Congenital Cystic Neck Masses: Embryology and
Imaging Appearances, With
Clinicopathological Correlation
Santhosh Gaddikeri, MD,a Surjith Vattoth, MD,b Ramya S. Gaddikeri, MD,c
Royal Stuart, MD,d Keith Harrison, MD,e Daniel Young, MD,d
and Puneet Bhargava, MDa,f
Congenital cystic masses of the neck are uncommon and
can present in any age group. Diagnosis of these lesions
can be sometimes challenging. Many of these have
characteristic locations and imaging findings. The most
common of all congenital cystic neck masses is the
thyroglossal duct cyst. The other congenital cystic neck
masses are branchial cleft cyst, cystic hygroma (lymphan-
gioma), cervical thymic and bronchogenic cysts, and the
floor of the mouth lesions including dermoid and epider-
moid cysts. In this review, we illustrate the common
congenital cystic neck masses including embryology, clin-
ical findings, imaging features, and histopathological
findings.
Introduction
Congenital cystic masses of the neck are uncommon
but important pediatric neck lesions. Many of these
have characteristic locations and imaging findings.
The most common congenital cystic neck mass is
thyroglossal duct cyst (TGD).1 The other common
lesions are second branchial cleft cyst and cystic
hygroma (lymphangioma). The uncommon cystic
From the aDepartment of Radiology, University of Washington, Seattle,
WA; bDepartment of Radiology, University of Alabama Hospital,
Birmingham, AL; cDepartment of Neuroradiology, Rush University
Medical Center, Chicago, IL; dDepartment of Radiology, University of
Alabama Children’s Hospital, Birmingham, AL; eDepartment of Pathology,
University of Alabama Children’s Hospital, Birmingham, AL; and fVA
Puget Sound Health Care System, Seattle, WA.
Reprint requests: Santhosh Gaddikeri, MD, Department of Radiology, 1959
NE Pacific Street, University of Washington, Seattle, WA 98195. E-mail:
santhosh.gaddikeri@gmail.com.
Curr Probl Diagn Radiol 2014;43:55–67.
& 2014 Mosby, Inc. All rights reserved.
0363-0188/$36.00 + 0
http://dx.doi.org/10.1067/j.cpradiol.2013.12.001
Curr Probl Diagn Radiol, March/April 2014
lesions include first and third branchial cleft cysts,
fourth branchial apparatus anomaly, cervical thymic
and bronchogenic cysts, and the floor of the mouth
lesions including dermoid and epidermoid cysts.
Anomalies of the branchial apparatus could also
present as sinuses and fistulae in addition to cysts.
In this article, we discuss the common congenital
cystic neck masses in an orderly fashion, including
embryology and pathogenesis, clinical findings, imag-
ing features, and histopathological findings.
A. Thyroglossal Duct Cyst
Embryology and Pathogenesis
The thyroid gland develops in the region of foramen
cecum of the tongue during the third gestational week.
The thyroid anlage descends down to reach the thyroid
bed anterior to laryngeal cartilages through the TGD
by the seventh week of gestation, and TGD begins to
involute by 8-10 weeks of gestation.2 If any segment
of the TGD fails to involute, then the persistent
secretory activity from the epithelial lining owing to
repeated infection or inflammation would give rise to
TGD cyst.1
Clinical Findings
Most TGD cysts are located either at or below the
level of the hyoid bone, with 50% at hyoid bone and
20%-25% in suprahyoid neck, often in the midline.
The remaining 25% are located in the infrahyoid neck
where they are in the midline or within the strap
muscles in a paramidline location (Fig 1).1 The TGD
cyst commonly presents as a painless, enlarging mass
in a child or young adult with varying sizes ranging
from 0.5-6.0 cm. The duct and cyst characteristically
55

