotympanum 3. Primary brain tumour 4. Cholesterol granuloma 5. Aberrant intrapetrous internal carotid artery 6. Aneurysm 7. Arteriovenous malformation 8. Prominent jugular bulb 9. Persistent stapedial artery
  • 37. References 1. Scott-Brown's Otorhinolaryngology and Head and Neck Surgery, Eighth Edition 2. Glasscock-Shambaugh Surgery of the Ear 3. Otolaryngologic Clinics of North America - Journal - Elsevier