J Cutan Pathol 2007: 34: 815–829 Copyright # Blackwell Munksgaard 2007

doi: 10.1111/j.1600-0560.2007.00746.x
Blackwell Munksgaard. Printed in Singapore Journal of
Cutaneous Pathology

Perspectives in Dermatopathology

Dermal cysts: a dermatopathological

perspective and histological reappraisal
Dermal cysts constitute an extremely common entity routinely Joseph R. Jenkins1 and Michael
encountered in dermatology and dermatopathology practice. Their B. Morgan2,3
ubiquity, overlapping clinical presentation, dermal location, and 1
Department of Pathology, University of South
histologic diversity can engender diagnostic quandary. Though Florida College of Medicine, Tampa, FL, USA,
2
basically defined by the histologic presence of an epithelial lining, cysts Department of Pathology, University of South
derive from a variety of sources including developmental defects, Florida College of Medicine, Tampa,
FL, USA, and
trauma, and tumoral degeneration. Herein, we will discuss the 3
Department of Pathology, James A. Haley VA
dermatopathologic attributes of the more common dermal cyst Hospital, Tampa, FL, USA
entities, updating the most recent and pertinent literature. Joseph R. Jenkins
Department of Pathology, University of South
Florida College of Medicine, and James A. Haley VA
Hospital, 12901 Bruce B. Downs Boulevard,
Tampa, FL 33612, USA
Tel: 11 813 972 2000 ext. 6473
Jenkins JR, Morgan MB. Dermal cysts: a dermatopathological Fax: 11 813 903 4813
e-mail: [email protected]
perspective and histological reappraisal.
J Cutan Pathol 2007; 34: 815–829. # Blackwell Munksgaard 2007. Accepted for publication January 23, 2007

Dermal cysts constitute an extremely common entity and tumoral degeneration. Herein, we discuss the
routinely found in dermatology and dermatopathol- dermatopathological attributes of the more common
ogy practice. Their ubiquity, overlapping clinical dermal cyst entities, updating the most recent and
presentation, dermal location and histological diver- pertinent literature.
sity can engender diagnostic quandary. Although Each discussed entity follows categorization based
basically defined by the histological presence of an on the particular mechanisms of pathogenic deve-
epithelial lining, cysts derive from a variety of lopment and microanatomic location, shown in the
sources including developmental defects, trauma schematic below.

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Jenkins & Morgan
Cysts of follicular differentiation
The follicular unit comprises the most important
source of dermal cysts, with named cysts derived
from every portion of the structure.

Infundibular
Synonyms
Epidermal inclusion cyst (EIC), sebaceous.

Fig. 1. Low-power view of an infundibular cyst arising in conjunc-

tion with Gardner’s syndrome.

a localized granulomatous reaction, with heavy

inflammatory cell infiltration in the adjacent dermis.
Fibrosis usually follows resolution of the inflammation.
Cultures of infected cysts have grown Staphylococcus
aureus and other common skin flora, but cultures of both
Description inflamed and non-inflamed cysts have grown similar
It is the most common type of cyst. Infundibular organisms, indicating an alternate cause of inflamma-
cysts present as solitary, slow-growing lesions, found tion probably engendered by emancipated squamous
mainly on the face, neck and trunk and usually cells of the epithelial lining when ruptured and exposed
measuring no more than 5 cm in diameter, although to the dermis and subcutaneous fat.
larger variants have been identified.1 They are
grossly identified by a raised nodule on the surface of Histopathology
the skin, often accompanied by a surface punctum. Infundibular cysts are lined by stratified squamous
They most commonly arise in the mid- to lower epithelium with epidermal keratinization, thus
dermis and do not shell out easily at removal. supporting a probable origin from the infundibular
Morphological variants such as sprouting appen- portion of the hair follicle. These cysts express
dages arising from the main cyst, tuber-shaped cysts keratin 106 and have been shown to exhibit
and gigantic (up to 17 3 20 cm) cysts have been varied histological forms of the epithelial lining,
reported in the literature,2 as well as concomitant including basal hyperpigmentation in black patients,
conditions such as Bowen’s disease, Paget’s disease
and nevi.3 Although rare, occurrences of Merkel cell
carcinoma,3 basal cell carcinoma4 and squamous
cell carcinoma5 have been reported in conjunction
with infundibular cysts.
Infundibular cysts derive from the infundibulum of
the pilosebaceous follicle. The traumatic variant is
found most often on the palms and soles and also in
the subungual regions. To be specific, the traumatic
variant is most probably the Ôtrue’ form of infundib-
ular cyst because the trauma may cause the insertion
of epidermal tissue into the dermis, that is an ÔEIC’.
Even though most patients present with a single cyst,
multiple cysts can be seen sporadically or in conjunc-
tion with Gardner’s syndrome (Figs. 1 and 2). The
histological features of these cysts often show matricial
differentiation (to be discussed forthcoming). Cysts
may become infected and rupture into the dermis or Fig. 2. High-power view of a Gardner’s-associated infundibular cyst
occasionally onto the surface of the skin, inducing showing keratinization pattern.

