Cysts of the Jaws& Maxillofacial regions

Definition:
True cyst: pathological cavity lined by epithelial lining containing fluid
or semisolid material.
Pseudo- cyst: not lined by epithelium.

Cysts are classified according to their site into:

I- Cysts of the jaws.
II- Cysts of the soft tissues of the mouth, face, neck and salivary glands.
I- Cysts of the jaws
 True cysts of the jaws
 Odontogenic cysts.
1) Inflammatory odontogenic cysts.
− Cysts derived from epithelial rests of malassez.
1. Radicular cyst (periodontal cyst).
A. Apical (periapical) cyst.
B. Lateral radicular cyst.
C. Residual cyst.
2) Developmental odontogenic cysts.
− Cyst derived from reduced enamel epithelium.
1. Dentigerous cyst.
2. Eruption cyst (eruption hematoma).
− Cyst derived from dental lamina rests.
1. Odontogenic keratocyst.
2. Lateral periodontal cyst.
3. Gingival cyst of adult.
4. Gingival cyst of newborn.
 Non odontogenic developmental cysts:
− Cyst of vestigial ducts.
1. Nasopalatine duct cyst (incisive canal cyst and cyst
of palatine papilla).
2. Nasolabial cyst.
 Pseudo cysts of the jaws.
1. Latent bone cyst.
2. Traumatic bone cyst.
3. Aneurysmal bone cyst.

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II- Cysts of the soft tissues of maxillofacial regions:
− Cyst of vestigial tract.
1. Thyroglossal tract cyst.
− Lymphoepithelial cyst.
1. Oral Lymphoepithelial cyst.
2. Cervical Lymphoepithelial cyst.
− Cyst of embryonic skin.
1. Dermoid and epidermoid cysts.
− Cyst of salivary glands (mucocele).
1. Mucous extravasation cyst.
2. Mucous retention cyst.
3. Ranula.

I. Cysts of the jaws

1) True cysts of the jaws
A.Odontogenic Cysts
A cyst in which the epithelial lining of the lumen is derived from the
epithelial rests of the tooth-forming tissue such as:
1. Epithelial rests of Malassez.
2. Reduced enamel epithelium.
3. Remnants of the dental lamina (epithelial rests of Serres).

1) Inflammatory Odontogenic Cysts

Radicular cyst (periodontal cyst)
a) Periapical cyst.
b) Lateral radicular cyst.
c) Residual periapical cyst.

A. Periapical cyst
(Radicular cyst; Apical periodontal cyst)
Periapical cysts are inflammatory cysts derive their epithelial lining from
the proliferation of epithelial rests of Malassez

Etiology and pathogenesis:

 A periapical cyst develops from a preexisting periapical granuloma
located at the apex of a non-vital tooth.

 Persistence of chronic inflammatory stimuli derived from the necrotic

pulp stimulates proliferation of the epithelial rests of Malassez;

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 Because epithelial cells obtain their nutrients by diffusion from the
adjacent connective tissues, progressive growth of an epithelial island
moves the inner cells of that island away from their nutrients.
Ultimately, these inner cells undergo necrosis, producing a central
cavity (lumen) surrounded by viable epithelium.

 Breakdown of cellular debris within the cyst lumen raises the protein
concentration, leading to fluid transport across the epithelial lining
into the lumen from the connective tissue side. Fluid ingress assists in
outward growth of the cyst.

 Other bone-resorbing factors, such as prostaglandins, permit

additional cyst enlargement through osteoclastic bone resorption.

Clinical features:
Age: any age but rarely is seen before age of 10 years.
Site: maxillary region> mandibular region.
Sign and symptoms:
 Asymptomatic and are often discovered during routine dental
radiographic examination.
 Associated tooth is non-vital.
 As they enlarge they cause slowly progressive bony swellings often
on the labial or buccal side.
 When the rate of expansion exceeds the rate of subperiosteal bone
deposition, leading to progressive thinning of the bone to eggshell
thickness, a crackling sensation may be felt on pressure.
Radiographic features:
 Round to ovoid radiolucency associated with apex of non-vital
tooth, with a narrow, opaque margin that may become hazy if the
cyst becomes infected.
 In long-standing cysts, root resorption of the associated tooth may be
seen.

