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10 Lateral Cervical Swellings
237
in the middle age (40–60 years) [252]. Lipomas
commonly occur subcutaneously anywhere in the
body where adipose tissue is present; however, subcutaneous (SC) lipomas are rare in children but can
occur as part of the inherited disease “BannayanZonana syndrome” [253, 254]. Common locations
include upper back, shoulders, and abdomen but can
occur in other sites including the neck [255].
Lipomas are usually single, but about 5% are multiple (lipomatosis). They are usually relatively small
in size with diameters of about 1–3cm, but in rare
cases, they can grow over several years into “giant
lipomas” that are 10–20cm across and weigh up to
4–5kg [256, 257].
10.6.2.2 Etiology
The tendency to develop a lipoma is not necessarily hereditary, although hereditary conditions
such as familial multiple lipomatosis might
include lipoma development [258, 259]. Genetic
studies in mice have shown a correlation between
the HMG I-C gene and development of lipoma.
These studies support prior epidemiological data
in humans showing a correlation between HMG
I-C and mesenchymal tumors [260].
Some cases have been reported where minor
injuries are alleged to have triggered the growth
of a lipoma, called a “post-traumatic lipoma”
[261]. However, the link between trauma and the
development of lipomas is still controversial
[262]. Risk factors reported include family history, obesity, and lack of exercise [263].
10.6.2.3 Types/Subtypes
Simple (Typical) Lipoma
Simple (Supercial) Subcutaneous (SC)
Lipoma
Simple or typical SC lipoma is the commonest
type that presents just below the surface of the
skin as a soft, lobulated, mobile swelling with a
well-dened slippery edge. The SC sites include
any part of the body where fat is located, but the
commonest are the neck (Fig. 10.23), back,
shoulder, trunk, buttocks, thigh, and forearm.
The overlying skin is usually attached to the
capsule by brous bands so that it is often dimpled or becomes so on moving the tumor.
Pseudo- uctuation can usually be elicited due to
its fat contents. It is not tender and trans-illumination is negative. In children, it is sometimes
mixed with angiomatous tissue forming a
hemangio-lipoma. It should be differentiated
from other SC lesions such as dermoid cysts and
cold abscesses. Treatment is usually by surgical
excision [264].
Simple Lipomas inOther Locations
Other than the SC location, a simple lipoma may
be submucous, subserous, sub-synovial, subperiosteal, parosteal, extradural, and intraglandular.
It may also occur at the cerebello-pontine angle,
internal auditory canal, and corpus callosum
[264].
Fig. 10.23 A
subcutaneous lipoma in
the posterior triangle of
the neck in a 62-year-old
gentleman; (a) front
view, (b) lateral view
ab
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238
M. Sakr
Inltrating (Atypical) Lipoma
Subfascial Lipoma
A subfascial lipoma may be found under the palmar or plantar fascia in close relation to the tendon sheath. Its lobulations are, therefore, usually
not felt. It should be differentiated from tenosynovitis. It may be found also in the areolar
layer under the epicranial aponeurosis (sub-
aponeurotic lipoma) causing a depression in the
skull where it should be differentiated from a dermoid cyst. Investigations include Plain X-Ray,
CT scan, and biopsy. Treatment is by surgical
excision or liposuction.
Intramuscular andIntermuscular Lipoma
In 1963, Greenberg etal. recognized that inltrating lipomas may be either intermuscular or intramuscular [265]. Intramuscular lipomas have
nearly identical histology to supercial lipomas;
however, they tend to invade the adjacent musculature and commonly lack a capsule, and hence
they are sometimes known as “inltrating
lipoma.” In contradistinction, intermuscular lipomas do not exhibit local invasion and tend to be
lobular or dumb-bell-shaped, easily separated
from adjacent soft tissues during surgical resection [266]. Subsequently, in 2014, it was also recognized that intramuscular lipomas can present
as inltrative, well-dened/non-inltrative, and
mixed (with areas of inltration and well-dened
areas) variant [267].
