Benign

Nonodontogenic
Lesions of the Jaws
 Benign nonodontogenic lesions of the jaws
represent a mixed group of tumors,

 there are some lesions within this group

that actually only seem to occur in the
jaws, and, therefore, although they do not
contain any histologic or
immunohistochemical evidence of
odontogenic structures,
 the mere fact that they only occur in the
jaws may mean that they are in fact
odontogenic.
Benign Fibro-osseous
Disease
 Differences remain in the classification
and diagnosis of fibro-osseous disease

 common entity for all of the lesions is

the replacement of normal bone with a
tissue composed of collagen fibers and
fibroblasts that contain varying amounts
of mineralized substance, which can be
either bone or cementum-like material.
 Fibrous Dysplasia

 Fibrous dysplasia is considered to be a

developmental hamartomatous
fibroosseous disease of unknown
etiology.
 subdivided into four different
forms:
 1. Monostotic fibrous dysplasia affecting only
one bone
 2. Polyostotic fibrous dysplasia affecting
multiple bones
 3. Albright’s syndrome in which multiple
lesions are associated with hyperpigmentation
and endocrine disturbances, predominantly
precocious puberty and/or hyperthyroidism
 4. Craniofacial fibrous dysplasia confined to
bones of the craniofacial complex
 The jaws are commonly associated with all
forms of fibrous dysplasia.
 the onset is usually during the first and second
decades,
 and it produces painless swelling of the
involved bones
 the radiographic appearance shows a ground-
glass opacity without clearly defined borders
 Expansion can cause compression of nerves
and blood vessels. The optic canal can be
narrowed by fibrous dysplasia,
 The maxilla appears to be affected more
often than the mandible, and females are
affected more commonly than males.

 periods of activity and periods of

quiescence.

 When they are active, they are often

symptomatic /throbbing or discomfort, the
swelling increases, and the lesions appear
hot on a bone scan
 quiescent phase they may be totally
asymptomatic.

 Teeth can be displaced by the lesion

 The lesions of fibrous dysplasia may be under
hormonal control,
 particularly in Albright’s syndrome, and cases of
increased activity and reactivation during pregnancy
have been noted.

 Although not normally recognized as a premalignant

lesion, sarcomatous change has been noted in
fibrous dysplasia

 Early cases appear to have been associated with the

use of radiation therapy for treatment but cases of
spontaneous sarcomatous degeneration have been
noted,
 ground-glass
appearance of lesions.
 Treatment
 is generally symptomatic; if the lesions are asymptomatic,
a biopsy for diagnosis alone may be adequate without
carrying out any definitive treatment.
 Surgical treatment should be limited during an active
phase because the lesions are vascular and can bleed
quite profusely.
 Treatment is best reserved for quiescent periods, at which
time cosmetic recontouring is the normal treatment of
choice.
 Regrowth can be expected following this treatment in 25
to 50% of cases, particularly if undertaken at a young age.

 Some investigators have suggested more aggressive

surgical procedures including mandibular and maxillary
resections
 Cemento-osseous
Dysplasia

 The cemento-osseous dysplasias

represent a pathologic process of the
tooth-bearing areas and probably
represent the commonest manifestation
of fibro-osseous disease
 they are frequently asymptomatic and
require no treatment,
 In this condition there is a disordered
production of bone and cementum like
tissue in the jaws.
 The three forms include periapical, focal,
florid osseous dysplasias, and familial
gigantiform cementoma, which are
probably variants of the same pathologic
process but which can be differentiated by
clinical and radiographic features.
 The etiology of these lesions remains in doubt,
but local trauma may play some part, as
abnormal occlusal forces.

 It is suspected that the periodontal ligament

may be the origin of the fibrous tissue found in
the cemento-osseous dysplasias.

 Histologically the three types of cemento-

osseous dysplasia are indistinguishable,
Periapical Cemento-osseous
dysplasia

 circumscribed lesions in periapical areas

associated with vital teeth, with the
anterior mandible being most usually
 Radiographically

 the lesions can be radiolucent, mixed

density, or radiopaque, depending on
their stage of development
Periapical cemento-osseous dysplasia
of the left maxilla (an atypical site).
 Focal Cemento-osseous
Dysplasia
 present as nonexpansile radiolucencies with
associated opacities, often in edentulous areas of
the mandible.