FIG 1. Schematic representation of the thyroglossal duct (TGD)
extending from the region of foramen cecum (curved arrow) up to
the thyroid gland (TG). Note the close relationship of TGD cyst (broken
arrow) with the hyoid bone (solid arrow). (Color version of figure is
available online.)
move upward with tongue protrusion. Most of the
times the TGD cyst comes to notice owing to repeated
infection.3 Another rare but worrisome complication
of the TGD cyst is its association with thyroid
carcinoma. Complete excision of the cyst with resec-
tion of the central portion of the hyoid bone and a core
of tissue following the expected course of the TGD to
the foramen cecum (Sistrunk procedure) is the recom-
mended surgical approach. This procedure involves a
recurrence rate of 2.6%.4,5
Imaging
An uncomplicated TGD cyst has the appearance of a
cystic mass either in the midline of the anterior neck at
the level of the hyoid bone or within the strap muscles
in paramidline location. On ultrasound (US), an
anechoic mass with a thin outer wall in close associ-
ation with the hyoid bone easily establishes the
diagnosis of a TGD cyst. However, this “classic”
appearance is seen in less than half (42%) of the cases.
Other manifestations of these cysts include hypoechoic
masses with increased through-transmission, homoge-
neous or heterogeneous with variable degrees of fine to
coarse internal echoes, and may be associated with
56
thick cyst walls (Fig 2A). Fluid levels may be seen
owing to settling in of debris. There is no correlation
between the sonographic appearance and pathological
evidence of infection and inflammation. Heterogeneity
seen in TGD cysts on sonograms is more likely due to
the proteinaceous content of the fluid secreted from the
cyst wall rather than to infection.6 Preoperative sono-
graphic visualization of normal thyroid tissue is
sufficient to exclude a diagnosis of ectopic thyroid
tissue and obviates routine thyroid scintigraphy. Peri-
cystic fluid or inflammatory changes have been
described in infected TGD cyst.
On computed tomography (CT) scans, a TGD cyst
is a benign-appearing cystic neck mass, which may
show septations in it. Suprahyoid TGD cyst (Fig 2B
and C) may project into the pre-epiglottic space, and
infrahyoid TGD cysts are often off-midline embedded
in the strap muscles. Contrast administration may
demonstrate thin peripheral enhancement. However,
in the presence of infection, this enhancement can be
significant. If there is associated thyroid carcinoma in
the cyst, it can appear as an eccentric mass, which
may demonstrate foci of calcifications in it.
On magnetic resonance imaging (MRI), an uncom-
plicated TGD cyst has low signal intensity on
T1-weighted images, sometimes it can be isointense
to hyperintense related to proteinaceous contents of
the cyst. Most of them are homogeneously hyper-
intense on T2-weighted images (Fig 2D), which reflect
its fluid content. The rim will be nonenhancing unless
inflammation is present.7 In case of infection or
hemorrhage, a thick irregular rim may be visualized.
Nuclear scintigraphy is not routinely used. It is
recommended only if the sonography fails to identify
the normal thyroid tissue.
Histopathology
On histologic examination, the TGD cysts are lined
by stratified squamous epithelium or ciliated pseudos-
tratified columnar epithelium and filled with colorless,
viscous secretion (Fig 3).4 Sometimes they may
demonstrate mucous glands. Ectopic thyroid tissue
along the course of the duct is variably reported in up
to 62% of cases.
B. Branchial Cleft Cyst
Embryology and Pathogenesis
Branchial apparatus include 6 arches (mesoderm)
interfaced by 4 clefts (ectoderm) and pouches
Curr Probl Diagn Radiol, March/April 2014
FIG 2. Imaging manifestation of thyroglossal duct (TGD) cyst. (A) US longitudinal section of the midline neck TGD cyst (curved arrow) with
pseudosolid appearance. The cyst is closely related to the hyoid bone (solid arrow). (B) Sagittal contrast-enhanced CT image of the neck
demonstrates a well-circumscribed, nonenhancing TGD cyst (solid arrow) in close relation to the thyroid gland (arrowhead) inferiorly and hyoid
bone (small arrow) superiorly. (C) Axial contrast-enhanced CT image of the neck at the level of true vocal cords demonstrating small peripherally
enhancing thyroglossal duct cyst in the anterior strap muscle in right paramidline (arrow) location. (D) Sagittal T2-weighted MRI demonstrates a well-
circumscribed TGD cyst (solid arrow) in the region of foramen cecum. (Color version of figure is available online.)

(endoderm) on each side at the end of fourth week of
embryonic life (Fig 4).2 The fifth arch is rudimentary
and does not contribute to formation of any of the
FIG 3. Histopathological section (H&E, 4) shows the cyst wall
parallel to hyoid bone with an epithelial lining that transitions from
ciliated columnar to stratified squamous epithelium. (Color version of
figure is available online.)
adult structure. Incomplete obliteration of I, II, III, or
IV branchial apparatus can lead to respective branchial
remnants like isolated cyst (most common), fistula, or
sinus (rare). Second branchial cleft cyst accounts
for 490% of all branchial cleft anomalies in teens
and adults and 65%-75% in children.
Clinical Findings
The branchial apparatus remnants can appear as
cyst, sinus, or fistula. First branchial cleft cyst
commonly occurs in periauricular region, usually
presenting as recurrent parotid abscesses. Two types
of first branchial cleft cysts (Fig 5) have been
described by Work.8,9 Type I (periauricular cyst) in
which the cyst is located in close proximity to the
external auditory canal (EAC) either anterior, inferior,
or posterior to and often runs parallel to it. Sometimes
this lesion may “beak” toward the bony-cartilaginous
junction of EAC. Type II (periparotid) cysts are
usually found in the superficial parotid and may
extend into the deep lobe or the parapharyngeal space
and may even extend inferiorly up to posterior