Mixed Lipoma
Mixed lipomas include the following varieties
(subtypes) (1) angio-lipoma (lipoma with distinct
vascular and brous components rather than
being purely lipomatous), [268], (2) angio-lipoleiomyoma (well-circumscribed SC tumors composed of smooth muscle cells, blood vessels,
connective tissue, and fat) [264], (3) adenolipoma ( a lipoma that is associated with eccrine
sweat glands) [269], (4) bro-lipoma (a lipoma
with focal areas of large amounts of brous tissue) [264], (5) neural bro-lipoma (an overgrowth of bro-fatty tissue along a nerve trunk)
[270], (6) broblastic or spindle cell lipoma composed of fat cells and broblasts in a myxoma-
tous stroma) [264], and (7) pleomorphic lipoma
(characterized by oret giant cells with overlapping nuclei) [264].
Hibernoma
Hibernoma is a rare benign tumor that commonly
affects adults in the fourth decade, with a slight
male predominance. Hibernoma are most commonly identied in the SC and muscle tissue of
the head and neck region (shoulders, neck, and
scapular), followed by thigh, back, chest, abdomen, and arms [271]. Cytologically, FNAC
smears show small, round, brown fat-like cells,
with uniform, small cytoplasmic vacuoles and
regular, small, round nuclei [272]. There is a rich
vascular background of branching capillaries. It
is not uncommon to also have mature fat cells.
Immunohistochemistry (IHC) shows that the
neoplastic cells are S100 protein-positive (~80%),
and show membrane and vacuole CD31 immunoreactivity. Uncoupling protein 1 (UCP1), a
unique brown fat mitochondrial protein, is also
positive [273]. It is important to differentiate
hibernoma from adult rhabdomyoma, a granular
cell tumor, and a true liposarcoma. Complete sur-
gical excision is the treatment of choice, associated with an excellent long-term clinical
outcome.
Diuse (Hereditary) Lipomatosis
In 5–15% of patients, lipomas are multiple, and
approximately a third of these will be familial.
Diffuse (hereditary) lipomatosis is characterized
by the presence of multiple lipomata in the body.
It may occur around the neck (Madelung’s dis-
ease), in the supra-clavicular region and trunk
(Dercum disease), around the hips and thighs (in
menopausal women), or in the gluteal area (in
hypo-pituitrism). Generally, for treatment of
lipomatosis, suction techniques offer a more
attractive and feasible approach than multiple
resections [274].
In some cases, multiple lipomas (lipomatosis)
are associated with syndromes and other diseases, including Bannayan-Zonana syndrome,
Cowden syndrome, Frolich syndrome (adiposegenital dystrophy), Proteus syndrome, Dercum
disease (adipose dolorosa) [275], Madelung’s
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10 Lateral Cervical Swellings
239
disease (benign symmetric lipomatosis), and
familial multiple lipomatosis [276, 277].
be difcult to identify on US [274]. Inampudi
et al. (2004) reported a wide range of sonographic appearance of biopsy-proven lipomas
10.6.2.4 Clinical Presentation
[278]; hyper-echoic (20–52%), iso-echoic
(28–60%) and hypo-echoic (20%). Lipomas
Symptoms
The patient with a lipoma usually complains of a
slowly growing, painless, lump probably of many
years’ duration. It may cause disgurement or
interfere with movement (especially if pedunculated). If knocked repeatedly it may become hard
tend also to display other US characteristic features, such as absence of acoustic shadowing
and no or minimal color Doppler ow [279].
Heterogeneous echo-texture, more than minimal color Doppler ow, or large size is suspicious for liposarcoma [279].
and painful due to fat necrosis. Repeated friction
may cause ulceration of the overlying skin.