 They frequently occur in sites of previous dental

extractions and may represent some type of
abnormal healing following dental extraction.

 Since they are usually asymptomatic, cases are

often noted on routine panoramic radiographs. They
are normally well circumscribed and rarely exceed 2
cm.

 Differentiation from ossifying fibroma may be difficult

 Florid Cemento-osseous
Dysplasia Florid
 presents as a painless nonexpansile lesion
often involving two or more jaw quadrants.

 Radiographically it appears as multiple lobular

radiopaque masses in tooth-bearing areas .

 Lesions may be associated with superimposed

infection and osteomyelitis,and have also been
associated with idiopathic bone cysts
 Many patients are partially or totally
edentulous when the condition is first
discovered.

 Cortical expansion is usually absent or of

limited degree.

 It has been suggested that chronic diffuse

sclerosing osteomyelitis may represent a
variant of this condition,
Florid cemento-osseous dysplasia
of the mandible
 Fibro-osseous
Neoplasms Ossifying
Fibroma
 Ossifying fibroma (cemento-ossifying
fibroma)

 usually presents as a well-demarcated

mixed radiolucency/ radiopacity with
smooth and often sclerotic borders

 The lesions are usually solitary and most

commonly occur in the mandible.
 The ossifying fibroma is felt to be a true
neoplasm and occurs at a later age than
does fibrous dysplasia,

 the treatment is surgical; in fact, the

lesions often shell out easily at surgery,
 some authors recommend aggressive
treatment for more aggressive lesions,
including aggressive curettage,
Ossifying fibroma
 Juvenile Aggressive
Ossifying Fibroma
 variant of ossifying fibroma.

 The lesions classically occur in younger

children and adolescents and present
with an aggressive behavior,
Osteoblastoma and Osteoid
Osteoma
 Osteoblastoma and osteoid osteoma are
generally felt to be variants of the same
lesion and are related to fibro-osseous
disease.

 benign neoplasms.

 The osteoblastoma occurs primarily in the

vertebrae and long bones, but it has been
described in the jaws
 Clinically
 grows rapidly
 pain,
 Although felt to be a true neoplasm, there
have been reports of regression after biopsy or
incomplete removal, which could point to it
being a reactive process
 Most cases of osteoblastoma occur in the
second decade of life; rarely occur after age
30 years.
 In the head and neck, the mandible is the most
common site.
 Radiographic features
 usually consisting of a combination of
radiolucency and radiopacity
 The designation osteoblastoma is normally
reserved for lesions > 2 cm in diameter.
 well circumscribed radio opacity with a thin
radiolucency surrounding the variably
calcified contents.
 A sunray pattern of new bone formation
similar to that described in malignant bone
tumors may be evident.
 Differentiation must be made from the
ossifying fibroma, fibrous dysplasia, and
osteosarcoma.
 Osteoblastoma of the left mandible
in 24-year-old female. Note mixed radiolucency/
radiopacity with a radiolucent rim.
Treatment of the osteoblastoma

 conservative surgical excision either

with curettage or local excision.
 Recurrences are rare but have been
reported and may necessitate more
aggressive treatment
 The osteoid osteoma represents a smaller
version of the osteoblastoma and is felt to
be a true neoplasm.

 It is normally < 2 cm in diameter clinically

and radiographically. It again occurs in the
second and third decades of life with a
male predominance.
 Pain is again the major clinical feature.
 Classically, the pain is worse at night and is
relieved by acetylsalicylic acid.
 Chondroma
 A chondroma is a benign tumor of mature
cartilage.

 The occurrence of these lesions in the jaws

is extremely rare;

 mandibular condyle,

 presents as a painless slowly progressive

swelling,
 Radiographically they present as irregular
radiolucent lesions, although foci of
calcification may occasionally be present.

 surgical excision is normally

recommended.

 doubtful nature …… some authors have

suggested wide excision
???????chondrosarcoma,
 Osteoma
 Osteomas are benign tumors consisting of mature
compact or cancellous bone.