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 Dermal cysts
melanophagic proliferation, seborrheic keratosis-like
changes, focal epidermal proliferation and hyper-
keratosis. The inspissated contents are characteris-
tically laminated or basket weave in configuration.
Inflammatory cells, red blood cells and cholesterol
clefts may also be seen. Lesions suspected to be cysts
should always be submitted for histological exami-
nation, as one study showed that 75% of tissues
submitted as infundibular cysts were confirmed as
benign keratinizing cysts, whereas the remainder
was of varying histological forms, with 2.7%
confirmed as malignancies.7
Description
Vellus hair cysts may present as a solitary papule or
Human-papillomavirus-related infundibular occur as discrete reddish-brown papules, usually 1–4
Description mm in diameter, most often on the chest and axillae
of children and young adults and less often on the
Two distinct but rare types of infundibular cyst are neck, face and extremities.12 They can either have
associated with human papillomavirus (HPV) infec- a sporadic presentation or show autosomal domi-
tion. The first type reported can be found on nant inheritance and may spontaneously regress
pressure points of the plantar surface of the foot3 following elimination or aspiration of cyst contents.
and is associated most commonly with HPV The abrupt onset of cysts consistent with eruptive
serotype 60.8 Multiple lesions are rarely seen. The vellus hair cysts has been reported in conjunction
cyst wall can often be seen containing eccrine ductal with steatocystoma, suggesting a common etiology.
structures, suggesting HPV infection of eccrine ducts Although they have been postulated to derive from
in the pathogenesis of such cysts. Recent immuno- within the sebaceous duct, the pattern of keratin
staining studies have shown definitive proof of the expression is different between the two types: vellus
presence of HPV-60.9 hair cysts express only K17, whereas steatocystomas
The second type involves verrucous changes in express both K17 and K10.13 Most believe that
the epithelial lining of cysts in a non-palmoplantar these pathologically derive as developmental cysts
location and is seen much less commonly. Recent from the infundibulum of vellus follicles. They have
studies have shown HPV-60 to be responsible for been reported in conjunction with basal cell
these lesions as well.10 There have also been reports carcinoma in older adults14 and possibly in con-
of HPV-related tricholemmal cysts, although a spe- junction with neurological abnormalities in an
cific serotype was not identified.11 undisclosed neurocutaneous syndrome.15
Histopathology Histopathology
HPV-60-associated cysts present as well-demarcated Vellus hair cysts are small, upper-to-mid dermal
dermal inclusions, with epidermis overlying the top inclusions lined by stratified squamous epithelium,
of the cyst. Scattered keratinocytes can be seen in
the upper layers, exhibiting intracytoplasmic, eosi-
nophillic inclusions and vacuolar structures within
the keratin contents of the cyst. Parakeratosis can
usually be seen in the keratinous material.8
The other variant of HPV-related cysts is the
verrucous cyst (Figs. 3 and 4), which is lined by a
papillated epithelium possessing irregular and coarse
keratohyaline granules and hypergranulosis. Squa-
mous eddies and papillary lumenal projections may
be seen within the wall structure.3

Vellus hair
Note: The vellus hair and follicle, although similar in
configuration to the fully developed terminal hair
and follicle, are much smaller. The image below Fig. 3. Low-power view of the verrucous variant of a human-
should be used as a point of spatial reference only. papillomavirus-associated infundibular cyst.