Computed Tomography (CT) Scan
Classic appearances are of a supercial circum-
Clinical Examination
Physical examination will reveal a single swelling (rarely multiple) around 2–5cm in size but
may be larger reaching up to 20 cm (giant
lipoma). It is usually spherical in shape but may
be attened or pedunculated. The surface is
smooth or lobulated and the edge is well-dened
and lobulated but slippery (slip-sign). Skin overlying is usually normal but may show faint blue
streaks (veins) or dimpling due to attachment to
the overlying skin by strands. A lipoma is soft
(pseudo-uctuant) in consistency with no thrill
and is dull on percussion. It is freely mobile and
not tender. Local LNs and tissues are usually normal. Clinically, it should be differentiated from
scribed, low attenuation mass with minimal
internal soft-tissue component. Areas of calcication may be present although they are more
frequently associated with well-differentiated
liposarcoma [274]. Deeper or larger lipomas
may have scattered areas of internal soft-tissue
density. These may represent areas of fat necrosis, brous tissue, blood vessels, or muscle
bers; these lesions cannot be condently differentiated from liposarcoma by imaging.
Intramuscular lipomas may invade and interdigitate with the associated skeletal muscle, resulting in a characteristic striated appearance, which
may help distinguishing it from liposarcoma
[274].
other SC tumors and cystic or pseudo-cystic
lesions such as sebaceous or dermoid cysts, and
cold abscesses.
Magnetic Resonance Imaging (MRI)
MRI is the modality of choice for imaging lipomas, not only to conrm diagnosis but also to
10.6.2.5 Investigations
better assess atypical features suggesting liposarcoma and to demonstrate the surrounding anat-
Imaging Studies
omy [280]. As expected, lipomas follow SC fat
signal on all sequences; T1 (high signal, saturates
Plain Radiograph
Large lipomas may be appreciated as a region of
low-density exerting mass effect. Calcication
may be present in up to 11% of cases, although
more commonly associated with welldifferentiated liposarcoma [274].
on fat-saturated sequences, no or minimal
enhancement), T2 (high signal on FSE T2, saturates on fat-saturated sequences: persistent areas
of high T2 signal are worrisome). When no suspicious features are present, the diagnosis of lipoma
can be made with condence with MRI being
100% specic [281]. Similarly, if suspicious fea-
Ultrasound (US)
Lipomas appear as soft variably echogenic
masses, commonly encountered on
US.However, if encapsulated, the capsule may
tures are present, then the sensitivity of MRI is
100% [281], although specicity is lower, as
some masses with atypical features will be
lipomas.
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240
M. Sakr
Biopsy/Histopathology
A lipoma is a circumscribed soft mass composed
of lobules of fatty areolar tissue (Fig. 10.24)
enclosed within a thin true capsule, surrounded
by a brous pseudo-capsule (from surrounding
tissues) and separated from the true capsule by a
plane of cleavage along which the tumor can be
enucleated. Histology demonstrates mature adipocytes with no cellular atypia or pleomorphism.
Any non-adipose components must be carefully
assessed to exclude a more aggressive component. Myxomatous degeneration (liquifaction) or
calcication may occur. Sarcomatous change
(liposarcoma) is very rare (<1%) except in certain sites (back, nape and retro-peritoneum),
where the risk is higher [274].
10.6.2.6 Treatment
Usually, treatment of lipomas is not necessary,
unless they become painful or restrict movement.
They are usually removed for cosmetic reasons if
they grow very large or for histopathology to
verify that they are not malignant (liposarcoma).
Simple Excision
Lipomas are removed by simple excision through
the line of cleavage [252]. Excision can often be
done under local anesthetic and takes less than
30 min. This cures the great majority of cases,
Fig. 10.24 Histopathology of a lipoma composed of lobules of fatty areolar tissue and mature fat cells. H&E stain
with about 1–2% of lipomas recurring after excision if a fat lobule (or more) is missed and left
behind [282].
Liposuction (Suction-Assisted Lipectomy)
Liposuction is another option if the lipoma is soft
and has a small connective tissue component.