Histopathologic features:
 Epithelial lining: non-keratinized stratified squamous epithelium of
variable thickness.
o In newly formed cysts the epithelial lining is hyperplastic
exhibiting long anastomosing cords of epithelium forming
arcades or rings over vascularized connective tissue and many
inflammatory cells.
o In fully formed cysts the epithelial lining become more regular
and of even thickness.
 The lining epithelium contains hyaline eosinophilic bodies "Rushton
bodies" characterized by a hairpin or slightly curved shape. The origin
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 of these bodies is related to previous hemorrhage. Metaplasia of the
epithelial lining to mucous cells, rarely, respiratory epithelium may
be found.
 Connective tissue capsule: infiltrated with inflammatory cells. As
inflammation subsides, the connective tissue capsule tends to become
more fibrous and less vascular with reduction of the inflammatory cell
infiltration.
 Plasma cell infiltration is always associated with “Russell bodies”
which are spherical intracellular eosinophilic, large, homogenous
inclusions found in a plasma cell undergoing excessive synthesis of
immunoglobulin.
 Cholesterol clefts: These are fine needle-shaped spaces within the
cyst capsule which are left by cholesterol crystals that dissolved-out
during preparation for tissue sectioning.
 The cystic lumen contains proteinaceous fluid and necrotic cellular
debris and cholesterol crystals in great amounts.

B. Lateral radicular cysts

 It develops at the side of the root of a non-vital tooth as a result of the
opening of accessory root canal through which pulpal inflammation
can exit to form granulomas and stimulate epithelial rests of Malassez
located on the lateral aspect of the roots of teeth.
 They are rare and must be distinguished from developmental lateral
periodontal cysts.

C.Residual cysts
 The residual cyst is a radicular cyst that persist in the jaw and fail to
resolve following extraction of the involved tooth.
 Residual cysts are a common cause of swelling of the edentulous jaw
in older persons and interferes with the fitness of dentures, or it may
slowly regress spontaneously.

2) Developmental Odontogenic Cysts

Dentigerous cyst; Follicular cysts
 The term “dentigerous” means containing tooth.
 It is a developmental odontogenic cyst that encloses the crown of an
unerupted tooth and is attached to the tooth at cement- enamel
junction; resulting in a cyst in which the crown is located within the
lumen.
Etiology and pathogenesis: The pathogenesis of this cyst is uncertain.
 It develops by accumulation of fluid between the reduced enamel
epithelium and the tooth crown due to obstruction of the venous flow.
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 On occasion, few cases appear to have an inflammatory pathogenesis,
dentigerous cyst may arise around the crown of an unerupted
permanent tooth as a result of spread of periapical inflammation from
an overlying primary tooth to involve the follicle of the permanent
successor.

Clinical features:
Age: second and third decades.
Site:
 It is associated with any unerupted tooth, most often they involve
third molar and the maxillary permanent canines (the most
commonly impacted teeth due to their late eruption time).
 Small dentigerous cysts are asymptomatic and are discovered on a
routine radiographic examination or to determine the reason for the
failure of a tooth to erupt.
 Dentigerous cysts may be associated with a painless expansion of the
bone. Extensive lesions may result in facial asymmetry.
Radiographic features:
 A dentigerous cyst presents as a well-defined, unilocular,
radiolucency enclosing the crown of an unerupted tooth surrounded
by sclerotic margins indicative of its slow and uniform growth.
 Root resorption of adjacent erupted teeth can occur.
 Dentigerous cyst often displaces unerupted tooth for a considerable
distance.
− The mandibular third molar may be displaced to the lower
border of the mandible or higher up into the ascending ramus.
− The maxillary canine may be displaced into the floor of the
nose, and may be moved through the maxillary sinus to the
floor of the orbit.
Histopathologic features:

 . The epithelium lining is thin non-keratinized stratified squamous

epithelium of two to four cell layers, the epithelium and connective
tissue interface is flat.

