CAROTID BODY TUMOR, HEMANGIOMA, LYMPHANGIOMA ENT | FINALS (2nd Sem)

OUTLINE o Three phases of growth, which occur independent

I. VASCULAR ANOMALIES of the growth of the patient:
A. Hemangioma  (1) Proliferative—first 9 to 12 months of life
B. Vascular Malformations  Can have two periods of rapid
proliferation
I. VASCULAR ANOMALIES (a) One to 3 months, usually 80% of
growth occurs
A. Hemangioma (b) Five to 6 months
 (2) Involution—variable course of
regression over many years
Presentation and Natural History
 Graying of lesion, “herald spot,” is
 True vascular tumor. usually the first indication of
 Two types: congenital and infantile. involution.
 Congenital hemangioma is the rare tumor present at birth.  (3) Involuted—almost all involuted by 9
o Two types: rapidly involuting and noninvoluting years of age; classic teaching is 50%
o Glucose transporter 1 (GLUT1) negative involuted by 5 years, 70% by 7 years and
90% by 9 years
o Three distributions:
 (1) Focal—classic solitary mass
 (2) Multifocal—multiple masses
 When more than five cutaneous
masses present, must rule out liver
and gastrointestinal (GI) involvement
with abdominal ultrasound (US)
 (3) Segmental distribution—multiple
cervicofacial subunits or large areas of upper
aerodigestive tract
 Usually follows trigeminal territory
 Infantile hemangioma is the most common vascular (V1, 2, and/or 3).
anomaly in head and neck.  Two-thirds of children with V3
o Presents after birth, usually as a distinct, bright red (“beard”) distribution will have
mass. synchronous subglottic hemangioma
o Firm and rubbery (unlike compressible vascular —must evaluate airway.
malformations).  PHACES syndrome—anomalies of:
o Diagnosis typically made clinically. (a) Posterior cranial fossa
(b) Segmental Hemangiomas
o GLUT1 positive; stain used to confirm diagnosis if in
question.
(c) Intracranial or cervical Arteries
(d) Cardiac (heart and aorta)
(e) Eye
(f) Sternum

o Three anatomic locations:

 (1) Superficial: bright red, cobblestoned,
cutaneous, or mucosal mass
 (2) Deep: no cutaneous/mucosal component,
bluish hue of overlying skin
 (3) Compound: superficial and deep
components

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 Subglottic hemangioma
o Must be ruled out as a source of stridor in any infant
with a cutaneous hemangioma
o Typically, a bluish or reddish, compressible mass in
the posterior left subglottis
 Parotid hemangioma
o Most common parotid tumor of infancy
o Deep, firm mass within substance of gland
Diagnosis and Management
 Diagnosis usually made clinically and biopsy not
necessary.
 Ultrasound can be helpful in differentiating from
arteriovenous malformation (AVM) if in question.
 Subglottic hemangiomas may show asymmetric
narrowing of subglottis on neck x-ray and typically require
rigid endoscopy for full assessment.
 Classically, observation is advised in most cases of
cutaneous hemangiomas although trend is for earlier
intervention prior to rapid growth phases to prevent scarring
and cosmetic deformities.
 Intervention imperative for:
o Symptomatic—for example, bleeding, ulcerated,
massive and resulting in chronic heart failure (CHF)
or Kasabach-Merritt phenomenon (consumptive
coagulopathy)
o Critical anatomic locations—eyelids, lips, ears,
airway
 Inconspicuous hemangiomas can be managed with
observation and reassurance.
 Medical management:
o Intralesional steroid injections.
o Systemic steroids or chemotherapy (alpha-
interferon, vincristine) have significant side effects
but are used for symptomatic or critically located
tumors.
o Most recently propranolol has shown success in
causing regression of hemangiomas and may
become a first-line therapy, pending further
experience and data from specialized centers.
Prelims (1st Sem)
PATHOLOGY
 Laser therapy:
o Flash lamp pulsed-dye laser (585 nm) effective for
superficial lesions (no deep component).
o Nd:YAG has deeper penetration and can be used
for superficial and deep lesions.
o Laser therapy also used to manage ulcerated
lesions, promote resurfacing, and for postregression
telangiectasia.
o Scar may still require surgical excision after
regression of lesion.
 Surgical therapy:
o Used for localized lesions or postregression
remnants
 Subglottic hemangiomas—no consensus on
management:
o Rarely observation for nonobstructing lesions.
o Medical therapy
(1) Steroid injections and dilation for small lesions
(2) Systemic or combined therapy for larger lesions
(3) Beta blockers (propranolol) may become first-line
therapy
o Endoscopic CO2 or KTP laser ablation or excision
effective, but should be avoided in circumferential
lesions because of high risk of subglottic stenosis.
o Open removal with airway reconstruction if
necessary.
o Regardless of approach, preservation of mucosa is
key to prevent subglottic stenosis.
o Tracheotomy usually is not required and should be
avoided unless absolutely necessary; tracheotomy
is detrimental during key speech and language
milestones of childhood.
B. Vascular Malformations
Presentation and Natural History
 Not considered as tumors
 Tend to grow with the patient
 Categorized by blood flow: slow flow and fast flow
 Slow flow: capillary, venous, and lymphatic
o Capillary (venular)
 Telangiectasias, nevus flammeus (port wine
stain), spider angioma
 Sturge-Weber syndrome (port wine stain of
face with ipsilateral intracranial
angiomas/AVMs)
o Venous
 Usually diagnosed early in life with finding of
a soft, compressible mass.
 Continue to grow with patient throughout life,
by both expansion and proliferation.
 Incidence is 1:10,000, usually sporadic.
 Superficial lesions have bluish coloration to
overlying skin or mucosa.
 Deep lesions associated with muscle groups.
 Can present later in life with continued
growth or pain and rapid expansion
secondary to clot formation.
o Lymphatic
 Usually diagnosed in childhood when noticed
at birth or when expand secondary to local
infection (eg, upper respiratory infection [URI]
or otitis media).
 Noncompressible lesions, usually
deep with normal overlying skin.
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 Mucosal lesions often have overlying

