Download Lateral Temporal Bone Tumors - Iowa Head and Neck Protocols

Lateral Temporal Bone Tumors
(nothing good grows in there)
Sarah Mowry
March 28,2007
Lecture Goals
• Temporal bone anatomy
• Benign tumors
– Glomus
– Others
• Malignant tumors
– SCCA and other primary tumors
– Metastatic tumors
• Surgical discussion
COCHLIA
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What are the three most common neoplasms of the auricle?
What are the four most common neoplasms of the external auditory
canal (EAC)and temporal bone?
Review the anatomy of the EAC and how this plays a role in the
spread of tumors.
Discuss the staging of temporal bone carcinoma and survival rates
using this system. Ann Otol Rhinol Laryngol 1990;99:714 and
Otolaryngol Head Neck Surg 1989;101:330.
What is the most common true neoplasm of the middle ear? Review
the pathophysiology, presentation and work-up.
Discuss the classification and management of the above neoplasm.
What are the surgical approaches for resection of this neoplasm?
What other tumors affect the middle ear?
What tumors commonly metastasize to the temporal bone?
COCHLIA
• What are the known complications of temporal bone and lateral
skull base surgery? Who should get a pre-op balloon occlusion
test?
• Discuss the role of radiation therapy in the treatment of glomus
tumors.
• What are jugular foramen tumors? What is jugular foramen
syndrome?
• Discuss the classification of glomus tumors (Fisch vs.
Glasscock)
• What are the surgical approaches to lateral skull base and what
are the indications for each (Fisch)?
• Discuss the pre-operative evaluation of skull base tumors with
suspected ICA involvement.
• Pneumocephalus-etiology and management of this post-op
Brief Anatomy Review
Surgical Anatomy
encompasses the
mastoidectomy (CWD), facial
recess approach (may be
extended), and infratemporal
fossa approaches. Malignant
tumors require wider resections
such as LTBR and TTBR.
Important Considerations
•Vectors of spread
•Cartilaginous EAC poor
barrier, fissures of Santorini
allow spread anteriorly into
glenoid and parotid
•TM
•Eustachian tube
•Oval and round windows
may be breached although
bone of otic capsule is very
resistant
•Mastoid air cells – tumor
super highway
Benign Tumors
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Glomus/paraganglioma
Neural tumors – neuromas/schwannoma
Mesenchymal tumors
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Bone/cartilage tumors
Lipoma
Hemangioma
Teratoma
Others
Adenomas
– Cerumenous adenoma and other epidermal appendage lesions
– PA
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Endolymphatic Sac tumors
Ectopic tissues
Granulomas and Dystrophies
– Histiocytoma, Eosinophilic granuloma
– Fibrous dysplasia, Paget’s, OI
Glomus Tumors
• After Acoustic Neuroma, the
most common tumor of T bone
• F:M 5:1 most frequently in 5th6th decades but reported in all
age groups.
• Usually solitary
– AD with variable
penetrance in some groups
• These patients have
likelihood of having
other paraganglioma
Fun Glomus Tumor Facts
• Arise from the paraganglionic extraadrenal neuroendocrine system
• These paraganglionic cells are
associated with the sympathetic
system. They are accordingly
found associated with blood
vessels and cranial nerves
• Can be found throughout the H/N
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Jugulotympanic
Carotid
Subclavian
Laryngeal
Orbital
More Fun Glomus Tumor Facts
•These tumors can be stained for neurosecretory granules
•1-3% of these tumors are active. Blood levels 5x nml are needed to
produce symptomatic expression.
•Screening tests include serum & urine VMA and hydroxyindoleacetic acid
•10% of nonfamilial cases additional glomus/paraganglioma
tumors can be found
–Usually Jug/tymp assoc with carotid tumors
•4% may demonstrate malignant degeneration
–Must be regional or distant met in a location other than where
paragangliomas normally develop.
Pathology
• Histologically, there
are clusters of cells
with abundant
cytoplasm and round
to ovoid nuclei. The
cells are organized
around small blood
vessels.
Glomus Classification
Fisch
2 staging systems
•Fisch (same system for
Tympanicum and Jugulare)
•Glasscock-Jackson
(2 separate classification
systems)
•Type A – tumor limited to the middle ear
cleft
•Type B – tumor limited to the
tympanomastoid with no involvement of the
infralabrinthine compartments
•Type C – tumor in infralabyrinthine
compartment and into the petrous apex
•Type D1 – intracranial extension >2cm
•Type D2 – intracranial extension <2cm
Glomus Classification
• Glasscock-Jackson
– Tympanicum
• Type 1 mass limited to promontory
• Type 2 completely filling middle ear cleft
• Type 3 extending into the mastoid process
• Type 4 into mastoid, through TM, may extend anterior to ICA
– Jugulare
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Type 1 tumor involving jugular bulb, ME and mastoid process
Type 2 extending under IAC, may extend intracranially
Type 3 into petrous apex, may to intracranially
Type 4 beyond petrous apex into clivus or infratemporal fossa, may go
intracranially
Tympanicum GJ type 4 (right ear)
Diagnosis
• Most common symptom is pulsatile tinnitus,
followed by hearing loss and aural fullness.