This technique involves freeing up of fatty deposits by probing between the SC brous septa and
then suctioning the loosened fatty tissue by powerful suction machines. Liposuction typically
results in less scarring; however, with large lipomas, it may fail to remove the entire tumor, which
can lead to regrowth [283].
Newer Methods
New methods under development are supposed
to remove the lipomas without scarring. One is
removal by injecting compounds that trigger
lipolysis, such as steroids or phosphatidylcholine [252, 284]. Other potential methods
based on tissue-targeted heating include cauterization, electro-surgery, and harmonic scalpel
[285].
10.6.2.7 Prognosis
Lipomas are rarely life-threatening, and the common SC lipomas are not a serious condition.
Lipomas growing in internal organs can be more
dangerous; for example, lipomas in the gastrointestinal tract can cause bleeding and ulceration
[286, 287].
Recurrence rates of 4–5% are reported [274],
most in deeper lesions, because complete surgical removal of deep lipomas is not always possible [288].
Malignant transformation of lipomas into
liposarcomas is very rare, and most liposarcomas
are not produced from preexisting benign lesions
[282]. Liposarcoma is found in 1% of lipomas
and is more likely to occur in lesions of the lower
extremities, shoulders, and retro-peritoneal areas.
It occurs most frequently in old patients and in
men more than women. Clinically, it is hot, vascular (dilated veins on the skin overlying), tender,
and grows rapidly. Signs of dissemination may be
detected. Other risk factors for liposarcoma
include large size (>5cm), associated with calci-
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10 Lateral Cervical Swellings
241
cation, rapid growth, and/or invasion into
nearby structures or through fascia into muscle
tissue [289]. It is liable to recur after excision.
10.6.3 Collagenous Fibroma
10.6.3.1 Epidemiology
Collagenous bromas (desmoblastic broblastomas) are rare brous soft tissue tumors that usually arise in the SC tissue or skeletal muscle, and
rarely dermal involvement, at several anatomical
sites. They occur predominantly in males, in the
fth or sixth decade of life, with a median age at
presentation of 50years. They most commonly
arise in the arm or shoulder [290, 291] but have
also been described in the neck [292], back [293],
hip [294], extremities [295], parotid [296], and
palate [297, 298].
10.6.3.2 Clinical Presentation
Collagenous bromas commonly present as a
painless, non-tender, slow-growing, and mobile
masses [290]. They usually measure approximately 4 cm in maximum diameter, although
tumors up to 20cm in size have been reported in
the literature [299, 300].
10.6.3.3 Investigations/Diagnosis
Imaging Studies
US usually shows a mass with mixed echogenicity [301–303]. On CT scan, desmoplastic
broblastomas usually appear as nonspecic
inhomogeneous soft tissue density mass that may
be suggestive of a soft tissue sarcoma [290, 303].
The preferred imaging modality at present to
delineate collagenous bromas is MRI of the
neck; it shows a lesion of intermediate T1 and
low T2 signal intensity (ascribed to the low cellularity of these lesions) that may not be separated from the adjacent muscles [291].
hypo-cellular lesion that consists of mature fat,
lymphoid tissue and nerve trunks, containing a
pauci-cellular brous lesion with spindle to stellate-shaped broblasts (Fig.10.25) and a diffuse,
permeative architecture mimicking inltration and
extending between and around nerves and muscle
bundles. No mitotic gures, nuclear hyperchromasia, or cytological atypia are present to suggest a
malignant neoplasm. There is no necrosis and it
can have scattered blood vessels, sometimes with
perivascular hyalinization.
Immunohistochemistry (IHC) is usually not
necessary for diagnosis. On IHC, broblasts
exhibit a myobroblastic phenotype expressing
calponin and can have focal staining for smooth
muscle actin and factor XIIIa. Lesional cells are
negative for S100 protein, pancyto-kerating, desmin, CD34 and HMB45.