vesicles.
 Most common site in body is the
cervicofacial region; diffuse lesions can
involve the upper aerodigestive tract.
 Large upper aerodigestive tract lesions may
be diagnosed on prenatal ultrasound and
require emergency airway management or ex
utero intrapartum treatment (EXIT) procedure
during delivery.
 Main classification: macrocystic and
microcystic
 Macrocystic is easier to treat and
have a better prognosis.
 Some small, posterior triangle,
Common types of arteriovenous fistulas. Left, Radiocephalic
macrocystic lymphatic malformations
fistula. Middle, Brachiocephalic fistula. Right, Brachiobasilic
regress spontaneously within the first
year of life. transposition.
 Orbital lymphatic malformations can have
associated intracranial vascular anomalies Diagnosis and Management
that must be ruled out.  Slow-flow vascular malformations
 Fast flow: arteriovenous malformations (AVMs) AVMs and o Ultrasound is most useful for diagnosis.
arteriovenous fistulas (AVFs)  Venous malformations will have phleboliths
o AVMs and slow blood flow on Doppler.
 Form from a nidus of abnormal capillary  Determination between macrocystic and
beds. microcystic can be made for lymphatic
malformations.
 Lesions usually present as a vascular blush
o MRI is helpful for delineating extent of lesions and
that expands as the patient grows. relationship to surrounding structures.
 (Lesions can be pulsatile to palpation and o Conservative management with elevation of head
have a bruit.
of bed is used to discourage swelling and
 (Advanced lesions can have local tissue and
expansion; warm compresses and nonsteroidal anti-
bone destruction, bleeding, and pain.
inflammatory drugs (NSAIDs) are used for
 Often misdiagnosed as hemangioma in
thrombosis of venous malformations.
infancy until regression does not occur, with
o Sclerotherapy to stimulate inflammation and
considerable additional morbidity.
 Can present much later in life (eg, fourth-fifth fibrosis, ultimately decreasing expansion and
shrinking lesion.
decade), with evidence of a posttraumatic
etiology.  Most are delivered via radiologic guidance.
 Procedure of choice for macrocystic
 Most diagnosed in infancy or childhood and
grow intermittently secondary to lymphatic malformations.
 Ethanol, sodium tetradecyl sulfate,
environmental stimuli.
 Recent evidence of hormonal receptors in bleomycin, glues, and polymers.
 OK-432, inactivated Streptococcus
AVMs; indeed, hormonal changes such as
puberty and pregnancy appear to stimulate pyogenese, is the subject of several clinical
trials and has been shown to be 80% to 90%
growth.
effective for macrocystic lymphatic
malformations but less so for microcystic
lymphatic malformations.
 Must ensure sclerosant if not systemically
absorbed, especially for highdrainage venous
malformations.
 Complications include overlying skin
necrosis, scarring, and neuropathy.

o Laser therapy
 KTP and Nd:YAG lasers are first-line therapy
for skin and mucosal venous malformations.
 Pulsed-dye lasers are also useful for capillary
o AVFs malformations.
 Posttraumatic  Interstitial Nd:YAG can be used for deeper
 Defined by single arteriovenous connection, venous malformations.
rather than a nidus of multiple connections o Surgery
 Can be challenging, but best option for
“cure.”
 More effective than sclerotherapy for
microcystic lymphatic malformations.

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 Preoperative sclerotherapy to decrease

intraoperative blood loss should be
considered for venous malformations.
 Must ensure complete excision to prevent
recurrence.
 Postoperative scarring and fibrosis can be
significant, especially for lymphatic
malformations.
 AVMs
o Doppler US will demonstrate rapid blood flow, and
MRI will usually show flow voids.
o CT angiogram is useful for operative planning.
o Treatment is difficult and multidisciplinary.
 Embolization of nidus with alcohol or
polymers.
 Surgical resection following embolization.
 Small, easily resectable lesions
 Life-threatening, destructive lesions;
often requires massive resection
with adjacent or free tissue transfer
 Recurrence is common.
 AVFs
o Angiography or CT angiography demonstrates
single arteriovenous connection.
o Embolization only for deep, inconspicuous lesions.
o Surgical resection in highly visible lesions (eg, lip) to
eliminate residual scar and return of normal function.
 Consider preoperative embolization to
decrease intraoperative blood loss.
 If no embolization, it is useful to identify and
clip feeding artery early in resection. References
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