TITLE:
Vascular Tumors of the Head and Neck
SOURCE: Grand Rounds Presentation, UTMB, Dept. of Otolaryngology
DATE: April 11, 2001
RESIDENT PHYSICIAN: Russell D. Briggs, MD
FACULTY PHYSICIAN: Anna Pou, MD
SERIES EDITOR: Francis B. Quinn, Jr., MD
"This
material was prepared by resident physicians in partial fulfillment of
educational requirements established for the Postgraduate Training Program of
the UTMB Department of Otolaryngology/Head and Neck Surgery and was not
intended for clinical use in its present form. It was prepared for the purpose
of stimulating group discussion in a conference setting. No warranties, either
express or implied, are made with respect to its accuracy, completeness, or
timeliness. The material does not necessarily reflect the current or past
opinions of members of the UTMB faculty and should not be used for purposes of
diagnosis or treatment without consulting appropriate literature sources and
informed professional opinion."
INTRODUCTION
Vascular tumors of the head and neck
comprise a group of neoplasms that share a common origin with components of the
vascular system. While these tumors do
have an anatomic origin with the vascular system, they have vastly different
presentations, biologic characteristics, and management options between them. A number of authors have classified vascular
lesions based on descriptive, pathologic, embryological, or biological
characteristics, however no classification scheme has been universally
accepted. Vascular tumors of the head
and neck can be divided into benign and malignant varieties which allows for
comparisons between tumor types. This
division of tumor types will be used as the classification scheme for the
purposes of this text.
BENIGN TUMORS
Vascular
Birthmarks
Nomenclature is the major obstacle
in the understanding and management of vascular birthmarks. Much of the confusion stems from early
textbook classifications that offered an array of histologic and descriptive
words to document these lesions. Terms
describing hemangiomas have included “capillary,” “juvenile,” “strawberry,” and
“cavernous,” among others. These terms
have permeated the medical literature and have lead to a great deal of
confusion. Mulliken and Glowacki have
revised the classification system of vascular birthmarks into hemangiomas and
vascular malformations. According to
this system, vascular birthmarks are differentiated into these two categories
according to their biologic, cellular, and clinical characteristics.
Hemangiomas
Hemangiomas are the most common
tumor of infancy occurring in 8-12% of infants. A female predominance has been reported to range from 3:1 to
5:1. Most of these lesions are small,
solitary, and uncomplicated, however 60% do arise on the face presenting a
cosmetic concern to the parents.
Clinical presentation is the most
important factor in making the proper diagnosis of a hemangioma. A hemangioma is usually not seen at birth,
but subsequently arises within the first 8 weeks of life. Many hemangiomas are appreciated at birth as
a cutaneous precursor lesion, which may vary from a faint red macule to a pale
nevus that may mimic a bruise. Only
rarely does a fully developed hemangioma present at birth, and in these rare
instances, usually do not proliferate further and begin to regress quite
rapidly. For the majority of
hemangiomas, however, the natural history consists of a proliferative phase
that usually lasts 3 to 9 months, followed by a slow regression phase. Most hemangiomas remain as
well-circumscribed lesions 0.5-5 cm in diameter and involve the superficial
layer of skin, thus being appropriately called superficial hemangiomas. Some, however, may proliferate into the
lower dermis and subcutaneous tissue with little involvement of the superficial
dermis. These deeper lesions, once labeled
“cavernous” hemangiomas are more appropriately designated as deep hemangiomas. The involution phase of hemangiomas
typically progresses at a rate of 10% per year, so that 50% have regressed by
five years. After complete regression,
normal skin is restored in 50% of patients while 10-38% have permanent changes
including scarring caused by ulceration, skin atrophy, puckering, and
fibrofatty residuum. However, of the
remaining 50% that do not involute by 5 years, 80% will leave a substantial
residual cosmetic deformity.
Complications from hemangiomas may
occur in 20% of all hemangiomas, but few are life threatening. Ulceration is the most common complication
occurring in less than 10% of patients, typically during the rapid
proliferation phase. Compromise of
function may occur with compression of vital structures such as those
hemangiomas that affect the upper eyelid which may impair vision , leading to
amblyopia or strabismus. Hemangiomas in
the oropharynx, larynx, or nasal passages can lead to respiratory or feeding
disturbances.