10.6.3.4 Dierential Diagnosis
It is important to differentiate collagenous
bromas from other reactive or neoplastic soft
tissue lesions, particularly malignant soft tissue tumors due to implications regarding
appropriate treatment. The main differentials
include neuro- broma (S100-positive, neural
cells in myxoid stroma), bromatosis (much
more cellular, often fascicular growth pattern),
elasto-broma (typically on shoulder, has
prominent wavy elastic bers), malignant
Biopsy
Macroscopically, the tumor is a wellcircumscribed, rm, oval mass, tan-grey in color,
with a homogeneous consistency. Microscopically, collagenic broma is a well- circumscribed
t.me/Dr_Mouayyad_AlbtousH
Fig. 10.25 Histology of collagenous broma. High
power showing a prominent collagenous stroma and scattered bland stellate and spindle-shaped broblast. H&E
stain
242
M. Sakr
brous histiocytoma, and calcifying brous
pseudo-tumor [290, 291, 304].
10.6.3.5 Treatment
The recommended treatment is complete local
surgical excision to minimize potential morbidity
[290–300, 305, 306]. There are no reports of
local recurrence or metastases to date [265–275,
282], the longest follow-up period being 12years
[307].
10.6.4 Fibromatosis (Desmoid
Tumor)
10.6.4.1 Denition/Synonyms
Fibromatoses or desmoid tumors are a group of
rare, locally aggressive broblastic/myobroblastic tumors arising from the connective tissue of the muscle, the overlying fascia or
aponeurosis, with no metastatic potential
[308]. Although considered benign, they are
locally inltrative and can cause extensive
morbidity by destruction of adjacent vital
structures and organs and can have a propensity for local recurrence [309]. They may be
associated with familial adenomatous polyposis (Gardner syndrome) [310].
Synonyms include aggressive bromatosis,
musculo-aponeurotic bromatosis, desmoid
tumor, and deep bromatosis.
10.6.4.2 Epidemiology/Sites
Incidence of desmoid tumors is estimated at
2.4–4.5 cases per million persons annually
[311]. They constitute approximately 0.03% of
all neoplasms and <3% of all soft tissue tumors.
There is a female predominance from puberty to
age 40years. The mean age of involvement is
36–42 years [312]. Desmoids occur sporadically, but in 3–35% they can be seen in patients
with familial adenomatous polyposis (FAP)
[313, 314] with a male predominance of 3:1
[315]. Most of head and neck cases (80%) occur
in the antero- lateral aspect of the neck, followed
by the posterior triangle, face, oral cavity, scalp,
and para-nasal sinus [316].
10.6.4.3 Etiology/Pathophysiology
Desmoid lesions may be driven by a combination of
genetic mutations, high estrogen states, and antecedent trauma, which leads to activation of the
canonical Wnt/β catenin pathway. Salas etal. (2010)
stated that CTNNB1 and APC gene mutations can
account for up to 89% of bromatosis cases [317].
10.6.4.4 Clinical Picture
Although desmoid tumors are benign their clinical behavior is unpredictable. Rock etal. (1984),
in a series of 194 patients with extra-abdominal
desmoid tumors, reported a 68% recurrence rate
after the rst treatment [318]. Such an aggressive
behavior combined with the presence of many
vital structures in a conned area in the head and
neck imposes a major therapeutic challenge.
10.6.4.5 Investigations/Diagnosis
Imaging Studies
Ultrasonography (US) usually shows an oval,
well- to poorly marginated solid soft tissue mass
with variable echogenicity. Desmoid tumors may
have thin, linear extension along fascial planes
(fascial tail sign), or intramuscular nger-like
extensions (stag horn sign). CT scan is most
commonly usually demonstrating a soft tissue
mass with well- to poorly dened margins, variable attenuation, and mild-to-moderate enhancement. The preferred modality for diagnosis is
MRI.Fibromatosis appears as heterogeneous isoto hyper-intense to skeletal muscle on T2, and
iso-intense to muscle on T1. Non-enhancing linear bands (Band sign) can be seen in nearly
60–90% of cases. In addition, MRI shows a moderate to marked enhancement after gadoliniumbased contrast [319].
