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Fibroosseouslesion 160114073747
Fibroosseouslesion 160114073747
Contents
1. Introduction 4. Cherubism
2. Various classification criteria a) Introduction
b) Genetics
3. Fibrous dysplasia c) Clinical features of each type
a) Introduction
d) Radiological features
b) Etiology/Pathogenesis
e) Histological features
c) Clinical features of each type
f) Differential diagnosis
d) Radiological features
g) Treatment and prognosis
e) Histological features
f) Differential diagnosis
g) Treatment and prognosis
Introduction
• Fibro-osseous lesions are a diverse group of processes that are
characterized by replacement of normal bone by fibrous tissue containing a
newly formed mineralized product.
1. Monostotic form
2. Polyostotic form
3. Craniofacial form.
THREE disease patterns
• Each type usually presents as an asymptomatic, slowly expanding portion of one or
more bones.
• The condition develops in children and teenagers primarily, with few if any cases
beginning after the age of 25 years.
• Two-thirds of patients with polyostotic disease are asymptomatic before they are aged
10 years.
• With monostotic disease, patients as old as 20 or 30 years are asymptomatic.
• No specific racial predilection exists.
• The incidence is equal in males and females.
• Clinical findings of increasing pain and an enlarging soft tissue mass suggest malignant
change.
• All types of fibrous dysplasia result from a defect in bone maturation
that begins in the embryo.
• At certain times in the histodifferentiation phase of the embryo,
a genetic mutation or deletion occurs in the gene that encodes for an
intracytoplasmic transducer protein required for bone maturation.
• Consequently, all the daughter cells of this original aberrant cell will
lack this signal transducer, and therefore a certain population of cells
in the individual will be able to
• Produce only fibrous dysplastic bone rather than mature bone.
• If the genetic defect occurs early in embryonic development, a large
number of daughter cells will be affected, some of which may not yet
have migrated to their eventual skeletal site.
• When such early term‐altered cells migrate into several skeletal sites,
they produce polyostotic fibrous dysplasia.
• If the genetic defect occurs in an even earlier phase of embryonic
development, the original cell may produce daughter cells of divergent
differentiation that is, some that will migrate into bone primordia,
some into skin primordia, and some into endocrine gland primordia
and thus produce either the McCune‐Albrightsyndrome or the Jaffe‐
Lichtenstein type of polyostotic fibrous dysplasia.
• The time at which these genetic alterations occur is thought to be
before the sixth week of fetal life.
• When the embryo is in its sixth week of development, most
histodifferentiation and cell migration have already occurred.
• If the same genetic defect occurs around this time, the daughter cells
will be localized to one region and thus may produce the craniofacial
type of fibrous dysplasia, which involves several contiguous bones in a
broad area.
• If the genetic defect occurs slightly later, the daughter cells will be
even more localized and will thus produce monostotic fibrous
dysplasia.
MONOSTOTIC FIBROUS DYSPLASIA
• Monostotic fibrous dysplasia, which involves a single focus in one
bone, accounts for about 75% of fibrous dysplasia cases.
• Frequently occurs in the rib (28%), femur (23%), tibia, craniofacial
bones (10–25%), and humerus.
• Seen most frequently in the body of the mandible or in the premolar‐
molar regions of the maxilla.
• Degree of bone deformity is relatively less severe compared with that
of the polyostotic type.
Clinical Features
• Equal predilection for males and females
• More common in children and young adults than in older
• Mean age of occurrence is 27-34 years.
• The first clinical sign of the disease is a painless swelling or bulging of
the jaw.
• The swelling usually involves the labial or buccal plate, seldom the
lingual aspect, and when it involves the mandible it sometimes causes
a protuberant excrescence of the inferior border.
• Tipping or displacement of the teeth due to the progressive expansile
nature of the lesion
• Mucosa is almost invariably intact over the lesion.
Fibrous dysplasia of the maxilla
• Serious form of the disease since it has a marked predilection for
occurrence in children and is almost impossible to eradicate without
radical, mutilating surgery
• These lesions are not well circumscribed, commonly extend locally to
involve the maxillary sinus, the zygomatic process and the floor of the
orbit, and even extend back toward the base of the skull.
• Severe malocclusion and bulging of the canine fossa or extreme
prominence of the zygomatic process, producing a marked facial
deformity, are typical sequelae of this disease.