Hemangiomas occurring in the larynx
usually occur in the subglottis and are found mainly in the pediatric
population. These lesions present
classically with a previously healthy infant who suddenly develops biphasic
stridor during the first few months of life, many times misdiagnosed as croup. These lesions are usually located in the
posterolateral subglottis and are submucosal, soft, and compressible
masses. These lesions behave like other
hemangiomas with most regressing by age 5 years. For this reason, a conservative approach is often employed as
long as the airway is acceptable.
Approximately 50% of these hemangiomas are associated with cutaneous
counterparts, typically in the beard area of the face.
In certain cases such as deep
hemangiomas or hemangiomas detected early in neonatal life that have not
reached the progressive phase, radiographic investigation of such lesions may
be indicated. Ultrasound with Doppler
flow imaging is the most cost effective, however MRI imaging is the most informative
noninvasive study, safely providing the information of both CT and angiographic
data. MRI can accurately determine the
extent of the lesion and the finding of serpentine high-volume flow voids
surrounded by nonvascular soft tissue is characteristic of hemangiomas. One particular indication for further
radiographic study is for individuals with large facial hemangiomas, usually
unilateral, as these may be associated with Dandy-Walker syndrome or posterior
fossa malformations.
Indications for treatment have
historically been based on tissue destruction or disfigurement, severe
bleeding, obstruction of vital functions, high-output cardiac failure, or
platelet trapping with a coagulopathy.
Recently, many authors have advocated early treatment of facial
hemangiomas due to a better understanding of the diagnosis, histopathology, and
natural history of these vascular lesions.
In addition, these authors contend that advances in laser technology,
appropriate use of systemic steroids, safer pediatric anesthesia, and improved
surgical techniques have allowed for better cosmetic results and, in turn,
improved psychological well-being in these children.
A number of treatment options are
available for management of hemangiomas during the proliferative phase
including observation, systemic steroid therapy, intralesional steroid
therapy, pulse-dye laser therapy,
surgical debulking or excision, arterial embolization, alpha 2b-interferon,
radiation therapy or combinations of the above.
Most parents desire some method of
treatment for their child’s hemangioma, and they have difficulty accepting that
many lesions do best if they are left alone to involute naturally, leaving
either normal or slightly blemished skin.
As most will eventually regress, initial management generally consists
of observation. In these cases where
observation is chosen, serial photographs demonstrating the slow involution are
helpful. Regular visits with parental
reassurance at 3 to 6 month intervals are sufficient to document the involution
of the hemangioma.
Systemic steroid therapy was once
considered first-line therapy for complicated hemangiomas. Steroid use, if used cautiously, is
effective with minimal long term side effects.
This therapy arrests the growth of the lesion and therefore an immature
proliferating hemangioma is far more responsive to steroids than a stable or
involuting lesion. Systemic steroids
may be used in selected infants that 1) have a rapidly growing lesion that
seriously distorts facial features, 2) have a lesion causing recurrent
bleeding, ulceration, or infection, 3) have a lesion that interferes with
normal physiological function (breathing, hearing, vision, or eating). In general, 2 to 4 mg/kg/day of prednisone
are given orally in a single daily dose for as many as 6 weeks, followed by a
slow taper over 2-3 months. Results are
variable with a dramatic response in 30%, an equivocal response in 40%, and no
response in 30%. Side effects include
cushinoid effects, adrenal suppression, infections, and growth
suppression. The child must be closely
monitored during the treatment course for any evidence of steroid toxicity.
Intralesional steroid therapy has
been used predominately in cases of vision-threatening periorbital hemangiomas,
but is also used for cases involving globular tumors of the lips, nasal tip,
cheek, or ears. Some authors also
utilize intralesional injections for those lesions that rebound from prior
systemic steroid therapy and for deep proliferating lesions in patients not
candidates for systemic steroid therapy.
Typical doses have included 1-2 mL of a combination of beta-methasone
(6mg/mL) and triamcinolone (40 mg/mL) given in repeat injections as necessary
at 4-6 week intervals. This therapy has
the benefit of a rapid action typically within one week, however its success is
similar to that of systemic steroids.