Pathology
Gross Appearance
A desmoid tumor is typically 5–10cm in size; it
may be may be poorly dened or wellcircumscribed. It is rm in consistency, glistening white, gritty and with a coarsely trabeculated
cut-surface resembling scar tissue.
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10 Lateral Cervical Swellings
243
Cytology
FNAC shows bland spindle cells with long, fusiform nuclei, and metachromatic matrix material.
Tumor cells are individual or as fragments within
matrix [320]. There may be long fascicular
arrangement of spindle cells [321]. FNA is fairly
reliable for diagnosis but core-needle biopsy is
generally considered to be better [322].
Histological Picture
Typically, bromatosis has poorly dened borders and inltrating margins (Fig.10.26) Several
histological patterns have been recognized [323];
conventional pattern (most common), hypocellular/hyalinized pattern, stag horn vessel pattern, myxoid pattern, nodular fasciitis-like
pattern, and hyper-cellular pattern.
Immuno-histochemistry (IHC)
Fibromatosis is positive for SMA and MSA
stains and rarely focal desmin [324]. It is also
positive for beta-catenin (70–98%) [325, 326],
cyclin-D1 (71–75%) [327], calretinin (75%)
[328], sex hormones [329, 330], vimentin
(100%), and cathepsin (100%) [304].
Fibromatosis is characteristically negative for the
following stains: CD-34, S-100, cytoketating
[324], DOG-1 [327], c-kit [331], MUC-4 [332],
and Ki-67 (very low positivity, 1–3%) [333].
Molecular/Cytogenetic Analysis
CTNNB1 mutations are reported in approximately 90% of patients with bromatosis, usually in exon 3; most common mutations are
T41A, S45F, and S45P [334].
10.6.4.6 Dierential Diagnosis
Fibromatosis should be differentiated from nodular fasciitis, myo-broma/myo-bromatosis, calcifying brous pseudo-tumor, solitary brous
tumor, nerve sheath tumors, brosarcoma,
inammatory myo-broblastic tumor, low-grade
myo-broblastic sarcoma, and low-grade bromyxoid sarcoma [335].
10.6.4.7 Treatment
At present the management of desmoid tumors
includes the use of surgery, radiation therapy
(RT) as well as cytotoxic and non-cytotoxic chemotherapy. Given the benign nature of these
tumors, treatment strategies aim at achieving
local control while preserving proper function
and providing with an adequate cosmetic result
[210].
Surgical Treatment
When feasible, complete resection of the tumor
with negative microscopic margins is the rstline and mainstay treatment [336]. In contrast to
some published series by Posner et al. (1989)
[309] and Ballo etal. (1999) [336], a more recent
study by Gronchi etal. (2003) showed that the
presence of microscopic disease did not affect
long-term disease-free survival in patients with
primary presentation of extra-abdominal desmoid tumors [337].
Fig. 10.26 Desmoid tumor showing inltrativeappearing margins with involvement of the surrounding
fat
t.me/Dr_Mouayyad_AlbtousH
Radiation Therapy (RT)
“Primary” RT is an appropriate alternative for
treatment of patients who are not good surgical
candidates [338]. On the other hand, the use of
postoperative “adjuvant” RT is unclear [339,
340]. Some researchers advocate that RT should
not be pursued in patients with primary disease
and either negative or positive surgical margins
but only in cases with gross residual disease after
surgery [341, 342].
