ME Tumors Flashcards
most common benign soft tissue tumor of the ME (rarely malignant)
paragangliomas
can happen anywhere in body, including under nail beds
paragangliomas
Second most common benign tumor of the temporal bone
paragangliomas
Arising along the course of the Jacobson nerve primarily in the tympanic cavity
glomus tympanicum
Arise from the dome of the internal jugular vein bulb and involve jugular foramen and related structures
glomus jugulare
slow progressive growth spreading through path of least resistance such as temporal bone air cells and ET
Diagnosis can occur ~ 4 to 10 years after initial occurrence
They can grow to large proportions (typically, glomus jugulare)
glomus tympanicum & jugulare
describe paraganglioma (glomus tumor)
typically unilateral, reddish-purple, highly vascular, and lobulated
Glomus tumors arise from paraganglia cells
They were incorrectly called glomus tumors originally because it was believed that they originated from true glomus (arteriovenous) complexes
small groups of flat, oval, vascular paraganglionic bodies connected with the ganglia of the sympathetic nervous system
paraganglia cells
exist throughout the temporal bone including the dome of the internal jugular vein, promontory of the ME, Arnold and Jacobson’s nerves
paraganglia cells
why were they incorrectly called glomus tumors originally
it was believed that they originated from true glomus (arteriovenous) complexes
But the name glomus tumor persists
jacobsons nerve is one of the areas it can come from
glomus tympanicum
heredity of glomus tumors
Sporadic or AD inheritance with 100% penetrance
Mutation of gene SDHD mapped to 11q23 may be involved in these tumors
Genomic imprinting may be involved
Affected individuals inherit the disease from their father but expression of the phenotype may not be observed in off-springs of affected females until transmission through a male carrier
glomus tumors
incidence of paraganglioma
Patients usually present after the 5th decade of life
More common in females (males may be carriers if genetic)
Even with modern genetic testing, the majority of paragangliomas appear to be sporadic
However, approximately one-third to one-half are associated with an inherited syndrome including some cases of NF1
So far 10 genes have been associated with paragangliomas with or without pheochromocytoma
Typically arises from the promontory of the ME
glmous tympanicum
signs of glomus tympanicum
Pulsatile tinnitus because of the tumor vascularity is often the first presenting symptom
Tympanic membrane may appear red due to increased vasculature of the ME cavity
Lateral growth through the TM can mimic a bleeding polyp
Growth of tumor can inhibit ossicular mobility resulting in a conductive hearing loss
Medial growth towards the inner ear can cause SNHL, facial nerve dysfunction, and/or vertigo
treatment of glomus tympanicum
Surgery: Complete tumor removal is seen in > 90% of cases
arise from the dome of the internal jugular vein or proximal portion of Arnold’s or Jacobson’s nerve
More common and extensive than glomus tympanicum because of the space available to grow
glomus jugulare
symptoms of glomus jugulare
Hearing loss
Otalgia
Aural fullness
Vertigo, if involvement of vestibular division of VIII N
Hoarseness and dysphagia due to involvement of the CN IX, X, and XI because of proximity to site of origin of glomus jugulare
Involvement of XII (hypoglossal) nerve indicates more extensive disease
otoscoppy of glomus jugulare
red mass filling the ME cavity or lower portion of the ME cavity may be visible
pure tone results of glomus jugulare
conductive hearing loss may be present depending on the size of the tumor and involvement of ossicles
Sometimes a mixed hearing loss may occur if neural involvement has occurred
immittance
Jerger type AS tymps
Jerger type B
Tympanogram also will show jagged edges which will correspond to the patient’s pulse
Pulsating tympanogram, suspect glomus tumor
treatment of jugulare
surgery or radiation