Curr Probl Diagn Radiol, March/April 2014 57


FIG 4. Schematic representation of branchial apparatus demonstrates
arching of second branchial cleft (solid arrow) over III and IV arches
with formation of “Cervical sinus of His” (*). Incomplete obliteration of
this cervical sinus leads to second branchial cleft cyst, sinus, or fistula.
submandibular space. Rarely this cyst may “beak” to
bony-cartilaginous junction of EAC.
Second branchial cleft cyst is the most common of
all branchial cleft anomalies comprising up to 95% of
them. Bailey classified second branchial cleft cysts
into 4 subtypes (Table 1).10 They commonly occur at
the level of angle of mandible (Fig 6). Third branchial
cleft cyst is the second most common posterior
triangle area neck mass after cystic hygroma. How-
ever, it could rarely present as a cyst anterior to
sternocleidomastoid in the lower neck. Fourth bran-
chial apparatus anomaly usually presents as recurrent
FIG 5. Schematic representation of type I (periauricular) and II
(periparotid) first branchial cleft cysts in relation to the EAC, pinna,
and parotid gland.
58
suppurative thyroiditis particularly in the left lobe.
Surgical excision is the recommended therapy of
choice for this lesion.11
Imaging
The best diagnostic clue for first branchial cleft cyst
is cystic mass around the pinna (type I) or extending
from the EAC to the angle of mandible (type II). On
US, an uncomplicated first branchial cleft cyst appears
as anechoic mass in the periauricular or periparotid
area. It may show fine to coarse internal echoes in it
with or without thickening of cyst wall when there is
associated infection of the cyst. On CT, a first
branchial cleft cyst appears as a well-circumscribed
low-density mass either in the superficial or deep lobe
of the parotid gland (Fig 7). Cyst wall thickness and
enhancement are variable and increase with recurrent
infections. Induration surrounding the mass suggests
infection. Two types of first branchial cleft cyst have
been described—type I, in which the cyst is located in
close proximity to EAC (either anterior, inferior, or
posterior to EAC) and often runs parallel to it.
Sometimes this lesion may “beak” toward the bony-
cartilaginous junction of EAC. Type II cysts are
usually found in the superficial parotid or even in
the parapharyngeal space and may even extend
inferiorly up to posterior submandibular space. Rarely
this cyst may “beak” to bony-cartilaginous junction of
EAC. On MRI, the noncomplicated cysts have low
intensity on T1 and high intensity on T2 with no wall
enhancement on T1 postcontrast MRI. In the setting of
previous or current infected cyst, wall may show
thickening and enhancement. Contrast-enhanced CT
or MRI is recommended for evaluation of first
branchial cleft cyst. Sometimes US may also be used.
On US, a second branchial cleft cyst is seen as a
well-circumscribed, round to ovoid, anechoic mass
with a thin peripheral wall with or without septations
in it, which displaces the surrounding soft tissues. The
mass is compressible and shows distinct acoustic
enhancement. Occasionally, it may be hypoechoic
with fine, indistinct internal echoes, representing
debris, or may even have “pseudosolid” appearance
(Fig 8A). There may even be thickening of the cyst
wall if infected. On CT, these cysts are typically well-
circumscribed, low-density masses surrounded by a
uniformly thin wall.12 The mural thickness may
increase after infection. Following contrast adminis-
tration, they do not show any wall enhancement unless
infected. Another sign for infected cyst is fat stranding
Curr Probl Diagn Radiol, March/April 2014
TABLE 1. Bailey classification of second branchial cleft cysts
Type I Anterior to sternocleidomastoid muscle (SCM) and beneath
the platysma muscle.
Type II Most common subtype. It is classically located along the
anterior surface of SCM, lateral to carotid space and