244
M. Sakr
Chemotherapy/Systemic Therapy
Systemic therapy of desmoid tumors includes a
variety of non-cytotoxic (tamoxifen, testolactone
and nonsteroidal anti-inammatory drugs) and
cytotoxic agents (methotrexate, vinblastine and
doxorubicin) [343]. Sze et al. (2009) [340]
reported a signicant tumor shrinkage with lowdose methotrexate and vinblastine. Antihormonal therapy (tamoxifen) can be used as rst
line in asymptomatic patients. In general, possible candidates for systemic therapy include those
patients with Gardner’s syndrome and unresectable (advanced) or recurrent desmoid tumors,
involving the mesentery [344].
10.6.4.8 Prognosis
Fibromatosis has a highly variable natural course,
with up to 25% undergoing spontaneous regression. Complete surgical excision of desmoid
tumors may cause signicant morbidity and mortality in certain locations [345–347]. Local recurrence is encountered in 20–30% of bromatosis
lesions. The margin status has been shown to predict local recurrence in primary tumors but was
not signicant in recurrent presentations. Other
factors such as age, gender, presentation status,
tumor size, tumor focality, and histological type
have shown inconsistent predictive value. Van
Broekhoven etal. (2015) reported that CTNNB1
S45F mutation is associated with a signicantly
increased risk of recurrence [348].
10.6.5 Virchow’s Lymph Node
10.6.5.1 Surgical Anatomy
Currently, “Virchow’s node” in the left supraclavicular fossa refers to the terminal node along
the thoracic duct, while “Troisier’s sign” actually
refers to the clinical exam nding of an enlarged,
rm left supra-clavicular node [349]. Virchow’s
node receives afferent lymphatic drainage from
the left head, neck, chest, abdomen, pelvis, and
bilateral lower extremities, which eventually
drains into the jugulo-subclavian venous junction
via the thoracic duct.
The supra-clavicular fossa refers to the depression in the skin above the clavicle and in the
lower neck. It is dened as two compartments,
the lesser and greater compartments. The lesser is
a depression between the sternal and clavicular
heads of the SCM muscle. The greater is the base
of the posterior triangle of the neck, also known
as the omo-clavicular triangle. The boundaries of
the supra-clavicular fossa on CT scan are the trachea medially, the SCM, platysma, and deep cervical fascia anteriorly, the anterior scalene
postero-laterally, the carotid artery and IJV
postero- medially, and the subclavian artery
postero- inferiorly [350].
Supra-clavicular LNs are invested in a fat pad
that lies directly over the anterior scalene muscle,
just lateral to the carotid sheath. The phrenic
nerve and the transverse cervical and suprascapular arteries run through this region, as does
the thoracic duct on the left side [350]. A normal
LN normally has an elongated appearance with a
fatty hilum and a central vascular pedicle, giving
it a reniform morphology [351–356].
10.6.5.2 Pathophysiology
The cisterna chyli serves as the central depository
of lymphatic drainage for the lower intercostal,
gastric, superior mesenteric, inferior mesenteric,
lumbar, internal iliac, and external iliac lymphatics. The cisterna chyli subsequently empties into
the thoracic duct, which then drains superiorly in
the thorax, subsequently draining the left “jugular, subclavian, and mediastinal” LNs. At the terminal end of the thoracic duct is Virchow’s node,
and the thoracic duct nally empties into the left
subclavian vein [357].
Theories for the mechanism of involvement of
the terminal nodes involve seeding of these terminal nodes due to reux within the low-pressure
lymphatic system, resulting in focal retrograde
drainage into Virchow’s node [357]. Obstruction
of the thoracic duct is not mandatory to cause this
reux as it is seen in nearly 50% of patients without an obstructed thoracic duct. After the node is
seeded, the tumor proliferates, resulting in an
enlarged, rm, and abnormal node that may be a
sign of an abdominal malignancy on physical
examination, (Troisier’s sign) [358, 359].