• Multiple cranial nerve palsies are common,
including
– Hoarseness, tongue weakness, vertigo, facial weakness,
dysphagia
• Unless you can see around the mass, you cannot
tell the difference b/t Tympanicum and Jugulare.
Diagnosis
• History and physical
• Audio and serum/blood screening
• Radiology
– CT and MRI complimentary
– Formal Angiography
– Embolization
• Biopsy is contraindicated due to brisk bleeding.
Should do an exploratory mastoidectomy.
Treatment
• Surgery
– Tympanicum – unless G-J type 1/Fisch A,
requires transmastoid approach with extended
facial recess approach to the hypotympanum.
• If the entire lesion is visible thru TM, transcanal
resection is feasible
– Jugulare
• G-J type 1 or 2 – Short mobilization technique
• G-J type 3-4 – Infratemporal fossa approach
Fisch ITF Approaches
• Type A – radical mastoidectomy, anterior
transposition of the FN, exploration of the
posterior IFT and cervical dissection to the JB,
petrous carotid and posterior ITF
• Type B – explores petrous apex, clivus and
superior ITF
• Type C – exposure of the NP, peritubal space,
rostral clivus, parasellar area, pterygopalatine
fossa and anterior superior ITF
Type A
• Closure of the EAC, removal of the EAC
skin, TM, malleus.
• Upper neck dissection for control of vessels
and nerves, preserve as much of Great
Auric as possible for nerve grafting.
• Ligate the occipital and ascending
pharyngeal as they are major supply to the
tumor
Type A
• FN is dissected from
geniculate to foramen
• The Eustachian tube
is obliterated with
muscle
• Radical
mastoidectomy is
completed
Type A
• The sigmoid is
occluded
– Mastoid emissary
vein should be
preserved
– May be suture ligated
or packed off
• Mastoid tip and
styloid process are
removed
• The mandible is
disarticulated
anteriorly
Type A
• IJ is ligated and dissection carried along the
ICA
• The ligated sigmoid is removed with its
lateral bony covering.
• Intracranial extension requires a posterior
fossa crani
– Resection may be staged if intracranial portion
is >2cm.
Type A
• Wound closure
– Fascia for dural defects
– Abdominal fat to fill space with a temporalis
rotational flap
• Alternatively a free tissue transfer is also
appropriate
– 3 layer skin closure and big ‘ole pressure
dressing to help minimize CSF leakage
Type B
• Also starts with EAC closure
and radical mastoid but FN left
in situ
– FN transposition not
necessary
• Limits of operation are middle
cranial fossa floor, condyle and
temporalis muscle
• Middle meningeal artery and V3
are sacrificed
• The ICA can be transposed to
improve access to petrous bone
and clivus
Type C
• Anterior extension
of type B
– Permits access
anterior to foramen
lacerum to the
posterior wall of
the maxillary sinus
Surgical Complications
• Glasscock’s group reported on 133 patients who
underwent surgery; most had jugulare tumors.
• Cranial nerve injury during jugular removal
– X, IX and XII are most commonly damaged
• CSF leak (10%), aspiration (5%), ear infection
(4%), death, meningitis, and stroke were the other
common major complications.
• 80% of facial nerves were preserved. The others
had the nerve reconstructed.
Other Benign tumors
• Glandular tumors (primary adenomatous
tumors) – rare. May arise from mucosal
glands in ME. Present as mass behind TM
with extensive bony erosion on CT. Tx is
surgery
• Carcinoid tumors – very rare. Surgery is
curative
Other Benign tumors
• Endolymphatic Sac tumors
– Associated with von Hippel
Lindau disease
• AD with retinal and cerebellar
angiomatosis.
– They are papillary in nature
and very vascular.
– Posterior fossa involvement is
common.
– Osteolytic on CT with
calcifications.
– Present with HL, tinnitus,
vertigo and facial paralysis.
Still more benign lesions
• Cholesteatoma of petrous apex – very rare.
Treatment is matrix removal, FN
preservation and avoiding a CSF leak.
• Cholesterol granuloma – inflammatory
reaction to cholesterol crystals.
– Expansion results in HL, disequilibrium
dysfunction of V, VI and VII.