posterior to the submandibular gland.
Type Extends medially between the bifurcation of the internal and
III external carotid arteries to the lateral pharyngeal wall.
Small projection of the cyst between ICA and ECA at
bifurcation called the “beak sign,” which is pathognomonic
for this cyst.
Type Lies against the pharyngeal wall and may extend to skull
IV base.
surrounding the cyst. The “classic” location of the
Bailey type II cyst (on either CT or MRI) is at the
anteromedial border of the sternocleidomastoid
muscle, lateral to the carotid space, and at the
posterior margin of the submandibular gland (Fig
8B). The cyst typically displaces the sternocleidomas-
toid muscle posteriorly or posterolaterally, pushes the
vessels of the carotid space medially or posterome-
dially, and displaces the submandibular gland ante-
riorly. Bailey Type III second branchial cleft cyst may
sometime demonstrate a small projection of the cyst
FIG 6. Schematic representation of the expected tract of second
branchial cleft cyst extending from the tonsillar fossa (not shown)
superiorly and traversing between the internal carotid artery (ICA) and
the external carotid artery (ECA), deep to sternocleidomastoid (SCM)
muscle to the lower third of the neck between 2 heads of SCM.
Hypoglossal nerve (HG) lies superior and glossopharyngeal nerve (GP)
is inferior to the tract. (Color version of figure is available online.)
Curr Probl Diagn Radiol, March/April 2014
FIG 7. Axial contrast-enhanced CT image of the neck demonstrates
work type II (periparotid) first branchial cleft cyst in the superficial lobe
of left parotid gland (curved arrow) with slight extension into the
deep lobe.
between internal carotid artery and external carotid
artery at the bifurcation called the “beak sign,” which
is pathognomonic for this cyst.13 On MRI, the
uncomplicated cyst usually has low intensity on T1
and high intensity on T2 with no peripheral enhance-
ment. Complicated cysts may demonstrate septation,
thickening, and enhancement of the cyst wall. “Beak
sign” can also be demonstrated on MRI in type III
second branchial cleft cyst.
A third branchial cyst most commonly appears as a
unilocular cystic mass centered in the posterior
cervical space (Fig 9) and the fourth branchial cyst
in the region of left lobe of thyroid gland on US, CT,
and MR images. There may also be wall thickening
because of previous or current infection (Fig 10).
There may be wall enhancement and surrounding fat
stranding on postcontrast CT and MRI. As with other
branchial cleft cysts, the cyst fluid may vary in signal
intensity on T1-weighted images depending on the
protein concentration and is typically hyperintense
relative to muscle on T2-weighted images.
Histopathology
Branchial cysts are usually lined by squamous
epithelium (90%) or ciliated columnar epithelium
(8%) and rarely by both types of epithelium (2%)
occasionally present are salivary tissue, seba-
ceous glands, and cholesterol clefts with a foreign
body reaction. Lymphoid tissue is generally present
(Fig 11).14
59
FIG 8. Type II second branchial cleft cyst. (A) Transverse section US of upper neck demonstrates a thin-walled, hypoechoic Bailey type II second
branchial cleft cyst with internal echoes related to debris within it. Color Doppler show medially displaced vessels in the carotid sheath (solid arrow).
(B) Axial contrast-enhanced CT image of the neck demonstrates a “classic” location of the Bailey type II second branchial cleft cyst at the
anteromedial border of the sternocleidomastoid muscle (bent arrow), lateral to the carotid space (curved arrow), and at the posterior margin of the
submandibular gland (straight arrow). The cyst displaces the sternocleidomastoid muscle posterolaterally, the vessels of the carotid space
posteromedially, and the submandibular gland anteriorly. (Color version of figure is available online.)