Complications can be severe and include full thickness eyelid necrosis
and blindness.
Pulse-dye lasers have become
increasingly popular for the treatment of facial hemangiomas. These lasers, however are only useful for
superficial hemangiomas as the laser penetrates only 0.75-1.5 mm deep. It has been quite useful in cases with
ulceration as these typically are rapidly proliferating superficial lesions. The laser is relatively vessel specific and
thus has a low risk of scarring and is safe to use in young children. It is also quite useful for patients with
particular cosmetic defects after the involution phase of the hemangioma is
complete (residual telangiectasias).
Surgical therapy for hemangiomas has
historically been reserved as a last resort for treatment of complicated
lesions. Examples of such indications
include eyelid lesions, bulky lesions that will likely leave a sagging bag of
skin at involution, lesions involving the vermilion border, nasal tip, and eyebrow. Surgery can also be utilized for correction
of redundant skin after spontaneous resolution has occurred. CO2 laser excision of large subglottic
hemangiomas is useful is cases of severe obstruction and those cases that have
not responded to systemic steroids.
Arterial embolization is typically
reserved only for those alarming hemangiomas that are inoperable, hemorrhaging,
or have not responded to other methods.
Risks include thrombosis and backflow of material into undesired
vessels, unintentional embolization, and even blindness. Even with apparently effective treatment,
results are usually temporary.
Alpha 2b-interferon, radiation, and
cryotherapy have been used in the past but these are no longer recommended due
to serious complications.
Kasabach-Merritt syndrome is a
particular entity associated with large hemangiomas or diffuse hemangiomatosis
throughout the body. This disorder is
associated with severe thrombocytopenia and hemorrhage caused by localized
platelet trapping. The coagulopathy
associated with this syndrome may require administration of platelets, fresh
frozen plasma, and cryoprecipitate.
Lesions implicated in this syndrome are typically treated with one of
the above options, however, many lesions do not respond to medical therapy and
require surgical intervention to correct the coagulopathy.
Vascular Malformations
Compared with hemangiomas, vascular
malformations are developmental anomalies.
Thus, all cutaneous vascular malformations, by definition, are present
at birth. These lesions have no gender
predilection and are characterized by commensurate growth. These lesions do not have a proliferative
and involuting phase, rather, they persist throughout the life of the affected
person without treatment. These
malformations are categorized into their component vessels: capillary, venous,
arterial, lymphatic, or mixed.
Capillary malformations, commonly
referred to as “port-wine” stains, occur in approximately 1% of all
newborns. Diagnosis is usually
straightforward with most occurring on the face, especially in the distribution
of the trigeminal nerve. They are classically unilateral with respect to the
midline. Initially, capillary
malformations are pink, flat and sharply demarcated lesions. In older individuals, they often become more
nodular in appearance and a darker, purple color develops. Most of these lesions are isolated
anomalies, however some may signal underlying disorders that require additional
investigation. Sturge-Weber syndrome is
a sporadic disorder associated with a capillary malformation involving at least
the V1 distribution with associated ipsilateral leptomeningeal venous
malformations, atrophy and calcifications in the adjacent cerebral cortex,
seizures, visual field defects, and mental retardation. These patients may also develop retinal
detachment and either congenital or acquired glaucoma. Patients diagnosed with this condition
should have an early ophthalmologic and neurologic examination with MRI imaging
to detect the associated brain anomalies.
Treatment of capillary malformations
are varied. Cosmetic concealing makeup
are formulated to match the individual skin tones of patients. However, these are only helpful for flat
lesions and may take up to 20 minutes to apply. Similarly, tattooing of lesions has fallen out of favor due to a
poor cosmetic result secondary to scarring, a mask-like appearance, and poor
retention of the pigment. Surgical
excision is also losing favor due to the high risk of hypertrophic scarring and
permanent depigmentation in grafted areas.
Tissue expanders may provide role in moderate sized lesions allowing for
closures along facial unit borders. The
pulse-dye laser has become the most effective treatment for capillary
malformations although up to 50% of successfully treated lesions recur.