 Focal areas of mucous cells may be found in the epithelial lining of

dentigerous cysts. Rarely, ciliated columnar cells are present. These
elements are believed to represent the multipotentiality of the
odontogenic epithelial lining in a dentigerous cyst.

− The fibrous connective tissue wall is loosely arranged free of

inflammatory cells.

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Complications: The epithelial lining of a dentigerous cyst may undergo
neoplastic transformation into:
1. Ameloblastoma.
2. Squamous cell carcinoma.
3. Intraosseous mucoepidermoid carcinoma; that develops from
mucous cells in the epithelial lining.

Eruption cyst
(Eruption hematoma)
 The eruption cyst is a soft tissue variant of the dentigerous cyst.
 It results from fluid accumulation within the follicular space of an
erupting tooth's crown which erupted through bone but not soft tissue.
 The cysts developed because of dense collagen deposition in the
gingival connective tissue that resulted in a thicker, less penetrable,
pericoronal roof.
 The cyst appears as soft, translucent fluctuant swelling in the gingival
mucosa overlying the crown of deciduous or permanent tooth. Most
commonly associated with the first permanent molars and
maxillary incisors.
 Trauma from mastication will occasionally induce hemorrhage in an
eruption cyst, blood may appear within the cystic space, which cause
its blue or purple color, forming a so-called "eruption hematoma".
 No treatment is required because they spontaneously rupture as a
result of normal mastication. For those cysts that do not resolve
spontaneously, the crown of the involved tooth can be surgically
exposed by excising the roof of the cyst, allowing the involved tooth
to erupt.
Odontogenic keratocyst
Keratocystic odontogenic tumor
 It is a unique form of developmental odontogenic cyst because of its
specific histopathologic features, aggressive clinical behavior, unusual
growth pattern, a significant high recurrence rate and an association
with nevoid basal cell carcinoma syndrome (NBCCS).
Histogenesis:
Odontogenic keratocyst is derived from the dental lamina remnants.

 Growth of keratocysts occurs by active epithelial proliferation. The

epithelial lining exhibits high proliferation rate, with production of
bone-resorbing factors in the fibrous wall leading to folding and
finger-like projection of the cyst lining into surrounding cancellous
bone.
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Clinical features:
Age: second and third decades.
Site: mandible > maxilla (2:1 ratio).
− Keratocysts enlarge predominantly in an anteroposterior direction and
can reach large sizes without causing gross bony expansion.
− Small odontogenic keratocysts are usually asymptomatic and
discovered on routine radiographic examination.
− Larger odontogenic kerarocysts may be associated with painless
swelling.
− Multiple odontogenic keratocysts are one of features of the nevoid
basal cell carcinoma syndrome (Gorlin-Goltz syndrome).

Radiographic features:
− Odontogenic keratocysts demonstrate a well-defined unilocular or
characteristically multilocular radiolucent areas in large lesions with
radio-opaque margin. May be associated with an unerupted tooth.
Resorption of the roots of erupted teeth adjacent to the cyst commonly
seen,
Histopathologic features:
The epithelial lining composed of uniform, regular, layer of stratified
squamous epithelium ranging from 6 to 8 cell layers thick.
 The basal layer exhibits a characteristic cuboidal or columnar
palisaded and polarized with hyperchromatic nuclei giving
“tombstone” appearance.
 The luminal surface shows corrugated or wavy parakeratinized
surface layer.
 Budding of the basal cells into the connective tissue wall with
microcyst formation "satellite cysts" or "daughter cysts", also cords
or islands of odontogenic epithelium may be seen within the fibrous
wall.
 The epithelium-connective tissue interface is characteristically
flattened, with no epithelial ridge formation.
 Detachment of portions of the epithelial lining from the fibrous wall is
commonly observed.
 Folding of the cyst wall is seen between the surrounding cancellous
bones.
The fibrous connective tissue wall is relatively thin friable and often
free of an inflammatory cell infiltrate.
 In the presence of inflammatory changes, the typical features of the
odontogenic keratocyst may be altered. The parakeratinized luminal
surface disappears, and the epithelium may proliferate to form rete
ridges with the loss of the characteristic palisaded basal layer and the
diagnosis cannot be confirmed.
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The cystic lumen filled with a cheesy material that, on microscopic
examination consists of keratinaceous debris.