C. Cystic Hygroma (Lymphangioma) and constitutes approximately 5% of all benign tumors

Embryology and Pathogenesis
There are 4 types of lymphangiomas in the neck,
capillary, cavernous, cystic, and venolymphatic forms.
Of all these forms, cystic hygroma (cystic lymphan-
gioma) is the most common form of lymphangioma
of infancy and childhood.13 Cystic hygroma is thought
to arise from an early sequestration of the embryonic
lymphatic channels.15,16 This sequestration occurs
more commonly in the developing jugular lymph sac
pair than in the other 4 embryonic sites of the

FIG 10. Coronal contrast-enhanced CT image of the neck demon-

FIG 9. Axial contrast-enhanced CT image of the suprahyoid neck
demonstrates a well-circumscribed cystic lesion in the right posterior
cervical space. Histologically, this was verified as a branchial cleft
cyst. Location of the cyst in posterior cervical space suggests the
diagnosis of third branchial cleft cyst.
strates an abscess with enhancing and irregular walls, with its
epicenter in the upper pole of the left lobe of thyroid gland (curved
arrow). Significant mass effect and fat stranding is also seen.
Histologically, it was proved to be an infected fourth branchial
apparatus cyst.

60 Curr Probl Diagn Radiol, March/April 2014


FIG 11. Histopathological section (H&E, 4) shows that the epithelial
lining in this cyst transitions from stratified squamous to pseudostrati-
fied ciliated columnar epithelium. (Color version of figure is available
online.)
lymphatic system. Alternatively, a cystic hygroma
may arise from a failure of the juguloaxillary lym-
phatic sac to drain into the internal jugular vein,
producing a congenital obstruction of lymphatic
drainage.17
Clinical Features
Cystic hygroma is the most common cystic neck
mass involving posterior cervical space (75%-80%).
The other common site in children is the oral cavity.
Majority (80%-90%) of these lesions are detected by
2 years of age, which is also the age of greatest
lymphatic growth. Only a few cases occur in young
adults. The cystic hygroma most often manifest as
painless, soft, or mildly firm masses in the neck with
extremely variable size. Rarely death may result
owing to airway compromise related to extrinsic
pressure on the airway due to very large mass.
Treatment includes complete surgical excision, which
is difficult owing to infiltrative nature of the cyst with
a recurrence rate of approximately 15%. Prenatal
detection of cystic hygroma in the posterior neck soft
tissues can mimic amniotic fluid. Other markers for
aneuploidy should be looked for to exclude Turner
syndrome and trisomies 21, 18, and 13. There may
also be associated nonimmune hydrops and cardio-
vascular anomalies.
Imaging
On US, most cystic hygromas are multilocular
predominantly cystic mass with septa of variable
thickness. The echogenic portions of the lesion
correlate with clusters of small, abnormal lymphatic
channels.18 Sometimes fluid-fluid levels can be dem-
onstrated with a characteristic echogenic, hemorrhagic
component layering in the dependent portion of the
lesion. Prenatal US may demonstrate a cystic hygroma
in the posterior neck soft tissues. Size may be variable,
and large cysts mimic amniotic fluid. Other markers
for aneuploidy should be looked for to exclude

FIG 12. Cystic hygroma in a child and adolescent. (A) Coronal CT image in an adolescent patient demonstrates an infiltrative, nonenhancing,
cystic hygroma in the sublingual space (straight arrow). (B) Coronal contrast-enhanced CT image of the neck demonstrates a cystic hygroma, which
is a poorly circumscribed, multiloculated, low-density infiltrative, nonenhancing mass occupying the entire right neck with extension into the right
axilla (straight arrow) and also just extends into the superior mediastinum (arrow head). Airway is compromised, and it is grossly deviated to the
left. Endotracheal tube (curved arrow) is seen in situ. (C) Fat-suppressed axial T2-weighted MRI of the neck demonstrates a cystic, multiloculated,
infiltrative cystic hygroma. Fluid levels (straight arrows) are seen indicating hemorrhage in the cyst.