 They may arise on the surface of bone (periosteal

osteomas) or centrally within the bone (endosteal
osteomas).

 They are often discovered as asymptomatic radiopacities.

 commonly discovered during the second and fifth

decades of life, although they have been noted in all age
groups.
 Males appear to be affected more frequently than
females.
,
 Gardner’s syndrome is an autosomal dominant
condition in which patients have intestinal polyposis,
multiple osteomas (usually endosteal) of the jaws,
Fibromas of the skin, epidermal cysts, impacted
teeth, and odontomas.

 The clinical significance of this syndrome is that the

intestinal polyps, which frequently occur in the colon
and rectum, are premalignant and have a very high
rate of malignant transformation.

 The associated osteomas are often found in the

jaws, particularly in the angle region of the mandible,
as well as the facial bones and long bones.
 It has been suggested that any patient with
multiple mandibular osteomas should be
investigated for the possibility of Gardner’s
syndrome.

 Investigation should include a detailed

history of gastrointestinal disturbance and,
if positive, follow-up with colonoscopy; if
the diagnosis is confirmed, a prophylactic
colectomy should be performed.
 Periosteal osteomas usually present as
asymptomatic slow-growing bony masses.

 Endosteal osteomas are usually

asymptomatic and are noted on routine
radiographs.

 Radiographically they appear as well-

circumscribed sclerotic radiopaque masses
 Treatment of osteomas
 surgical excision This is often necessary
to establish the diagnosis.

 Asymptomatic cases may be followed

up clinically and radiographically without
treatment.

 Following excision, recurrences are

very rare.
 Synovial Chondromatosis
and Osteochondroma
 Both are conditions that occur in the temporomandibular
joints and may be considered variants of the chondroma
and osteoma.

 In synovial chondromatosis there is a proliferation of small

particulate, generally unattached chondromas within the
confines of the joint capsule.

 Although most frequently found in the knee, they have

been reported in most joints.

 Well recognized cases have occurred in the

temporomandibular joints with symptoms normally
consisting of pain and swelling but most often with
deviation of the mandible toward the unaffected side
 The etiology is unknown, but trauma has
been suggested.

 When these lesions become

symptomatic, they should be removed
via a standard preauricular approach.
Lesions Containing Giant
Cells
 There are a number of lesions that occur in the
jaws that contain giant cells within them.

 Histologically all of the giant cell lesions

appear similar, if not identical, and they usually
cannot be distinguished on light microscopy
alone.

 The clinical history, immunohistochemistry, or

genetic markers have to be used to
differentiate the lesions.
Central Giant Cell
Granuloma
 Central giant cell granuloma is a lesion occurring
almost exclusively in the jaws.

 not considered an odontogenic lesion,

 the fact that it only occurs in the jawbones probably

indicates some relationship to the teeth or tooth-
bearing structures.

 It occurs primarily in the anterior parts of the jaws in

people in the second and third decades of life, but it
has been recorded in all sites at all ages.
 it was considered a reparative lesion that was essentially self-healing.
There was little evidence of this,

 Worth showed in a study of a number of non treated lesions that

resolution often did occur as seen radiographically; even when the
lesions did not resolve completely radiographically, only a fibrous scar
was noted on surgical exploration.

 The current consensus, however, is that these are not reparative

lesions and that if they are not treated, they are progressive.

 Most appear to follow a fairly benign course, but more aggressive

lesions have been noted.

 The true nature of the central giant cell granuloma remains

speculative. It has been suggested that it may be an inflammatory
lesion, a reactive lesion, a true tumor, or an endocrine lesion.
 Older theories about the origin of these
lesions suggested that they may be
derived from the odontoclasts that were
responsible for resorption of the
deciduous teeth; this was said to explain
why they are normally found in areas
where deciduous teeth were present
and are found after the deciduous teeth
have resorbed.
 Radiographically