Some more benign lesions
• Ectopic tissues – aka Choristoma - salivary
tissue in the middle ear. Typically
associated with adherence to dehiscent FN.
• Granulomas and Dystrophies
– Eosinophilic granuloma
– Fibrous dysplasia, Paget’s, OI
Malignant tumors of the T bone
• Pinna
• EAC
• ME
• And beyond
Tumors of the Pinna
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Epidermal tumors
• SCCA – 45% of pinna
tumors,
– mets to parotid, JD,
posterior cervical.
Happen in about 15% of
cases
– Tx is surgery or
radiation
• BCCA
• Melanoma
• Sebaceous carcinoma
– Tx is surgery,
considered
radioresistant
Pinna tumors
– Merkel cell carcinoma
• Neuroendocrine Ca
of the skin. High
rates of recurrence,
regional and distal
metastases
EAC Tumors
• Benign
– Nevi, Osteomas, exostosis, hemangioma,
cerumen gland adenoma
– Cerumen gland adenoma is a non-ulcerated skin covered
mass in EAC.
» Male predominance 3:1
» Ill defined capsule and may erode into bone
» Requires WLE with skin graft of EAC
EAC Tumors
• Malignant
– SCCA – most common
• Bloody otorrhea and otalgia are most
common S/S
• Lateral lesions at the cartilaginous canal
can spread thru fissures of Santorini
into the preauricular area
• May be assoc with chronic
inflammation of OE or cholesteatoma
• Tx options
– En bloc resection of EAC, TM, parotid
PRN, etc +/- XRT
EAC Tumors
• BCCA – less common than on surface skin,
similar S/S as SCCA. Tx is WLE.
• Adenoid Cystic CA – most common
glandular CA of the EAC. Deep seated
sharp otalgia. Predilection for perineural
invasion.
– Tx is WLE with post op XRT
Adenoid cystic CA
Cerumen gland adenoCA
• Counterpart of
cerumen gland
adenoma
– Otalgia, bloody
otorrhea, ulceration
– Same histo but with
increased mitoses.
– Tx is WLE with post
op XRT
Deep Temporal Bone Tumors
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SCCA
Sarcoma
Glandular tumors
Lymphovascular tumors
Metastatic tumors
SCCA
• Most common
• No sex prevalence
• Most patients have H/O chronic
inflammation of some kind
• S/S are otorrhea, HL and deep seated
otalgia. 40% have a ME mass.
• Direct labyrinthine invasion is rare due to
otic capsule
SCCA
• Facial nerve involvement = advanced Dz
– CN VII paresis = 30-50% recurrence rate
– Paralysis = >60% recurrence
• Involvement of other CN = “dismal
prognosis”
• CT and MRI are complimentary
• Consider angio with embo if surgery is
feasible
TNM staging
• T stage
– T1- tumor limited to the EAC without bony erosion or
soft tissue extension
– T2 - tumor with limited EAC bony erosion (not full
thickness) or <0.5cm soft tissue involvement
– T3 - tumor eroding the osseous EAC with <0.5 cm soft
tissue involvement or tumor involving middle ear or
mastoid or presenting with facial paralysis
– T4 - tumor eroding the cochlea, petrous apex, medial
wall of middle ear, carotid canal, jugular foramen, dura,
>0.5 cm of soft tissue involvement
Staging
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N0
N1 – “portends a poor prognosis”
M0 vs. M1
Stage 1 – T1N0M0
Stage 2 – T2N0M0
Stage 3 – T3N0 or T1N1
Stage 4 – T4, >T2N1 or M1
• SCCA
Treatment
– LTBR
– STBR
– TTBR
Carotid Management
• T bone resection requires carotid control as
vessel passes thru medial to the Eustachian
tube before entering the cavernous sinus
• CT will show if the tumor is near the carotid
canal.
• 4 vessel angiography will show if vessel is
involved with tumor
Carotid BTO
• Balloon occlusion testing with Xenon/CT
– Investigate the collateral blood flow to
ipsilateral hemisphere
• 80% will tolerate ICA sacrifice
• 10% will not – necessitates prior bypass grafting
(ECA to MCA bypass) before T bone resection
• 10% grey zone – intraoperative or preoperative
revascularization
Lateral T Bone Resection
• En bloc removal of the entire EAC and TM
• Utilizes the extended facial recess approach
• May also include parotidectomy, ND and
mandibular condylectomy
• Involves resection of concha, may include
variable parts of the pinna and tragus PRN
LTBR
• Closure of the EAC
•Complete
mastoidectomy
•Extended facial recess
(sacrifice the chorda)
•Disarticulate the IS
joint
•Fracture the anterior
EAC just lateral to the
Eustachian tube with
osteotome
•Watch out for ICA!