Venous malformations are low-flow
lesions that are deeply invasive and may worsen slowly through the course of
the patient’s life. As with other
vascular malformations, these are present at birth. Diagnosis is usually clinical as these usually compress easily
with pressure and swell when dependent or with increased venous pressure. Management of these lesions varies greatly
depending on the size, location, and clinical presentation. Asymptomatic lesions can be observed, however
when loss of function or cosmesis becomes unacceptable, treatment often entails
multiple procedures over several years, using a combination of sclerotherapy
and surgical excision.
Lymphatic malformations , also
termed cystic hygromas or lymphangiomas, are vascular tumors arising from
maldevelopment of the lymphatic system.
As with other vascular malformations, the lesions are usually slow
growing but sometimes expand rapidly with respiratory tract infections which
can threaten the airway in patients with head and neck lesions. Complete excision is the recognized primary
treatment modality, but this goal can be achieved in only one-third of cases if
vital and functionally important structures are not sacrificed. Definitive management is indicated when
vitals structures are endangered or when episodic hemorrhage ensues. Another indication for surgical management
is associated macroglossia which may lead to articulation, feeding, dental
occlusion, or airway problems.
Nonsurgical treatment with diathermy, radiation therapy, and most
sclerosing agents has met with only limited clinical success. A newer sclerosing agent, Picibanil (OK-432)
has shown promising preliminary results.
MRI can be used to establish the diagnosis and estimate the extent of the
lesion. It is also helpful for
following patients after treatment to detect recurrence.
Arteriovenous malformations are
high-flow malformations resulting from persistent abnormal direct arterial and
venous connections. While present at
birth (congenital type), many of these lesions are not recognized until late
childhood, even into adulthood.
Violaceous color of overlying skin, palpable thrills, and bruits should
suggest such lesions. Also
hyperhidrosis, hypertrichosis, and hyperthermia may be present in the
area. Although usually clinically
apparent, these lesions can be diagnosed with Doppler ultrasound exams or with
MR imaging with angio-sequences which can better define the anatomic extent. Angiography can establish a nidus for such
lesions but is usually reserved for therapeutic efforts. Embolization can be used as a therapeutic
modality, however many lesions recur.
Embolization with operative excision within 48 hours allows for
decreased bleeding complications and allows for improved elimination of the
lesion.
Angiofibromas
Nasopharyngeal angiofibromas are the
most common benign tumors of the nasopharynx yet represent only 0.5% of all
head and neck tumors. In the past, the
term juvenile nasopharyngeal angiofibroma was used, however, this term is
inappropriate because the neoplasms occur in older patients as well. It is a benign, yet highly vascular tumor
that occurs almost exclusively in adolescent boys. These lesions may occur at any time, but are most commonly
diagnosed between the ages of 14 and 25.
The usual presenting complaints are
painless, progressive nasal obstruction and recurrent epistaxis for no apparent
reason. Clinically, the lesion appears
as a firm, friable mass in the nasopharynx or nose. As the tumor enlarges, the tumor interferes with eustachian tube
function producing a conductive hearing loss.
Persistent, unilateral middle ear effusions in this patient population
should alert the physician to this possibility and the need for appropriate
nasopharyngeal examination. As the
tumor enlarges, more obvious signs may develop related to the direction of
tumor spread. These include orbital
findings such as proptosis or limitation of ocular movements, cranial nerve
deficits, sinusitis, noticeable cheek swelling, or meningitis. These tumors have a characteristic for
aggressive local growth and a tendency for base of skull and intracranial
invasion. It is believed that
nasopharyngeal angiofibromas arise in the fibrovascular stroma normally present
in the posterolateral wall of the roof of the nasal cavity where the sphenoidal
process of the palatine bone meets the horizontal ala of the vomer. This is located at a point just above the
sphenopalatine foramen, thus allowing for extension into the nose, nasopharynx,
sinuses, temporal or infratemporal fossa, or cranium.
The diagnosis is typically made by
clinical exam and radiographic findings.
On imaging, opacification of one or paranasal sinuses is usually
seen. The presence of anterior bowing
of the posterior wall of the maxillary sinus is pathognomic. Computed tomography or MRI can document the
extent of the lesion and provide the diagnosis in most cases. Biopsy is rarely recommended, however, if
necessary should be done in a controlled setting under general anesthesia with
preparations made for tumor removal.