 the central giant cell granuloma can take

a number of forms from
 well-defined radiolucency,
 more ill-defined radiolucency or a
 multilocular radiolucency.
 Teeth can be displaced by the lesion,
although resorption of teeth is
uncommon
 central giant cell granuloma of
the anterior mandible causing the displacement
of teeth.
 Histologically
 these granulomas contain focal
arrangements of giant cells within a
vascular stroma with thin-walled
capillaries adjacent to the giant cells.
 Treatment
 is usually surgical and consists of local
curettage, which is usually curative.
 However, there is a 15 to 20% recurrence rate,
and if the lesions are large, even conservative
curettage may involve the loss of many teeth
and possibly the inferior alveolar nerve in the
mandible, and it may have sinus and nasal
implications in the maxilla.
 With the aggressive variants, more aggressive
surgery has been suggested including
mandibular resection and appropriate
reconstruction.
 Since the central giant cell granuloma
and the brown tumor of
hyperparathyroidism cannot be
separated histologically, it is advocated
that hyperparathyroidism be excluded
from the diagnosis by serum calcium,
phosphate, and parathormone
 A number of nonsurgical treatments have been
suggested, all of which have their advocates.
 Intralesional steroids (usually triamcinolone
injected into the lesion once per week for 6
wk) have been advocated and have shown
some success. Their mode of action is
unknown, but they may work by suppressing
the inflammatory component of the lesion.
 They are probably best reserved for smaller
lesions that can be more easily treated by
intralesional injections .
 Calcitonin given by subcutaneous injection
has also been advocated and has met with
some success The theory behind this
treatment is that the lesion may be caused
by an as-yet undiscovered parathormone-
like hormone, and that the use of calcitonin
antagonizes its action and allows the lesion
to heal.
 Since some of the giant cells have been
shown to have calcitonin receptors on
them, this may explain calcitonin’s
effectiveness
 α-Interferon given by subcutaneous injection has
also been advocated in the treatment of the central
giant cell granuloma and has again met with some
success.

 The rationale for this therapy is that the

antiangiogenic action of the α-interferon suppresses
the angiogenic component of this lesion, causing
healing to occur.

 In most cases surgery is still required after the α-

interferon treatment, but it may be less radical
surgery and there may be a smaller chance of
recurrence.
 Giant Cell Tumor
 The giant cell tumor is normally found in the long bones
and its presence in the jaws is not universally accepted;

 if it does occur, it is extremely rare.

 This lesion is an aggressive one and is felt by some to be

a variant of a low-grade osteosarcoma.

 The recurrence rate after local curettage is high, and the

appropriate treatment is in doubt. Some authorities
advocate local curettage, whereas some have advocated
resection.

 Histologically it is very similar to the central giant cell

granuloma,
 Hyperparathyroidism
 In hyperparathyroidism (primary, secondary, or tertiary), calcium
is mobilized from the bones into the blood stream to maintain
homeostasis in the face of increased renal excretion.

 Mobilization from bone takes place focally and produces

lesions in the bones (including the jaws) that are known as
brown tumors because of their fairly distinctive coloration on
surgical exploration.

 Clinically and histologically they are identical to the central giant

cell granuloma and cannot be distinguished on either clinical or
histologic grounds

 Therefore, whenever a lesion such as this is recurrent,

aggressive, or multiple, hyperparathyroidism must be excluded
by means of serum calcium, phosphate, and parathormone and
parathormone-related protein assays.
 hyperparathyroidism should be treated
appropriately.
The lesions normally resolve without any
further treatment being required.
 Cherubism
 Cherubism is a familial genetically
dominant condition
 Affected family members have multiple
lesions mainly affecting the facial bones.
Because of the involvement of the
maxilla and orbital floor, the face has a
rounded appearance and the eyes tend
to look upward, giving the patient a
cherubic appearance
 some patients having subclinical lesions
discovered only on radiographs and
some having extensive and clinically
obvious lesions.
 Radiographically the lesions appear
honeycombed and can be very
extensive.
 Teeth are often displaced, and in active
periods the lesions are extremely
vascular
 Because of its histologic similarity to
central giant cell granuloma, calcitonin
has been used in an attempt to cause
resolution, but it has not met with
success, suggesting that they are, in
fact, different lesions
 Treatment of cherubism is usually
conservative and expectant and into the
teenage years is devoted to trying to aid
eruption of the teeth, which is often abnormal.
 Later it is directed toward cosmetic
recontouring of the affected bones.