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Jenkins & Morgan

Fig. 4. High-power view of the projections in the wall of the Fig. 6. High-power view of the vellus hairs contained within a vellus
verrucous cyst in Fig. 3. hair cyst.

which can exhibit tricholemmal and/or infundibular skeletal and neurological anomalies. They most
keratinization patterns (Fig. 5).16 The lumen of the often resemble infundibular cysts but have been
cyst contains numerous small hairs (Fig. 6), which seen to contain brown purulent material unusual for
are doubly refractile under polarized light and may infundibular cysts. There have been reports of
contain a rudimentary hair follicle connected to the cutaneous keratocysts arising independently of basal
cyst wall. Focal rupture of the cyst wall may cause cell nevus syndrome.17
a localized granulomatous reaction, mild inflamma-
tion and dermal fibrosis. Spontaneously regressing Histopathology
vellus hair cysts usually possess a connecting pore to
the surface skin, thought to contribute to their Cutaneous keratocysts present with a festooned
lining continuous with a thick stratified squamous
regression.
wall that lacks a granular layer. There is a superficial
resemblance to steatocystoma multiplex (see below),
but sebaceous glands and lobules are typically
Cutaneous keratocyst
lacking.18
Description
Cutaneous keratocysts as a component of the basal
cell nevus syndrome are characterized by multiple Pigmented follicular cysts
basal cell carcinomas with early age of onset, Description
odontogenic keratocysts, palmoplantar pits and
Pigmented follicular cysts are rare, usually singular
lesions marked by small, grossly pigmented cysts and
papules that can present nearly anywhere on the
body.19 Multiple cysts have also been rarely
reported, as well as cysts in conjunction with
melanocytic nevi.20

Fig. 5. Low-power view of a vellus hair cyst.

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 Dermal cysts
To clarify, early publications considered pig-
mented follicular cysts to be follicular cysts with
melanin pigment contained within the epithelium,21
whereas most current usage of the term pertains to
follicular cysts with pigmented hair shafts.22–24 It is
this latter convention that is used here.

Histopathology
The cyst is confined to the dermis and is distinctive
by virtue of its contents, which contain layered
keratin and multiple pigmented pilar shafts that may
show degeneration (Figs. 7 and 8). If such degener-
ation or pigmentation is overlooked on histological
examination, the cyst may be misdiagnosed as an
infundibular cyst.23 A stratified squamous epithe-
lium containing rete ridges and papillae may also be Fig. 8. High-power view of the pigmented follicular cyst in Fig. 7
seen. Epidermal and tricholemmal keratinization showing melanin in the cyst wall.
patterns have been described when multiple cysts
are present.25 mutations in K17 similar to pachyonychia conge-
nita.28,29 Pachyonychia congenita type 2, also known
as PC-2 or Jackson-Lawler syndrome, is an auto-
Steatocystoma somal dominant disorder characterized by hyper-
Description trophic nail dystrophy, mild focal keratoderma,
Steatocystoma is a condition notable for single or multiple pilosebaceous cysts and other features of
multiple yellow to skin-colored cysts and papules in ectodermal dysplasia. K17 mutations underlie both
varying sizes ranging from less than 0.5 cm to more PC-2 and steatocystoma, and the alternate expres-
than 3 cm. They are most commonly found on the sion of phenotype seems to be independent of the
scalp, face, axillae, trunk and extremities, with specific mutation involved.30
a greater occurrence on the chest. Steatocystomas
are probably nevoid malformations arising from the
pilosebaceous duct and are usually sporadic, but
heritable cases are well documented (steatocystoma
multiplex).26 The cysts in this setting usually present
asymptomatically in adolescents but have shown to
become infected and painful in a limited percentage
of cases, at which time they are known as
steatocystoma multiplex suppurativum.27 Steatocys-
tomas express K10 and K17, with occasional