On the other hand, the right “jugular, subclavian, and mediastinal” LNs drain into the right
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10 Lateral Cervical Swellings
245
lymphatic duct, which drains into the right subclavian vein. The abdominal drainage pathway
does not empty into the right lymphatic duct and
terminal right lymphatic duct nodes, or right
supra-clavicular nodes, and thus, should not present as the initial point of metastasis for abdominal malignancy.
10.6.5.3 Clinical Signicance
Enlarged supra-clavicular LNs have a strong
association with malignancy. Nasuti etal. (2001)
[360] demonstrated that 72% of ne-needle aspirates of supra-clavicular LNs resulted in the diagnosis of malignancy with 63% being metastatic
carcinoma. Other prior studies reported rates of
malignancy varying from 58 to 90% resulting
from primary malignancies inferior to the supraclavicular nodes, either in the thorax, abdomen,
or pelvis [361–364].
Right supra-clavicular lymphadenopathy is
associated with cancers of the lungs, esophagus,
breast, head/neck, and skin, as well as lymphoma.
Left supra-clavicular lymphadenopathy is associated with the same primary malignancies as the
right, in addition to pelvi-abdominal malignancies, including stomach/bowel, gallbladder, kidney/genitourinary, testicular, ovarian/
gynecologic, and prostatic malignancies. In case
of abdominal malignancy, identication of supraclavicular lymphadenopathy is considered as distant metastatic (stage 4—M1) disease, which has
a 5-year survival rate of only 4%, and often precludes surgical resection [365]. Sarcoidosis, a
benign but debilitating condition, has also been
shown to present with supraclavicular lymphadenopathy [366].
Because of its anatomical relations to critical
structures such as the phrenic nerve, the subclavian vessels, and the brachial plexus, enlargement of Virchow’s node can compress these
structures and cause various pathologies such as
unilateral phrenic neuropathy, which might lead
to dyspnea, vascular and neurogenic thoracic outlet syndromes, and Horner’s syndrome. The latter
is due to the compression of the lower part of the
brachial plexus (C8-T1), leading to the disruption
of sympathetic innervation of the eye and usually
manifests as four key clinical signs on the ipsilat-
eral side; miosis, ptosis, anhydrosis, and enophthalmos. Thus, the presence of a Troisier sign
should be kept in consideration when any of these
pathologies are present [359, 367].
10.6.5.4 Investigations/Diagnosis
Imaging Studies
The use of imaging methods such as CT scan,
MRI, PET, and US has helped clinicians to reach
a diagnosis of the cause of supra-clavicular LNs
[368–370]. Invasive methods such as
bronchoscopy, thoracoscopy, mediastinoscopy,
and exploratory thoracotomy are sometimes
essential for the proper diagnosis of intra- thoracic
lesions [371–373].
On imaging, “benign” LNs are elongated with
a thin symmetrical cortex and a robust fatty
hilum. On US, the cortex is hypoechoic and the
hilum is echogenic with vascular structures seen
usually only in the hilum. The fat density of a
normal hilum is often appreciable on CT scan
and should be seen in conjunction with homogeneous attenuation of the cortex. The cortex of the
LN demonstrates homogeneous signal intensity
on MRI.In addition, normal LNs appear hyperintense on diffusion-weighted imaging sequences.
The fatty hilum demonstrates signal intensity
similar to SC fat on all sequences. Margins of
normal LNs are typically smooth on all imaging
modalities. Enhancement of LNs is normal on
both CT and MRI [374].
The abnormal LN has a rounded (globular)
shape and lobulated or irregular margins, often
with asymmetric enlargement of the cortex, and
is usually larger than 1 cm in the short axis,
although 1.5cm may be used as the cut-off in the
axillary, inguinal, and submandibular regions
[375].
Cytology/Histology
Fine-Needle Aspiration (FNA)/Core-Needle
Biopsy (CNB)
Both FNA and CNB are safe and effective when
sampling supra-clavicular LNs [376, 377]. FNA
is also rapid, painless, inexpensive, and does not
require anesthetic or hospital admission. Several
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