Angiography can delineate the vascular supply to the tumor, however is
typically used for embolization of the tumor prior to surgery. When possible, diagnostic angiography and
embolization should be carried out simultaneously 48 hours prior to
surgery. While most common feeding
vessels are from the external carotid system (internal maxillary and ascending
pharyngeal arteries), internal carotid contribution may occur with large tumors
and therefore angiography should include bilateral internal and external
carotid systems.
Surgery is the recommended treatment
for this tumor, however, because the major vascular supply arises from the
internal and external carotid arteries, surgery can result in extensive blood
loss. Most surgeons utilize
preoperative arterial embolization to decrease intraoperative blood loss and
some prescribe stilbestrol for 5 weeks preoperatively to reduce the
vascularity. Additional measures of
managing blood loss include autologous blood banking preoperatively and the use
of a Cell Saver device during the procedure.
Depending on the location, a number of surgical approaches have been
utilized. Transnasal endoscopic removal
of tumor is generally limited to those tumors confined to the nasopharynx,
nose, or paranasal sinuses. A lateral
rhinotomy or midfacial degloving procedure combined with a medial maxillectomy
or LeFort I osteotomy provides exposure of the nasal cavity, ethmoid sinuses,
maxillary sinuses, and pterygomaxillary fossa.
Transpalatal and transpharyngeal routes achieve access inferiorly and
preauricular subtemporal access to the infratemporal fossa laterally. A facial translocation or maxillary swing
can be extended to provide further access to the infratemporal fossa and skull
base. Intracranial extension may be
removed with a formal craniotomy, if necessary. The recurrence rate after surgical treatment varies from 20% up
to 40% in some studies, typically due to incomplete resection. Recurrent lesions may be managed with additional
surgery, however radiotherapy and chemotherapy can also be used. Because of the effects of radiotherapy on
the growth of the craniofacial skeleton and the potential carcinogenic effects
of radiotherapy, many clinicians reserve radiotherapy for unresectable, life
threatening tumors. Chemotherapy is
usually offered for unresectable disease in which radiation is
ineffective.
MALIGNANT TUMORS
Angiosarcoma
Angiosarcoma of the head and neck is
extremely rare. These tumors represent
less than 1% of all sarcomas in humans with 50% of the cases involving the head
and neck region. The most common region
in the head and neck is the scalp and facial skin, with the neck, oropharynx,
and sinonasal tract following in decreasing order. Most of these tumors are rapidly growing requiring prompt
diagnosis and treatment if long term survival is to be expected. The prognosis is very much dependent on
tumor size and degree of cellular differentiation. Treatment consists of wide surgical resection with tumor margins
being another important prognostic factor.
Unfortunately, accurate identification of margins are difficult due to
the presence of anastomosing vascular channels dissecting the underlying
stroma. Histologically, they are
classified as either high or low grade depending on the number of mitoses
observed and the overall appearance of the lesion. Radiation therapy is minimally effective and usually given as an
adjunct to surgical therapy or as palliative treatment. Angiosarcomas arising in the sinonasal tract
have been found to behave less aggressively than those found in other areas,
thereby allowing for improved survival in these areas. Overall survival is poor, less than 50% at
five years.
Hemangiopericytoma
Stout and Murray first described hemangiopericytomas
in 1942. These tumors arise from the
pericytes of Zimmerman, which are cells that normally give mechanical support
to capillaries and regulate luminal size.
Overall, 25% of hemangiopericytomas arise in the head and neck, however
they represent only a small portion of all head and neck tumors. Many series on this lesion in the head and
neck literature involve the sinonasal tract as the primary site. Similar to the angiosarcomas,
hemangiopericytomas arising in the sinonasal tract behave less aggressively and
have been termed “hemangiopericytoma-like” when arising in this tissue.
The tumor typically presents as a
painless mass in all age groups predominately in the 6th and 7th
decades of life, with no sex predilection.
The etiology is unknown, although these lesions have been linked to
trauma, prolonged steroid use, and hormonal imbalances. The treatment of choice is wide surgical
excision which are known to insidiously recur even years later (57%). Well-differentiated tumors rarely metastasize,
however given their propensity to recur locally, these lesions are considered
malignant. Similar to angiosarcomas,
these lesions are graded as high or low grade tumors with survival rates
improved in the latter group. Radiation
therapy is usually reserved for recurrent lesions not amenable to surgical
excision or those with a more active histology. These tumors are poorly chemosensitive, however a recent report
documented a good response to alpha-interferon in two patients.