Histopathology
A thin layer of squamous epithelium (often only 1–3
cells thick) with a characteristic dense cuticle on the
surface of the epithelium and devoid of a granular
layer lines these cysts and is characteristically
undulating as a result of collapse of the cyst
(Fig. 9).26 A pathognomonic finding is the existence
of sebaceous glands of varying size within the wall of
the cyst (Figs. 10 and 11), with a cord of epithelial
cells communicating with the epidermal surface.
The cord of epithelial cells may or may not be seen
depending on the level of the cut of the specimen.
Each individual cyst is associated with a single
Fig. 7. Low-power view of a pigmented follicular cyst. pilar unit but may contain multiple, small lanugo

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Jenkins & Morgan

Fig. 9. Low-power view of a steatocystoma. Note the tortuosity of Fig. 10. High-power view of the wall of a steatocystoma. Notice the
the cyst wall and a sebaceous gland. characteristic thick cuticle on the surface of the epithelium.

hairs similar to a vellus hair cyst, with which there is female predilection of 84% has been shown.34
a documented relationship.29 Occasional large, Multiple pilar cysts are common, often occurring
granular, polygonal cells consistent with monocyte/ on the scalp of adult women.
macrophage etiology can be found in the cyst lining, Malignant transformation is most often seen in
as well as smooth muscle cells contained within the the setting of proliferating tricholemmal tumors
cyst wall.31 An interesting yet intermittent feature is (Figs. 12 and 13). Some authors believe that these
spherules of erythrocytes suspended in an oily distinctive neoplasms consisting of squamous epi-
medium within the cyst lumen.32 thelium possessing tricholemmal differentiation
should all be considered as potentially malignant.35
Others similarly regard these lesions with acceler-
Tricholemmal ated, infiltrating growth and size beyond 5 cm with
Synonyms atypia and frequent mitoses as malignant.36
Pilar, isthmus catagen, Wen. Histopathology
These cysts are lined by stratified squamous
epithelium exhibiting tricholemmal keratinization
in which the cells grow increasingly larger as the
lumen is approached from the cyst wall (Fig. 14).
There is most often a lack of keratohyalin granules

Description
Tricholemmal cysts are either solitary or multiple
intradermal or subcutaneous cystic inclusions, 90%
of which are found in the scalp but can also be found
on the skull base,33 chest, back, buttocks and genital
areas, among other locations. A punctum is absent,
and unlike the infundibular cysts, these are easily
removed during surgical extraction. They are
smooth, with a white to cream-colored wall Fig. 11. High-power view of sebaceous glands within the wall of
containing similarly colored caseous contents. A a steatocystoma.

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 Dermal cysts

Fig. 12. Low-power view of a tricholemmal cyst within a pro-

liferating tricholemmal tumor.
Fig. 14. Low-power view of a typical tricholemmal cyst.

that most closely resembles the morphology seen in between the two differing types of keratinization.
the external root sheath at the follicular isthmus Other notable types of hybrid cysts combining
(Fig. 15).37 Cholesterol clefts are commonly seen infundibular/pilomatricial or tricholemmal/piloma-
and approximately 25% will exhibit focal calcifica- tricial or a combination of vellus hair cyst with
tion of the contents. In addition to the K10 steatocystoma, infundibular or tricholemmal types
exhibited by infundibular cysts, tricholemmal cysts have been reported.38 Hybrid cysts have also been
express K17.6 A small percentage is associated with reported in conjunction with nevus sebaceus.39
inflammation.

Hybrid
Description
The hybrid cyst, as its name implies, is a combina-
tion of the features of any two dissimilar types of
follicular differentiation, most commonly the infun-
dibular and the tricholemmal, with the upper
portion resembling an infundibular cyst and the
lower portion resembling a tricholemmal cyst. A
sharp line of demarcation can be easily seen

Histopathology
Histopathology, as mentioned above, is simply the
characteristics of each individual type seen within
a single cyst (Figs. 16 and 17). There are no
histomorphological features unique to hybrid cysts.