Curr Probl Diagn Radiol, March/April 2014 61

Turner’s syndrome and trisomies 21, 18, and 13.
There may also be associated nonimmune hydrops
and cardiovascular anomalies.
On CT images, cystic hygromas are poorly circum-
scribed, multiloculated, low-density masses. They typi-
cally have characteristic homogeneous fluid attenuation,
but may show increased attenuation when the fluid in it
is infected. Usually, the mass is centered in the posterior
triangle or in the submandibular space (Fig 12A). It is
not uncommon for some of these lesions to extend from
one space in the neck into another because of their
infiltrative nature (Fig 12B).19
Owing to higher soft tissue resolution, MRI best
demonstrates the relationship of a cystic hygroma to
adjacent soft tissues of the neck. The most common
pattern is that of a mass with low or intermediate signal
intensity on T1 and hyperintensity on T2WI. Rarely, this
lesion may be hyperintense on T1-weighted images,
which may be related to hemorrhage or high lipid
(chyle) content. In the case of hemorrhage, fluid-fluid
levels may be observed (Fig 12C).16
Histopathology
Cystic hygroma consists of multiple thin-walled
cysts lined by endothelial cells (Fig 13) with minimal
intervening connective tissue stroma supporting it and
filled by chylous fluid. All 4 types, capillary, cav-
ernous, cystic, and venolymphatic forms, are believed
to be manifestations of the same disease process. All 4
types can be seen in the single lesion at histologic
examination.
FIG 13. Histopathological section (H&E, 10) shows the cystic
hygroma with variably sized lymphovascular channels lined by bland
flattened endothelium and scant smooth muscle set within fibroadipose
tissue. Mononuclear cells can be seen in the lumen of some channels.
(Color version of figure is available online.)
62
D. Floor of the Mouth Cyst (Dermoid and
Epidermoid)
Embryology and Pathogenesis
The dermoid cysts are more common than epider-
moid cysts in the head and neck region. The dermoid
cysts typically occur along embryonic lines of fusion
suggesting that they exist because of entrapment of
epithelial elements during development. Lateral eye-
brow is the most common location for head and neck
dermoid, and the floor of the mouth is the second most
common location.20
Clinical Findings
Dermoid cysts usually manifest during the second
and third decades of life; however, the epidermoid
cysts appear much earlier, with most lesions evident
during infancy. There is no gender predilection.
Dermoids are more common than the epidermoids in
the floor of the mouth. The most common clinical
appearance of a dermoid cyst in the neck is a slow-
growing, painless, midline, subcutaneous or submu-
cosal suprahyoid neck mass (85%-90%). Other rare
presentations include dysphagia and airway encroach-
ment owing to large size. Rapid growth may occur
secondary to a sudden increase in desquamation,
during pregnancy, or in association with a sinus tract.
Approximately 5% of dermoid cysts undergo malig-
nant degeneration into squamous cell carcinoma.14
Location of the cyst with respect to the mylohyoid
muscle is important for correct surgical approach.
Imaging
US is useful for evaluation of superficial lesions.
They tend to have “pseudosolid” appearance; how-
ever, presence of posterior acoustic enhancement is
usually the clue for a cystic lesion (Fig 14A). Dermoid
cyst appears as moderately thin-walled, unilocular
masses, located in the submandibular or sublingual
space. It may show mixed internal echoes owing to fat
or shadowing posterior to hyperechoic calcifications.
Contrast-enhanced CT is the best diagnostic modal-
ity for evaluation of these lesions, unless patient has
dental amalgam resulting in significant streak artifacts,
in which case, MRI is preferred. On CT scans, they
usually appear as well-circumscribed, low-density,
unilocular mass in the floor of the mouth. Dermoid
may show fatty material in it, which may appear as
“sac of marbles,” owing to the coalescence of fat into
small nodules within the fluid matrix (Fig 14B)21 and
Curr Probl Diagn Radiol, March/April 2014
FIG 14. US, CT, and MRI manifestation of the floor of the mouth dermoid. (A) Transverse section US of the floor of the mouth dermoid cyst
demonstrates a well-circumscribed “pseudosolid”-appearing mass with multiple rounded echogenic lesions in it (arrowheads) giving appearance of
“sac of marbles,” which is virtually pathognomonic for a dermoid cyst in this location. (B) Axial contrast-enhanced CT image of the upper neck
demonstrates a unilocular, well-circumscribed, cystic lesion in the floor of the mouth region. Multiple tiny hypoattenuating areas in the cyst are due to
fat globules [sac of marbles] (arrows), favoring dermoid cyst. (C) Coronal fat-suppressed postcontrast T1-weighted MR image demonstrates a
unilocular, well-circumscribed, nonenhancing epidermoid cyst in the floor of the mouth. Compared with CT, relation to the mylohyoid muscle (arrow)
is very well demonstrated owing to higher soft tissue resolution of MRI.

sometimes even calcifications (o50%). This “sack-of- soft tissue resolution, MRI depicts the topographic
marbles” appearance is virtually pathognomonic for a relationship of these cysts to the mylohyoid muscle in
dermoid cyst in this location. Postcontrast adminis- the floor of the mouth and helps determine the surgical
tration may show subtle rim enhancement. Epidermoid approach. The coronal plane is optimal for determin-
cysts are predominantly fluid attenuation on CT. ing the location of the mass with respect to this muscle
On MRI, dermoids have more complex signal on pair.22
T1 when compared with epidermoids. On T2WI,
dermoids show heterogeneous hyperintense signal
whereas the epidermoids show uniform increased
signal intensity. Postcontrast administration may show
subtle peripheral enhancement (Fig 14C). Fat-
suppression T1 sometime show low signal in the
dermoid because of fat content in it. Owing to higher

FIG 16. Schematic diagram representing thymic cyst along the

FIG 15. Histopathological section (H&E, 4) shows the dermoid cyst course of thymopharyngeal duct extending from the level of angle of
lined by a thin layer of keratinizing stratified squamous epithelium with mandible (not shown) to the superior mediastinum commonly on left
a granular layer and variably attenuated adnexal structures. (Color side, lateral to the thyroid gland (TG). (Color version of figure is
version of figure is available online.) available online.)