Kaposi’s
Sarcoma
Kaposi’s sarcoma is a multicentric
proliferation of vascular and spindle cell components, first described in
1872. It is now considered to be a
viral-induced tumor and it is unclear as to whether the lesion is a true tumor
or hyperplasia. It is strongly
affiliated with AIDS and its course is greatly influenced by the immune status
of the individual. Kaposi’s sarcoma has
four distinct clinical entities: classic, endemic, transplant-associated, and
AIDS-related. Classic Kaposi’s is not
associated with HIV and typically affects elderly men of Italian heritage. Endemic Kaposi’s is seen in endemic portions
of Africa among young black children.
This entity is rapidly progressive and affects lymph nodes and internal
organs diffusely. Transplant-associated
Kaposi’s affects transplant recipients that are immunosuppressed and is
correlated with loss of cellular immunity.
AIDS related Kaposi’s is found primarily in male homosexuals with 40% of
affected AIDS patients developing this entity.
Lesions occur in many cutaneous locations, especially along lines of
cleavage and on the tip of the nose.
Various treatments have been used with Kaposi’s with varied
success. Small lesions can be
surgically excised but more recent therapies have concentrated on low-dose radiation
and intralesional chemotherapy and sclerosing solutions. For larger lesion, chemotherapy is effective
but can be quite morbid to the HIV infected individual.
OTHER VASCULAR TUMORS
Paragangliomas
Paragangliomas of the head and neck
are typically benign, slow growing tumors arising from widely distributed
paraganglionic tissue thought to originate from the neural crest. Paraganglia in the head and neck region are
closely aligned with the distribution of the parasympathetic nervous system and
often have a close spatial relationship with neural or vascular
structures. Paraganglia have been shown
to have chemoreceptor roles with modulation of respiratory and cardiovascular
function.
Carotid bodies are the largest
collection of paraganglia in the head and neck region and appear as small ovoid
structures on the medial aspect of the carotid bifurcation on each side of the
neck. Paraganglia are also located in
other locations in the head and neck including the middle ear, jugular bulb,
ganglion nodosum of the vagus nerve, larynx, and base of the heart. Histologically, their appearance is similar
to the normal histology of the paraganglia and includes two cell types. Type I cells (chief) cells are APUD type
cells with copious cytoplasm and large round or oval nuclei. Their cytoplasm contains dense core granules
that store and release catecholamines.
Type II (sustentacular) cells are elongated cells that closely resemble Schwann
cells although their function is not entirely clear. The two cell types are arranged into clusters with a core of
chief cells surrounded by sustentacular cells embedded in a fibrous
stroma. These clusters make up the
fundamental histologic structure (termed "Zellballen") and may be
somewhat enlarged in paragangliomas.
Nuclear pleomorphism and cellular hyperchromatism are common in benign
paragangliomas and should not be considered evidence of malignancy. In fact, there are no clear histologic
characteristics of malignancy in these lesions. Malignancy is based on the clinical finding of metastasis, not on
histologic examination.
Terms used in the past to describe
paragangliomas have included glomus tumors (general term used to describe a
cluster of specialized cells and more appropriately applied to tumors of the
skin and superficial tissues of the extremities), chemodectomas (describing a
tumor from chemoreceptor origin), carotid body tumors, and nonchrommafin tumors
(related to staining characteristics).
Currently, the correct terminology is paraganglioma based on the
anatomical location. The predominant
paragangliomas of the head and neck include carotid paragangliomas,
jugulotympanic paragangliomas, vagal paragangliomas, and laryngeal
paragangliomas. For the purposes of
this text, jugulotympanic paragangliomas will be excluded as these have been
discussed in other grand rounds presentations.