Matricial
Description
Seen more often in children and less so in young
adults, the matricial cyst shows a histological overlap
with pilomatrixoma and as such is thought to be
a benign cyst derived from hair matrix.40 These
cysts also show a strong correlation with Gardner’s
Fig. 13. High-power view of the cyst contents within a proliferating syndrome, which is characterized by familial colonic
tricholemmal tumor. polyposis, benign osteomas and multiple cutaneous

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Jenkins & Morgan

Fig. 15. High-power view of the cyst wall from Fig. 14. The cyst Fig. 16. Hybrid cyst showing characteristics of tricholemmal and
contains dense keratin, with no underlying granular layer. matricial histology.

cysts of infundibular, sebaceous and pilomatrixoma- Milium

like origin.41 Description
Milia are small, 1- to 2-mm dermal cysts that arise
from eccrine sweat ducts. They are most commonly
seen as congenital lesions in newborns but may also
occur later in life as a result of topical steroids,
radiotherapy, ultraviolet light exposure, dermabra-
sion or following subepidermal bullous conditions
and second-degree burns.42 They mainly occur as
multiple lesions on the forehead and cheeks but can
also occur on the genitalia or elsewhere on the body,
depending on location of the precipitating lesion. A
rare eruptive form and an erythematous plaque
variant (milia en plaque) have also been reported.43
Histopathology
Several layers of palisading basaloid cells that mature Histopathology
to anucleated, eosinophilic squamous epithelium Milia are lined by several layers of stratified
toward the lumen of the cyst recapitulating the hair squamous epithelium and contain keratin within
matrix are seen. A keratinous substance fills a lumen the lumen, resembling a small infundibular-type cyst
that may be surrounded by small cystic lesions within
the cyst wall. Rupture often induces a pronounced
granulomatous reaction in the surrounding dermis.

Cysts of eccrine and apocrine differentiation

The cysts described in this section are all derived from
the eccrine or apocrine sweat glands and/or ducts.

Fig. 17. High-power view of the matricial portion of the hybrid cyst
in Fig. 16.

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 Dermal cysts
(Fig. 18). They are most often associated with an Apocrine hidrocystoma
eccrine sweat duct.44 Description
Also known as apocrine cystadenoma or simply
apocrine gland cyst, these lesions are regarded by
Eccrine hidrocystoma
most practitioners in one of two ways: either as
Description a simple retention cyst or as an adenomatous
Eccrine hidrocystomas are most often solitary lesions hyperplasia of apocrine glands. They are almost
involving the face, trunk and popliteal fossae but exclusively small, solitary lesions, merely a few
occur most prominently in the periorbital area. millimeters in diameter and most commonly found
Typically, they affect women in hot, humid environ- in middle-aged and senior adults on the head and
ments45 and are thought to be related to hyper- neck, although a large variant and multiple cysts
hidrosis.46 A variant form with multiple cysts, have been reported.51 Grossly, they resemble eccrine
sometimes numbering into the hundreds, can also hidrocystoma in that they have a similar bluish,
be found.47 These lesions are unique from pre- transilluminating hue recently attributed to the
viously covered cysts in that they are translucent, Tyndall phenomenon, wherein colloidal particles
rounded papules with a characteristic blue colora- in the fluid scatter light and appear blue.52 The
tion. Malignant transformation is possible but contents can run the spectrum from colorless to
rare.48 A pigmented variant has also been reported brown/black on aspiration.
in the literature.49 Multiple cysts are usually the result of either one
There is a slight controversy whether or not of two rare hereditary conditions: Schopf-Schultz-
eccrine hidrocystomas actually exist as many practi- Passarge syndrome – which is defined as hyperkeratosis
tioners consider these to be of the apocrine type (see palmoplantaris with hypodontia, hypotrichosis and
below), with attenuation of the epithelial lining being apocrine cysts of the eyelids53 – or in conjunction
largely responsible for the histological lack of with focal dermal hypoplasia, also known as Goltz
decapitation secretion.49 syndrome.54

Histopathology Histopathology
The cysts are solitary and located in the dermis in The cysts may be singular or multiple and typically
close relation to eccrine glands. The lining is contain an inner columnar epithelial lining with
comprised of two layers of cuboidal epithelium a flattened outer layer of elongated myoepithelial
containing eosinophilic cytoplasm, which may be cells. Decapitation secretion typical of apocrine
seen exerting into the lumen. On electron micros- glands can usually be seen, although the attenuation
copy, the two cuboidal layers can be seen to have of secretory cells previously mentioned may repre-
a peripheral basement membrane and microvilli sent eccrine hidrocystoma.55 There may be areas of
along the luminal border similar to an eccrine duct,50 epithelial hyperplasia forming connective-tissue-
with occasional layers of flattened epithelial cells that cored papillae with microcystic inclusions within
can secrete carcinoembryonic antigen (CEA).46 the papillary projections (Fig. 19).56 The secretory

Fig. 18. Low-power view of a milial cyst. Fig. 19. High-power view of an apocrine hidrocystoma.