Orthokeratinized odontogenic cyst has a flat, non-corrugated surface,

cover by orthokeratin layer. The basal cell layer is less prominent, do not
demonstrate a hyperchromatic or palisaded nuclei.
− It differs from parakeratinized variant:
1. Less aggressive.
2. Lower rate of recurrence.
3. Not associated with nevoid basal cell carcinoma syndrome.

Causes of recurrence in case of parakeratotic cyst is related to difficulty

of complete removal of the cyst from the bone in one piece as:
1. Thin, friable easily ruptured cyst wall which is often difficult to
enucleate from the bone in one piece.
2. Finger-like projections (folding) of the cyst into cancellous spaces.
3. Budding of epithelium into connective tissue.
4. Presence of daughter or satellite cysts in fibrous capsule.

Calcifying odontogenic cyst

Gorlin’s cyst
− It is a developmental odontogenic lesion that demonstrates variable
histopathologic and clinical. calcifying odontogenic cysts include to
different lesions cystic and solid neoplasmas, neoplasms known as
“odontogenic ghost cell tumor”.
Histogenesis:
− It is derived from odontogenic epithelial remnants within the gingiva
or within the mandible or maxilla.
Clinical features:
Age: mostly in the second decade.
Site: may occur both intraosseously and extraosseously.
− Intraosseously, maxilla> mandible. Most cases are found in the
incisor and canine areas.
− Extraosseous calcifying odontogenic cysts appear as localized sessile
or pedunculated gingival masses.

Radiographic features:
− The central calcifying odontogenic cyst appears as unilocular, well-
defined radiolucency; occasionally some lesions may appear
multilocular.
− Radiopaque structures within the lesion, either irregular calcifications
or tooth like densities, are often present.
− In some cases, the radiolucent lesion is associated with an unerupted
tooth, most often a canine.
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− Root resorption or divergence of adjacent teeth may be seen.

Histopathologic features:
1. The cystic (non-neoplastic) forms appear as a well-defined cystic
cavity lined by odontogenic epithelium of 4 to 10 cells in thickness.
The basal cells may be cuboidal or columnar with hyperchromatic
nuclei and are similar to ameloblasts. The overlying layer of loosely
arranged epithelium may resemble the stellate reticulum of an
ameloblastoma.
2. The most characteristic histopathologic feature is the presence of
masses of swollen and eosinophilic keratinized epithelial cells called
“ghost” cells.
 These ghost cells are altered epithelial cells that lost their nuclei
but maintain of the cell outline and it is believed to be a form of
keratinization of odontogenic epithelium.
 Masses of ghost cells may fuse to form large sheets of amorphous,
acellular material.
 Dystrophic calcification of the ghost cells is common.
 Breakdown of the epithelium may release ghost cells into the
surrounding connective tissue resulting in a prominent foreign-
body reaction.
3. In the surrounding connective tissue wall, areas of an eosinophilic
matrix material that represent dysplastic dentin (dentinoid) may be
present adjacent to the epithelial component. This is believed to be
the result of an inductive effect by the odontogenic epithelium on the
adjacent mesenchymal tissue.
− Several variants of the cystic type of calcifying odontogenic cyst are
seen.
 Multiple daughter cysts may be present within the fibrous wall.
 Epithelial proliferation of the cyst lining into the lumen may
resemble ameloblastoma. These proliferations are intermixed with
varying numbers of ghost cells.
 Less commonly, cysts are associated with odontomas either
complex or compound.