Subtotal T Bone Resection
• Used with CA has penetrated into the ME space or
mastoid cavities
• Requires resection of the otic capsule
• Can be extended toward the ITF, jugular bulb or
dura as prescribed by tumor extent
• Should include monitoring of CN 7, 9, 10, 11
• If possible spare CN 7 by complete mobilization
from geniculate to foramen and transpose the
nerve posteriorly.
STBR
• The tegmen and posterior fossa plates are thinned
and then removed.
• A translab drill out of the IAC and jug bulb then
done
– Allows further mobilization of the FN from the porus if
needed.
– The transected end of CN VIII should be sent for frozen
section
• Entire tympanic ring drilled out but leaving
periostium over ICA and lower CNs.
STBR
• Neck dissection preformed
for vascular control of IJ
and ICA
• Involvement of jugular
foramen necessitates IJ
sacrifice and ligation of the
sigmoid
– Avoid injury to vein of
Labbe – drainage of the
temporal lobe and can result
in venous infarction of
temporal lobe. Bad.
STBR
• Dural extension can be
resected with help of
neurosurgeon to close
the dural defect.
• Extension into the ITF
accomplished by
including a Fisch A ITF
approach
Total Temporal Bone Resection
• Used if tumor involves the petrous apex
• Mandates proximal and DISTAL control of
the ICA
– Distal control accomplished with middle cranial
fossa approach
• Requires division of CN 7, 8, 9, 10 and 11
– Done through a suboccipital crani
• The ICA is completely mobilized or resected if involved
with tumor
• Osteotomy completed posterior to the foramen ovale
Outcomes
• Tumors limited to the EAC have 50-80% cure rate
after LTBR
• Tumor extending beyond the ME 0-15% survival
>2yrs
• Survival increases with dual modality therapy
• University of Pittsburg staging system
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Increasing T stage is inversely proportional to survival
T1 and T2 have reported 100% 2 yr survival
T3 lesions have 2 yr of 56%
2 yr survival of T4 tumors at 17%
Rhabdomyosarcoma
• Most common sarcoma of T bone.
• Almost exclusively in children. Most common
histologic type is embryonal.
• 20% are metastatic at presentation
• Thought to arise from ME mesenchymal cells but
also from the Eustachian tube.
• Most common present with otorrhea and HL.
– Advanced lesions develop FN paralysis, HA or
Abducen’s palsy (petrous apex involvement)
Rhabdomyosarcoma
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Staging follows guidelines for other
rhabdos
– Grade 1 – localized disease
totally resected
– Grade 2 – Residual microscopic
disease
– Grade 3 – Gross residual
disease
– Grade 4 – Distant mets present
Complete resection is difficult
– Triple modality therapy is
mainstay
• 3 agent chemo + XRT
Other reported sarcomas
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Ewing’s
Osteogenic sarcoma
Chondrosarcoma
Kaposi’s
– Much higher prevalence in AIDS (AIDS
defining illness)
– Usually involves the EAC and ME
Other Tumors
• ACC – usually in EAC, very rare primary in
ME
• AdenoCA
– Low grade papillary cystadenoCA or high
grade undifferentiated adenoCA
– LG 15% mortality while HG have “poor
prognosis”
– Treatment is surgery with post op XRT
Lymphovascular tumors
• Plasmacytoma
• Histiocytosis X – now know as eosinophilic
granuloma, Hand-Schuller-Christian,
Letterer-Siwe
• Malignant glomus tumors
• Hemangiopericytoma – primary T bone
very rare. Surgery + XRT
– 15-20% rate of mets to lung and bone
Mets to T bone
• Unusual site of metastasis
• Most common culprits
– Breast, lung and kidney
– Prostate is also common
• Spread by various routes
– hematogenous, direct extension, meningeal
carcinomatosis, or leukemic/lymphomatous
spread
Lymphoma/Leukemia of T Bone
• Primary disease is rare (duh)
• Lymphomas may present with HL and FN
paralysis accompanied by mass and otorrhea
• Leukemics present by tumor infiltration or
hemorrhage
– Petrous marrow acts as filter for tumor cells.
– Involvement of labyrinth results in SSNHL and
vestibular symptoms
References
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RF Canalis and P Lambert The Ear Lipencott-Williams, Philadelphia, 2000.
Cumming’s Otolaryngology Head and Neck Surgery, Vol 4. Elsevier-Mosby,
Philadelphia, 2005.
EN Myers Operative Otolaryngology Head and Neck Surgery, Vol 2.
Saunders, Philadelphia, 1997.
Strocker, A Head and Neck Pathology, 2006