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Jenkins & Morgan
cells may be periodic acid-Schiff (PAS) positive57 and Inflammation, if present, is minimal and largely
can occasionally be shown to be CEA positive as well. devoid of lymphoid follicles.

Developmental cysts Branchial cleft

The cysts described in this section most probably Description
derive from embryological remnants or arise from Branchial cleft defects can present in children or in
faulty ontogeny. young adults as cysts, skin tags, communicating sinus
tracts or any combination thereof and vary in
location according to which branchial cleft/pouch is
Bronchogenic involved. Anatomic locations ascribed to first
Description branchial pouch origin occur near the angle of the
Bronchogenic cysts usually present at birth and most mandible, whereas those of the second pouch are
usually occur as midline solitary masses in the often found on the anterior border of the sterno-
manubrium, chin, neck and scapular/shoulder cleidomastoid muscle and can be found at any depth
areas.58 Rare abdominal lesions have been re- between the pharynx and the skin.65,66
ported.59 They present as either a sinus or a cyst They contain cholesterol-rich turbid fluid and
and arise from sequestrated foregut-derived tracheo- may become secondarily infected, which can cause
bronchial bud cells, although a branchial origin (see inflammation and swelling of the lesions that may
below) may also be a potential cause. Cysts are misleadingly appear to be growth in the size of the
classified as either intrathoracic or extrathoracic.60 cyst itself. Squamous cell carcinoma, although rare,
Lesions are exclusively solitary, located in the dermis is a documented comorbidity in long-standing
and subcutaneous tissue and contain a milky to cysts.67
mucinous fluid, with a 4:1 male:female ratio.61
Histopathology
Histopathology Branchial cleft cysts are lined by a stratified
Bronchogenic cysts are lined by respiratory-related squamous epithelium, with deeper parts occasionally
epithelium in that it is cuboidal or pseudostratified, exhibiting ciliated columnar epithelium. A heavy
ciliated and mucin producing (Fig. 20). Stratified lymphoid infiltrate, including lymphoid follicles with
squamous epithelium may be seen on superficial germinal centers, characteristically surrounds the
areas of bronchogenic sinuses, with communication cyst wall.65
to the surface skin or if the cyst has become
infected62 and gastric-related mucosa has been seen,
consistent with its foregut etiology.63 Mucous glands Thyroglossal duct
and smooth muscle are usually found in the cyst Description
wall, with cartilage occurring much less often.64 Thyroglossal duct cysts result from the failure of
thyroglossal duct obliteration following the descent
of the thyroid in the sixth week of embryological
development and account for approximately 70% of
all congenital neck abnormalities. They usually
present as asymptomatic, 2- to 4-cm midline cervical
masses with a slight preponderance for deviation to
the left side of the trachea but can occur along any
point of the course of the thyroglossal duct
remnant.68 Most cases are sporadic, but a hereditary
form has been reported in the literature.69

Histopathology
The thyroglossal duct is frequently multiple and
branched, with the cyst being lined by a ciliated
columnar respiratory or a non-keratinized stratified
squamous epithelium or a combination of the two
epithelial types.70 Aspiration of the cyst most often
Fig. 20. High-power view of the respiratory epithelium and ciliated produces sparsely cellular smears, with a background
brush border of a bronchogenic cyst. of colloidal material that contain an abundance of