Gingival cyst of the newborn

− They are multiple small superficial keratin-filled cysts that are found
on the alveolar mucosa of infants.
− These cysts arise from remnants of the dental lamina that remain
within the alveolar ridge mucosa after tooth formation.
− Clinical features:
− The maxillary alveolus > mandibular.

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− Appear as small, multiple whitish papules on the mucosa overlying the
alveolar processes of newborn.
Histologic features:
− Cyst is lined by thin, flattened epithelial lining with a parakeratotic
surface.
− The lumen contains desquamated keratin.
Palatal cysts of the newborn
(Epstein's pearls; Bohn's nodules)
− Small developmental cysts found on the palate of newborn infants.
− These keratin-filled cysts appear as white or yellowish-white papules.
− These "inclusion" cysts may arise in one of two ways:
1. Epstein's pearls occur along the median palatal raphe.
− The palatal shelves fuse in the midline during embryonic life to form
the secondary palate, small islands of epithelium may become
entrapped below the surface epithelium along the line of fusion
(median palatal raphe) and form cysts.
2. Bohn’s nodules are scattered over the hard palate, often near the soft
palate junction.
− These cysts may arise from epithelial remnants derived from the
development of the minor salivary glands of the palate.

B. Non-odontogenic developmental cysts

Non-odontogenic cysts are those which their epithelial lining are derived
from ectoderm involved in the development of the facial tissues.

Nasopalatine duct cyst

(Incisive canal cyst)
The nasopalatine duct cyst is the most common non-odontogenic cyst of
the oral cavity, also known as incisive canal cysts, are located within the
nasopalatine canal or within the palatal soft tissues at the point of the
opening of the canal, where the lesions are called cysts of the palatine
papilla.
Etiology and pathogenesis:
− Nasopalatine ducts, an epithelial tissue, are found within the incisive
canals. These ducts normally degenerate but may leave epithelial
remnants behind in the incisive canals.
− In addition to the nasopalatine ducts, these canals contain the
nasopalatine nerve and arteries.
− Trauma or infection of the duct may stimulate these epithelial
remnants to proliferate and forming cyst.
Clinical features:
− Age: it may develop at any age.
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− Presents as asymptomatic bony swelling in the midline of anterior
region of the palate.
− Pain may follow secondary infection with sinus formation and
drainage occurs at the palatine papilla.
Cysts of the palatine papilla: a nasopalatine duct cyst may develop at the
point of opening of the canal in the soft tissues of the incisive papilla area
without any bony involvement. Cysts frequently demonstrate bluish
discoloration as a result of the fluid contents in the cyst lumen, which
may ruptures spontaneously with a discharge of salty fluid.
Radiographic features:
− Round or ovoid radiolucency with sharply defined margins in the
midline between or above the roots of the maxillary central incisors.
− The lesion may produce divergence of the roots of the maxillary
incisor teeth or less commonly induce root resorption.
− The anterior nasal spine is often centrally superimposed on the
radiolucent defect, producing a characteristic “heart shape” lesion.
− Cyst of the palatine papilla is negative on X-ray examination.
Histopathologic features:
− The epithelial lining ranges from stratified squamous to
pseudostratified columnar (when located near the nasal cavity).
− A mixture of two types of lining cells is frequently seen.
− The connective tissue wall: contains small arteries and nerves,
representing the nasopalatine nerve and artery as well as small mucous
glands present normally within the incisive canal.
− Frequently, a chronic inflammatory response is noted in the cyst wall
2) Pseudo Cysts of the Jaws
Traumatic bone cyst
 It is also called simple, solitary or haemorrhagic bone cyst.
 A traumatic bone cyst is an empty intrabony cavity that lacks an
epithelial lining.
Pathogenesis:
 Trauma to bone that results in an intraosseous hematoma. If the
hematoma does not undergo organization and repair, it may liquefy,
resulting in an empty bony cavity subsequent to hemolysis and
resorption of the blood clot.
Clinical features:
 Age: Teenagers (between 10 and 20 years).
 Sex: mostly in males.
 Site: commonly occurs in mandibular premolar and molar areas,
The simple bone cyst usually asymptomatic and is discovered only
when radiographs are taken for any reason.
 Few cases produce a painless swelling of the affected area.
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 Associated teeth with the cyst are vital.
Radiographic features:
 The lesion appears as a well-demarcated radiolucent area.
 When several teeth are involved in the lesion, the radiolucent defect
projects upward between the roots of teeth producing scalloped
contour. This feature is highly suggestive but not diagnostic of a
simple bone cyst.
 Teeth involved in the lesion occasionally show slight root resorption.
Histopathologic features:
 The walls of the defect lined by a thin band of vascular fibrous
connective tissue that is intermixed with trabeculae of reactive bone.
 The lining may exhibit areas of extravasated red cells and
haemosiderin, and multinucleated giant cells adjacent to the bone
surface indicative of past osteoclastic activity.
 The cyst cavity may contain red cells or it may be empty.
Aneurysmal bone cyst
 Aneurysmal bone cyst is an intra-osseous accumulation of variable-
sized blood-filed spaces surrounded by cellular fibrous connective
tissue with trabeculae of reactive woven bone.
Etiology and pathogenesis:
 The cause and pathogenesis of the aneurysmal bone cyst are unclear;
but it is regarded as reactive process.
 Aneurysmal bone cyst arises from a trauma, vascular malformation
or neoplasm (such as fibrous dysplasia, giant cell granuloma that
causes localized disturbance of the normal osseous hemodynamics
leading to an enlarging hemorrhagic extravasation  leads to erosion
and resorption of the surrounding bone by stimulation of osteoclasts
 followed by replacement by reactive osteoid and new bone
formation.
Clinical features:
 Age: younger than 30 years of age.
 Site: commonly in the shaft of a long bone or in the vertebral column.
 Within the jaws, mandible > maxilla, chiefly the molar areas.
 The lesion presents as a painful firm, non-pulsating swelling usually
developed rapidly.
 At the time of operation there is excessive bleeding of dark venous
blood “welling up” is encountered from the tissue which is described
as resembling “blood-soaked sponge” with large pores representing
the cavernous spaces of the lesion.
Radiographic features:

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 The cyst appears as unilocular or often multilocular radiolucent
lesion (honey comb or soap-bubble appearance) associated with
marked cortical expansion and thinning.
 Frequently, a ballooning or "blow-out" distention of the contour of
the affected bone cortex may be seen.
Histopathologic features:
 The cyst is characterized by sinusoidal blood spaces filled with
unclotted blood surrounded by fibrous connective tissue containing
fibroblasts, macrophages, extravasated red blood cells, haemosiderin
granules and variable numbers of multinucleated giant cells and
trabeculae of osteoid and woven bone.
 The blood-filled spaces are not lined by endothelium.

II- Cysts of the Soft Tissues of Maxillofacial Regions

Thyroglossal tract cyst
The most common developmental cysts of the neck, derived from
embryologic remnants of the thyroglossal tract.

Pathogenesis
− The thyroglossal duct epithelium normally undergoes atrophy.
− The remnants of this epithelium may persist and proliferate to give
rise to cysts known as "thyroglossal tract cysts". Inflammation is the
most frequently suggested stimulus for epithelial proliferation.

Clinical features:
Age: at any age, most commonly before the age of 20.
Site: occurs in the midline of the neck, below the level of the hyoid
bone, only few cases occurring within the tongue itself.
 The cyst usually presents as a painless, fluctuant, movable swelling.
 If the cyst attached to the hyoid bone and tongue it will move
vertically during swallowing or protrusion of the tongue.

Histopathologic features:
 Epithelial lining:
o Lesions occurring above the level of the hyoid bone  lined by
stratified squamous epithelium.
o Lesions below the hyoid bone lined by respiratory
epithelium.
 Connective tissue wall: contains thyroid tissue.
Complications: Malignant transformation into squamous cell
carcinoma or thyroid adenocarcinomas.

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