 The lesions normally become less active and

less vascular toward the end of the second
decade and into the third decade, and it is at
this time that most cosmetic remodeling is
carried out.
 Aneurysmal Bone Cyst
 Aneurysmal bone cyst is most commonly
found in the jaw bones

 Radiographically the lesion appears as a

wellcircumscribed soap bubble–type lesion
 It responds well to moderately aggressive
curettage, although hemorrhage can be a
problem. Recurrences are rare.
 Vascular Malformations
 Vascular malformations can occur
anywhere in the body and are felt to be
developmental lesions,
 can occur in soft tissue or bone.
 Central vascular malformations of the jaws
are a rare but well documented entity.
 They are in contrast to the true
hemangioma, which is a neoplasm of
vascular endothelium and is normally
present at birth, often enlarges, and then
frequently involutes
 The vascular malformation generally is not present at
birth, appears later, and does not involute.

 Vascular malformations can take a number of forms.

 The most practical classification is to divide them into

high-flow and low-flow vascular malformations.

 The high-flow vascular malformations are either

arterial lesions or arteriovenous fistulas.

 The low-flow malformations are mainly venous in

nature.
 The clinical significance of a vascular
malformation is that a central high-flow
vascular malformation can cause
torrential hemorrhage when surgical
intervention ensues.

 This has been fatal on occasion. Many

of these lesions are asymptomatic and
may even be difficult to detect
preoperatively
 on radiographs
 If there is a clinical presentation, it is often a
slow-growing asymmetric expansile lesion of
the jaw, and if it is high flow, it may be
associated with a bruit.

 Radiographically a high-flow malformation may

appear as an irregular poorly defined soap
bubble–type lesion, which may cause
resorption of the roots of teeth
 Lowflow malformations are similar but are
often somewhat better defined and may
contain calcifications or phleboliths within
them.
 The presence of phleboliths is
diagnostic of a low-flow malformation.
Diagnosis is usually confirmed by
computed tomography
 To avoid the possibility of inadvertently
carrying out a tooth removal or a biopsy in
the presence of a high-flow malformation,

 a diagnostic needle aspiration should be

carried out preoperatively.

 If bright red blood under pressure is

encountered, surgery should be
abandoned.
 Since the radiographic and clinical
appearances of a vascular malformation
are not diagnostic, the differential
diagnosis normally includes a number of
odontogenic and nonodontogenic
lesions, including the central giant cell
granuloma, the aneurysmal bone cyst,
ameloblastoma, odontogenic keratocyst,
and odontogenic myxoma.
 When a vascular malformation is
suspected or diagnosed, selective
angiography is normally performed via a
femoral approach.
 If a high-flow vascular malformation is
diagnosed, treatment is normally
preoperative embolization followed by wide
resective surgery.
 The embolization can involve a number of
materials, including muscle, polyvinyl,
pellets, and platinum coils, which are
inserted via the angiography catheter
 reestablishment of smaller collateral
vessels usually occurs within a few
days, and it is often impossible to
reembolize these smaller collateral
vessels.
 Therefore, definitive surgery should be
carried out within a small number of
days of embolization.
 Definitive surgery normally takes the
form of resection under hypotensive
anesthesia
 Following resection appropriate
reconstruction can be performed.
 Low-flow or venous malformations are
not as life-threatening and are normally
treated with an attempt to thrombose the
lesion by intra lesional injection of a
variety of agents, including sclerosing
agents, an absorbable gelatin sponge,
and platinum coils.
 This may bring about thrombosis,
allowing the necessary dental or surgical
treatment to be carried out.
Langerhans Cell
Histiocytosis
 Langerhans cell histiocytosis is the term currently employed for
what was previously known as histiocytosis X,

 the three separate conditions Letterer-Siwe disease, Hand-

Schüller-Christian disease, and eosinophilic granuloma.

 the common factor was the presence of histiocytes.

 The cells of origin of this disease have now been identified as

the Langerhans cells, which are dendritic cells in the skin and
mucosa that have a macrophage-like function.