Curr Probl Diagn Radiol, March/April 2014 63


FIG 17. Coronal CT image of the neck shows a multiloculated, well-
circumscribed and nonenhancing thymic cyst in the left neck along the
expected course of thymopharyngeal duct with extension into the
superior mediastinum (curved arrows).
An epidermoid cyst located entirely within the
sublingual space may be difficult to distinguish from
other cystic lesions in the floor of the mouth (eg, a
simple ranula) based on conventional imaging criteria
alone. Evaluation of diffusion-weighted images would
be helpful. Epidermoid cysts may show diffusion
restriction, although the apparent diffusion coefficient
values are typically only moderately low compared
with the lower apparent diffusion coefficient values of
intracranial epidermoids.23
Histopathology
Dermoid and epidermoid cysts show fairly similar
histologic appearance except for the lack of dermal
appendages (sebaceous glands, hair follicles, and
sweat glands) in epidermoid cyst, which is seen in
the dermoid cyst (Fig 15).23
E. Cervical Thymic Cyst
Embryology and Pathogenesis
The third branchial pouch forms the thymus and
inferior parathyroid glands during fetal development.
The thymus descends down to the mediastinum
through thymopharyngeal duct, traveling lateral to
the thyroid gland. The cervical thymic cyst arises
along this tract (Fig 16). The exact pathogenesis of
cervical thymic cysts remains controversial even
today. Two mechanisms (congenital and acquired)
are proposed. Most authorities favor the congenital
64
persistence of the thymopharyngeal duct remnants as
the cause of these lesions. Alternatively, some inves-
tigators believe that they result from acquired, pro-
gressive cystic degeneration of thymic (Hassall)
corpuscles and the epithelium reticulum of the thy-
mus.24 Whatever is the cause, the presence of thymic
tissue within the lesion is required for pathological
diagnosis.
Clinical Findings
About two-thirds of the cases occur in the first
decade of life and remaining one-third occur after the
first decade. There is slight male predominance and
usually involve left side of the neck.24 Most of the
thymic cysts are asymptomatic. The diagnosis is
seldom suspected preoperatively, most likely because
of the rarity of the lesion.25 Cervical thymic cysts can
be found anywhere along the path of the thymophar-
yngeal duct, immediately adjacent to the carotid
sheath from the angle of the mandible to the thoracic
inlet. They can present as gradually enlarging, soft,
compressible midlower cervical mass, which may
extend into the mediastinum. When large, they can
present with compressive symptoms. Rarely there may
be associated abnormal calcium metabolism, if there is
a functional parathyroid component. Complete surgi-
cal resection is the treatment of choice. Recurrence
rate is approximately 2% if incompletely resected.26
Imaging
On US, a thymic cyst appears as a large, usually
unilocular cystic mass extending downward, parallel
FIG 18. Histopathological section (H&E, 4) shows the interstitium of
the thymus expanded by multiple irregular cystic spaces lined by both
simple and stratified cuboidal epithelium. (Color version of figure is
available online.)
Curr Probl Diagn Radiol, March/April 2014
 F. Cervical Bronchogenic Cyst
Embryology and Pathogenesis
Cervical bronchogenic cysts are extremely rare.
Anomalous foregut development is suspected to play
a role in the development of bronchogenic cyst, but the
reason why these cysts reach an aberrant position in
the neck remains unclear.28 They have been reported in
infants as well as in adults and occur in males
approximately 3 times as often as in females.14,29