Carotid paragangliomas
Carotid paragangliomas are the most
common paragangliomas of the head and neck comprising approximately 60% of the
total. Their incidence is rare, occurring
in approximately 0.12% of all surgical specimens. They can occur at any age but mean age at diagnosis is 45-50
years. A slightly higher female
predominance persists. These tumors may
be multicentric 10% of the time with bilateral carotid body lesions being the
most common combination in multicentric lesions. Carotid paragangliomas are familial in 20% of cases in an
autosomal dominant fashion with a higher propensity for multicentric lesions in
familial types. Malignancy occurs in
approximately 10% of cases which ranks carotid paragangliomas as the most
frequently malignant of all paragangliomas in the head and neck.
The lesions usually present as a
painless mass that is slow growing along the anterior border of the
sternocleidomastoid muscle. They tend
to splay the carotid bifurcation as they enlarge and can extend along the
carotid artery to the skull base.
Patients typically have noted the mass for many years, on average 2-8
years. Very large lesions may present
with vocal cord paralysis or dysphagia.
On examination, the masses are freely mobile laterally however they are
immobile in a cephalad-caudad direction.
The mass may be pulsatile and a bruit may be auscultated. Carotid paragangliomas may also produce
catecholamines, therefore, patients with symptoms of catecholamine excess
should be screened for urinary metanephrines and VMA (vanillyl mandelic acid)
as well as circulating catecholamines.
Diagnosis can be made with either CT
or MRI which shows a mass arising from the carotid bifurcation and displaces
the internal and external carotid arteries.
The diagnosis is confirmed with arteriography by revealing a
characteristic tumor blush at the carotid bifurcation called the lyre sign. This modality can establish the diagnosis,
demonstrate multiple lesions, determine the size and vascularity of the tumor,
as well as evaluate its blood supply.
Additionally, it can be modified to include selective, controlled
balloon occlusion of the internal carotid artery to evaluate cerebral cross-flow. This information is important in
preoperative counseling of the patient as to the relative risk of surgery. Since embolization is not often used with
carotid body paragangliomas, MRA may be an appropriate alternative to
angiography in selected cases. Biopsy,
including fine needle aspiration is unnecessary and contraindicated in the
evaluation of paragangliomas. Some
argue for routine screening of urinary metanephrines and VMA and serum
catecholamines for all cases, however others recommend these tests only for
familial forms or in the presence of catecholamine excess.
Surgery is the mainstay of treatment
for carotid paragangliomas. The
recurrence rate is approximately 10% with the mortality rate intraoperatively
of up to 8%. Because of their close
approximation to important vessels and nerves, there is a real risk of
morbidity (usually CN X-XII and vascular injuries). Tumor size is important because those greater than 5 cm in
diameter have a markedly higher incidence of complications (67% vs. 15%). An extensive preoperative workup is
essential for safe resection of these tumors.
In cases where the internal carotid artery may require resection, a
vascular surgeon should be available for assistance. Perioperative alpha and beta adrenergic blockers should be
available for all catecholamine producing tumors. Mot authors do not recommend embolization preoperatively as this
interferes with the subadventitial dissection necessary for removal of these
tumors. Complications include permanent
nerve palsy (20%), baroreceptor failure (bilateral), and “first-bite” syndrome
(from denerved parotid myoepithelial cells).
Radiation is usually reserved for incompletely excised tumors (with
intracranial extension), recurrent tumors, or poor surgical candidates. The mortality rate of untreated carotid
paragangliomas is estimated at 8% per year, indicating the indolent nature of
this tumor. Malignancy rate in carotid
paragangliomas is estimated between 2-10%.
There is no histologic criteria for malignancy, only the finding of
spread to regional lymph nodes or distant sites.
Vagal paragangliomas
Vagal paragangliomas arise most
commonly at the level of the nodose ganglion but may occur anywhere along the
course of the vagus nerve in the neck.
Mean age at presentation is 50 years and there is a slight female
predominance. The presentation is
usually one of a painless slow growing mass located behind the angle of the
mandible that has been present for many years.
The patient may complain of tongue weakness, hoarseness, or have a
Horner’s syndrome. Imaging studies such
as a CT or MRI may delineate the tumor from surrounding structures with
angiography classically demonstrating a tumor blush that displaces the carotid
artery anteriorly and medially.