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 Dermal cysts
foamy or hemosiderin-laden histiocytes in nearly of the dorsum of the nose.81 Hair may arise from the
90% of cases. Multinucleated histiocytes can be center of this fistula.
rarely seen as well. Cholesterol crystals have been
reported in a few cases.71 Histopathology
Dermoid cysts are lined with keratinizing stratified
squamous epithelium and sebaceous glands but
Thymic may be non-keratinizing and feature conjunctival-
Description appearing goblet cells if the cyst is located near the
Thymic cysts are located in the neck or the orbital region. Sebaceous glands empty directly into
mediastinum and present as largely asymptomatic the lumen, which also contains keratinous material
swellings in children and adolescents71 and can and hair shafts. Other potential wall-bound struc-
cause respiratory difficulties depending on size and tures are eccrine/apocrine glands and smooth
location.72 They are most probably the remnants of muscle.82 The fistula in the dorsum of the nose is
the thymopharyngeal duct, which is a derivative of lined by similar structures as seen in the wall of the
the third and the fourth branchial pouches but may cyst. Granulomas may form secondary to rupture
also be associated with thymoma73 and thymic but are not commonly seen.
carcinoma.74 They usually occur on the neck just
posterior to a lateral lobe of the thyroid gland, with
a preponderance of occurrence on the left side.75 Omphalomesenteric duct
Cysts can be solitary or multiple, measuring Description
anywhere from 1 to 15 cm in diameter. The Omphalomesenteric duct (OMD) cysts arise when
contents of the lumen may contain cholesterol there is failure during the embryological stage of
crystals in a yellow to brown gelatinous substance. obliteration of the vitelline duct that connects the
midgut to the yolk sac. Such failure of obliteration is
Histopathology associated with Meckel’s diverticulum, umbilical
sinus, patent OMD and OMD cysts.83 These cysts
Thymic cysts can be lined with any combination of
are therefore found in the periumbilical area, usually
squamous, cuboidal, columnar or pseudostratified
at birth, and are exclusively solitary in nature. On
columnar epithelium and may even be devoid of an
rare occasion, this type of cyst may present initially
epithelial lining and instead be lined by simple
in adulthood.84
fibrotic tissue. Hassal’s corpuscles are almost always
present, along with lymphoid tissue, granulomas and
Histopathology
even parathyroid tissue.75 Non-cystic thymic rem-
nants have also been reported in the skin of the neck These cysts are lined by gastric, small bowel or
and upper chest areas.76 colonic mucosa, which becomes stratified squamous
epithelium toward the outer surface, and are often
open to the outside (Figs. 21 and 22). They fre-
Dermoid quently contain smooth muscle, adipose tissue and
focal calcifications and present either with or
Description
Dermoid cysts are rare, solitary, asymptomatic 1- to
4-cm masses that present at birth and are of
ectodermal origin, arising at lines of embryological
fusion, most commonly at the midline of the
forehead and the neck77 and also at the lateral
angle of the eye, floor of the mouth,78 scalp and
penis, although the latter three presentations are
exceedingly rare. The lumen of the cysts contains
fine hairs and a thick yellow sebaceous secretion.
Some authors have recommended the term Ôder-
moid cyst’ solely for cystic lesions in which there are
no adnexal structures. When other tissues (cartilage,
muscle, etc.) are present, these lesions should
probably be called teratomas.79,80
Cysts presenting in the dorsum of the nose may
have a communicating tract from a deep cystic
location to the outer skin, a condition termed fistula Fig. 21. Low-power view of an omphalomesenteric cyst.

825
Jenkins & Morgan
and may show areas of squamous metaplasia.89
There appears to be no identifiable risk of malignant
degeneration.