 Unknown mechanisims
 Some recent studies have suggested
that it may have some of the properties
of a tumor or have a viral etiology.

 Other studies propose that it may be a

response to an allergenic challenge,
 Letterer-Siwe disease represent the
acute disseminated form of Langerhans
cell histiocytosis, whereas Hand-
Schüller-Christian disease represents
the chronic disseminated form, and
eosinophilic granuloma represents the
chronic localized form.
 The acute disseminated form usually
affects young children. It is multisystem
in nature, affecting the skin, bones, and
internal organs (especially lungs and
liver), and is frequently fatal.

 Treatment is chemotherapy.
 The chronic disseminated form of the disease is
classically associated with a triad of punched-out bone
lesions (often affecting the skull and jaws), diabetes
insipidus (owing to posterior pituitary involvement), and
exophthalmos (owing to deposits in the posterior orbit).

 This normally affects an older age group, often in the

second and third decades but sometimes much older.

 The bone lesions often affect the jaws. Although they

usually appear as fairly well-defined punched-out
radiolucencies
 they can also be less well defined and can affect the apices of the teeth
only and lead to a possible differential diagnosis of periapical infection.

 A frequent aspect of presentation is loose teeth;

 radiographically they often appear as “floating teeth”

 The treatment of the chronic disseminated form of the disease is

variable, and for well-circumscribed lesions can consist of local
curettage.

 for more aggressive forms, chemotherapy is frequently employed as

well.

 Low-dose radiation therapy has also been used on isolated lesions,

 The chronic localized form of the disease is
commonly found in the jaws and usually shows as a
well-defined radiolu-cency, often in the bicuspid
region and more frequently in the mandible.

 Differential diagnosis in this case includes any fairly

well-defined radiolucency.

 Treatment usually consists of aggressive local

curettage, and the recurrence rate is low.
 Teeth are sacrificed as necessary.
 Intralesional steroids have also been employed with
some success, and cases of spontaneous regression
have been reported.
 Paget’s Disease
 Its alternative name is osteitis
deformans.
 It is a slowly progressive bone condition
of unknown etiology, predominantly
affecting males over the age of 50
years.
 One unproven theory is that Paget’s
disease may be a delayed or slow
reaction to a myxovirus stimulus.
 Clinically there is hyperactive bone turnover
with alternate resorption of bone, a vascular
phase, and finally a sclerosing phase.

 Most bones of the body are involved, and the

disease can result in considerable deformity.

 In the facial region the maxilla is affected more

often than the mandible.

 Family histories have been obtained

 The classic presentation used to be a
patient whose hat or gloves no longer fitted
correctly, or in whom false teeth,
particularly the maxillary denture, did not fit
owing to bone swelling.

 bone deformity or pain. In the head and

neck, headaches and symptoms owing to
vascular and nerve compression have
been noted.
 The classic radiographic appearance is of a
“cotton-wool” appearance in the skull and
maxilla of affected patients

 with hypercementosis around the roots of

teeth, and loss of lamina dura and obliteration
of the periodontal ligament space.

 This does make tooth extraction extremely

difficult in these patients.

 Root resorption has also been noted.

 Classically, patients have markedly
elevated serum alkaline phosphatase
levels.
 Treatment is both systemic and local.

 Systemic treatment currently consists of the use of

calcitonin or diphosphonates to inhibit bone
resorption.

 Calcitonin can be taken either subcutaneously or by

nasal spray, and diphosphonates are taken orally or
by injection.

 Treatment causes stabilization of the bone and a

lowering of the raised alkaline phosphatase levels.
 Localized treatment is directed to
cosmetic and/or functional recontouring
of bone.
 It should be noted that the bone of
Paget’s disease is often vascular, and
bleeding during recontouring can be
extensive.
 The classic causes of death in patients with
Paget’s disease are heart failure and
osteosarcoma.
 Heart failure caused by the excessive
blood supply to the remodeling bone can
cause high output or left heart failure in
elderly persons.
 Sarcomatous change has been reported in
5 to 15% of patients with Paget’s disease,
which should be considered a premalignant
condition
 Tori