Clinical Findings
Extrathoracic bronchogenic cyst usually is located in
the suprasternal notch, presternum, shoulder, neck, base
FIG 19. Sagittal contrast-enhanced CT image demonstrates a well- of the tongue, infraclavicular region, or chin and can
circumscribed, uniloculated, hypodense cystic mass in the sternal notch extend into the mediastinum.27 Coexisting congenital
region anterior to trachea. Histologically, this lesion was proved to be sublingual dermoid and bronchogenic cysts and cysts
a bronchogenic cyst in an atypical location.
with features of bronchogenic, thyroglossal, and bran-
to the sternocleidomastoid muscle. It may have some chial cleft origin have been reported.30,31 Subcutaneous
solid components in it. bronchogenic cysts are detected shortly after birth or in
On CT, the appearance is that of a uniloculated or early childhood as an asymptomatic neck mass or a
multiloculated, hypoattenuated cystic mass adjacent to draining sinus.32 Rarely, the cyst is diagnosed in an
the carotid space.27 The mass may extend into the adult. The cyst may fluctuate in size but usually
mediastinum (Fig 17). No enhancement is evident on enlarges with body growth. Local traction or compres-
contrast administration in an uncomplicated thymic sion on adjacent structures can cause symptoms of
cyst. Rarely there may be enhancing solid components dyspnea, cyanosis, or dysphagia.33 A neck abscess can
in it, likely representing the aberrant thymic tissue, also occur if the cyst becomes infected.34
lymphoid aggregate, or parathyroid tissue.
On MRI, the thymic cysts are usually hypointense on Imaging
T1, and sometimes isointense to hyperintense depend- On US, a cervical bronchogenic cyst appears as
ing on hemorrhage in the cyst of protein content of the an anechoic cystic mass usually in the sternal
fluid. It is predominantly hyperintense on T2. Most of notch region. On CT, the appearance is that of a
them are nonenhancing on postcontrast T1, except for
few cases which can demonstrate mildly enhancing
solid nodule owing to aberrant thymic tissue, lymphoid
aggregates, or parathyroid tissue. CT or MRI is
preferred over US, as the later cannot adequately access
the mediastinal component of the cyst.
Histopathology
Most cervical thymic cysts contain light amber to
dark brown fluid. The cyst wall may be composed of a
broad spectrum of epithelium, ranging from flattened
squamous or cuboidal cells to multilayered stratified
squamous epithelium to even primitive respiratory
epithelium (Fig 18). Lobulated lymphoid tissue in
the cyst wall contains Hassall corpuscles. Less com-
FIG 20. Histopathological section (H&E, 4) shows the bronchogenic
monly, small foci of thyroid or parathyroid tissue are
cyst lined by ciliated columnar epithelium with smooth muscle bundles
noted and emphasize the embryologic association of in the wall, seromucinous glands, and foci of hyaline cartilage. (Color
the thymus with these structures. version of figure is available online.)

Curr Probl Diagn Radiol, March/April 2014 65

TABLE 2. Classic diagnostic imaging features of cervical cysts
Thyroglossal duct cyst Close relation to hyoid bone or within strap
muscles when infrahyoid.
First branchial cleft cyst Close relation to parotid gland/EAC.
Second branchial cleft Type II in between submandibular salivary
cyst gland and sternocleidomastoid (SCM)
muscle. Other subtypes (rare) can be
anywhere from beneath the superficial
platysma muscle to tonsillar fossa of lateral
pharyngeal wall.
Third branchial cleft Posterior cervical space cyst, can present as a
cyst cyst anterior to SCM in the lower neck.
Fourth branchial Left lobe of thyroid cyst/abscess. (Both third
apparatus anomaly and fourth branchial sinuses could be
connected to pyriform sinus).
Cystic hygroma Most common posterior cervical space cyst,
usually large, poorly circumscribed, and
multiloculated.
Floor of mouth “Sac of marbles” fatty content pathognomonic
dermoid/epidermoid for dermoid cyst.
Cervical thymic cyst Uniloculated or multiloculated cystic lesion
extending from the angle of mandible along
the expected course of the thymopharyngeal
duct into the superior mediastinum.
Differential diagnosis includes third
branchial cleft cyst and cystic hygroma.
Cervical bronchogenic Extremely rare aberrant location in
cyst suprasternal notch, presternum, shoulder,
neck, base of the tongue, infraclavicular
region, or chin.
well-circumscribed, uniloculated or multiloculated,
hypoattenuated cystic mass in the sternal notch region
anterior to trachea (Fig 19). On MRI, the brochogenic
cysts are usually hypointense on T1, and sometimes
isointense to hyperintense depending on hemorrhage
in the cyst of protein content of the fluid. It is
predominantly hyperintense on T2. Most of them are
nonenhancing on postcontrast T1 unless infected, in
which case, it can show peripheral wall enhancement.
Histopathology
Cervical bronchogenic cyst is commonly lined by
columnar, ciliated, pseudostratified epithelium (Fig
20). Blood vessels, hyaline cartilage, smooth muscles,
seromucinous glands, and elastic fibers are also
variably present in the cyst wall. Rare squamous
metaplasia has been reported in cysts that have been
previously infected.35
Conclusion
Clinical evaluation including both history and physical
examination is important in the evaluation of a
suspected congenital neck mass. Most common
66
presentation includes fluctuant slow-growing neck
masses, and sometimes presents with repeated infec-
tions, especially the branchial cleft cysts. US and CT
are the most common imaging modalities used with
MR evaluation for more complex lesions (Table 2). A
systematic approach based on the knowledge of
embryology and anatomy of the cervical region allows
to arrive at a specific diagnosis or at least narrow the
differential diagnosis.
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