Median raphe
Description
Used to describe developmental cysts arising at the
midline at any point from the anus to the external
urethral penile meatus, these are small cysts (less
than 1.0–1.5 cm) most commonly located on or near
the glans penis and diagnosed within the first 30
years of life.90 They result from failed closure of the
median raphe during the embryological period but
may also be the result of urethral lining outgrowth,
Fig. 22. The wall of the omphalomesenteric cyst in Fig. 21. Note
the gastric mucosa of the cyst wall.
ectopic growth of urethral mucosa or trauma to or
infection of the raphe.91 Contents are turbid to clear,
without ulceration. Staining for CEA is seen in the depending on the presence of mucous glands within
cysts, and they have been reported to rarely undergo the cyst wall.
malignant transformation.84
Histopathology
Median raphe cysts are located in the dermis, with
Cutaneous ciliated cyst of the lower limbs no communication to the skin surface. The cysts are
Description lined by pseudostratified columnar epithelium that
The cutaneous ciliated cyst of the lower limb becomes more stratified squamous in nature as the
encompasses any of several developmental cysts of urethral meatus is approached. Ciliated cells are
varying origin that are similarly related only by the rare but do occur,92 and mucus glands are
presence of ciliated epithelium. They are seen in the commonly present in the wall. Cytokeratin 7 and
lower extremities or perineal areas of women of 13 positivity, along with positivity for CAM 5.2 and
early to middle reproductive age, although at least CEA, suggests mucinous columnar epithelium
five cases of occurrence in men has been docu- undergoing immature urothelial metaplasia.91
mented.85 They are usually less than 3 cm in
diameter and may be multilocular.
Apocryphal cysts
Histopathology The Ôcysts’ so labeled and presented in this section
The cysts are lined by pseudostratified ciliated lack an epithelial lining. These misnomers are
columnar or cuboidal epithelium and frequently discussed as they constitute important entities often
have papillary projections into the lumen.86 The included in the literature with this topic.
epithelial pattern is strikingly similar to that of the
fallopian tube, and immunohistochemical staining
indicates the presence of estrogen and progesterone Mucocele
receptors, consistent with a Müllerian or para- Description
mesonephric origin.87 Also known as a mucous cyst – a misnomer, as this
entity is typically devoid of the epithelial lining as
Vulval mucinous and ciliated discussed above – mucoceles are small (less than 1
cm), firm, white to blue nodules located most often
Description in the oral mucosa and lower lip and arise from
Also known as Bartholin’s cysts, these lesions are salivary gland rupture and mucus extravasation.93,94
found in the vestibule of the vulva and present in There is a strong female predilection.95
a wide range of sizes.88 They are believed to be of
urogenital sinus origin. Histopathology
Mucoceles present with a poorly defined lining,
Histopathology lacking any type of epithelial structure, in either of
They are most often lined by pseudostratified two ways: first, the extravasation mucocele with fib-
ciliated columnar epithelium with mucinous cells rous and granulation tissue containing muciphages

826
 Dermal cysts
and small mucin-filled cystic spaces within the wall (usually less than 2 cm) and are most always located
and second, the retention type, containing macro- on or near a tendon sheath or joint capsule. They
phages, fibroblasts and connective tissue. The mucin are most commonly seen on the extensor surfaces of
is strongly PAS positive, alcian blue positive at pH the wrists and feet and to a lesser extent on the
2.5 and positive with colloidal iron.95 lateral aspect of the knee. A variant more common
in women can also arise in the temporomandibular
joint.100
Metaplastic synovial
Histopathology
Description
Ganglions are often multilobulated with a fibrous
The term Ômetaplastic synovial cyst’ is another connective tissue wall, lacking a synovial cell lining
misnomer, describing unilocular foci contained and filled with clear viscous or gelatinous material
completely within the dermis. They possess a lining that may show central myxoid degeneration. There
reminiscent of joint synovium but tend to arise in is no communication with the adjacent joint cavity
sites unrelated to bones, joints or synovial structures. as with synovial cysts.101 One study that subjected
Typically, metaplastic synovial cysts are tender, ganglion aspirate to cytopathological techniques
erythematous, solitary nodules, but cases with mul- showed a myxomatous stroma, which exhibited
tiple cysts have been reported,96 and a variant with a Ôcrackling’ artifact on thick Papanicolaou-stained
multiple large papillary formations has been seen.97 smears. The slides were primarily hypocellular, but
Most often they are caused by trauma or are when cells were present, they were almost exclu-
associated with surgical scars, especially from sively mucinous macrophages, which stain positively
orthopedic surgery, and may rarely be the result of for cytoplasmic CD68. Romanowsky staining shows
implanted synovial tissue. In fact, orthopedic sur- a finely granular precipitate.102
geons have been known to intentionally induce
a synovial cyst at the site of a future tendon graft for
improved graft survival and lubrication.97 There References
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