Catecholamine producing vagal paragangliomas are virtually nonexistent
however are multicentric in approximately 25% of sporadic lesions. Vagal paragangliomas arising in the familial
form exhibit multicentricity in 78% of patients. Malignancy in these tumors is estimated at 18%.
Management is complicated due to
their propensity for multicentricity.
Some argue that surgical treatment is the mainstay however cranial nerve
deficits can be expected with their removal.
Radiation can be used but responses are often suboptimal.
Laryngeal paragangliomas
Paragangliomas of the larynx usually
arise from the superior laryngeal paraganglia above the anterior part of the
vocal folds near the aryepiglottic fold.
Hoarseness and dysphagia are the most common complaints and these are
associated with high rates of malignancy.
Laryngeal lesion usually require wide local excision or partial
laryngectomy. Radiation has not been
effective in controlling these rare entities.
Bacillary
Angiomatosis
Although not clinically a tumor,
bacillary angiomatosis can clinically mimic many of the other previously
described vascular tumors, particularly Kaposi’s sarcoma. This skin lesion is a vasoproliferative
response to infection by Bartonella species of bacteria. It is this bacteria that causes cat scratch
disease in immunocompetent children, however bacillary angiomatosis typically
occurs in immunocompromised individuals.
Treatment of this condition involves appropriate antibiotic therapy,
usually erythromycin.
References
Carrau
RL, Snyderman CH. Juvenile nasopharyngeal angiofibroma. In: Myers EN, Ed. Operative Otolaryngology-Head and Neck Surgery. Philadelphia: W.B. Saunders, 1997.
Johnson
JT. Carotid paraganglioma. In Myers EN, Ed. Operative Otolaryngology-Head and Neck Surgery. Philadelphia: W.B. Saunders, 1997.
Maves
MD, Stevens, C. Vascular tumors of the
head and neck. In Bailey BJ, Ed. Head and Neck Surgery-Otolaryngology. Philadelphia: Lippincott-Raven, 1998.
Willing
SJ. Atlas of Neuroradiology. Philadelphia: W.B. Saunders, 1995.
Gnepp
DR. Diagnostic and Surgical Pathology
of the Head and Neck. Philadelphia:
W.B. Saunders, 2001.
Mulliken
JB, Young AE. Vascular Birthmarks:
Hemangiomas and Malformations.
Philadelphia: W.B. Saunders, 1988.
Fagan
JJ, Snyderman CH, Carrau RL, et al.
Nasopharyngeal angiofibromas: selecting a surgical approach. Head and Neck, August 1997, 391-398.
Danesi
G, Panizza B, Mazzoni A, et al.
Anterior approaches in juvenile nasopharyngeal angiofibromas with
intracranial extension. Oto-HNS,
February 2000, 277-283.
Brown
TJ, Firedman J, Levy ML. The diagnosis
and treatment of common birthmarks.
Clinics in Plastic Surgery, October 1998, 509-525.
Greinwald
JH, Burke DK, Sato Y, Poust RI, Kimura K, et al. Treatment of lymphangiomas in children: An update of Picibanil
(OK-432) sclerotherapy. Oto-HNS,
October 1999, 381-387.
Williams
EF, Stanislow P, Dupree M, Mourtzikos K, Mihm M, Shannon L. Hemangiomas in infants and children. Archives in Facial Plastic Surgery,
April-June 2000, 103-111.
Netterville
JL, Jackson CG, Miller FR, et. al.
Vagal paraganglioma: a review of 46 patients treated during a 20 year
period. Archives of Oto-HNS, October
1998, 1133-1140.
Ungkanot
K, Byers RM, Weber RS, Callender DL, Wolf PF, Goepfert H. Juvenile nasopharyngeal angiofibroma: an
update on therapeutic management. Head
and Neck, January-February 1996, 60-66.
Netterville
JL, Reilly KM, Robertson D, Reiber ME, et al.
Carotid body tumors: a review of 30 patients with 46 tumors. Laryngoscope, February 1995, 115-125.
Carew
JF, Singh B, Kraus DH.
Hemangiopericytoma of the head and neck. Laryngoscope, September 1999, 1409-1411.
Billings
KR, Fu YS, Calcaterra TC, et al.
Hemangiocytoma of the head and neck.
American Journal of Otolaryngology, July-August 2000, 238-243.