Radiographic Features
Three basic patterns
• In one type, the lesion is generally a rather small unilocular radiolucency or a
somewhat larger multilocular radiolucency, both with a rather well-
circumscribed border and containing a network of fine bony trabeculae.
• In the second type, the pattern is similar except that increased trabeculation
renders the lesion more opaque and typically mottled in appearance.
• The third type of quite opaque with many delicate trabeculae gives a ‘ground-
glass’ or ‘peau d’orange’ appearance to the lesion. This latter type
characteristically is not well circumscribed but instead blends into the adjacent
normal bone
• In all types, generally the cortical bone becomes thinned because of
the expansile nature of the growth, but seldom is this bony plate
perforated, or is periosteal proliferation obvious.
1. Cherubism
2. Paget’s disease
3. Cemento-osseous dysplasia
a) Introduction
b) Etiology/Pathogenesis
c) Clinical features of each type
d) Radiological features
e) Histological features
f) Differential diagnosis
g) Treatment and prognosis
Cherubism
CHERUBISM
(Familial fibrous dysplasia of jaws, disseminated
juvenile fibrous dysplasia, familial multilocular
cystic disease of jaws, familial fibrous swelling of
jaws)
An autosomal dominant fibro-osseous lesion of the jaws
involving more than one quadrant that stabilizes after the
growth period, usually leaving some facial deformity and
malocclusion.
• Affect the jaws of
children bilaterally
and symmetrically,
usually producing
the so-called
cherubic look
• The disease was first described in 1933 by Jones, who called it familial
multilocular disease of the jaws.
Inflammation in the
jaw bones
Facial expansion of cherubism at age 9 years. Here the The high school graduation photograph
asymmetry is the result of of the individual shown at age
attempted osseous contouring of the left side. 17 years shows involutional clinical
The facial expansion of cherubism is usually remodeling without further surgery.
symmetric.
Possible explanation
• Typically, the earlier the lesion appears, the more rapidly it progresses.
• The self-limited bone growth usually begins to slow down when the
patient reaches five years of age, and stops by the age of 12–15 years.
• At puberty the lesions begin to regress. Jaw remodeling continues
through the third decade of life, at the end of which the clinical
abnormality may be subtle.
• The signs and symptoms depend on the severity of the condition and
range from clinically or radiographically undetectable features to
deforming mandibular and maxillary overgrowth with respiratory
obstruction and impairment of vision and hearing.
The jaw lesions are usually painless and symmetric and have florid
maxillary involvement.
• The lesions, which are firm to
palpation and non-tender, most
commonly involve the molar to
coronoid regions, the condyles
always being spared, and are
often associated with cervical
lymphadenopathy.
• Enlargement of the cervical lymph nodes contributes to the patient’s
full-faced appearance and is said to be caused by lymphoid
hyperplasia with fibrosis.
• The lymph nodes become enlarged before the patient reaches 6 years
of age, decrease in size after the age of 8 years and are rarely enlarged
after the age of 12 years.
• Intraoral swelling of the
alveolar ridges may occur.
When the maxillary ridge
is involved, the palate
assumes a V shape.
• A rim of sclera may be visible beneath the iris, giving the classic ‘eye
to heaven’ appearance.
Oral Manifestations
• Expansion and deformity of the jaws, and the eruption pattern of the
teeth is disturbed because of the loss of normal support of the
developing teeth.
• The endocrine disturbance also may alter the time of eruption of the
teeth.
Oral Manifestations
Numerous dental abnormalities
• Agenesis of the second and third molars
of the mandible,
• Displacement of the teeth,
• Premature exfoliation of the primary
teeth,
• Delayed eruption of the permanent teeth,
and
• Transpositions and rotation of the teeth.
• In severe cases, tooth resorption occurs.
Cherubism has occurred in association with these syndromes:
1. Noonan syndrome,
orbital rim displaces the globes upward, causing a scleral show. This
• Osseous dysplasia
• Rapid turnover remodeling of bone throughout the skeleton.
• Elderly--older than 50 years
• Most prevalent in the Britain and New Zealand—Classic paget’s.
• Men : women = 3:2
• The bones most commonly affected are the spine, femurs, skull,
pelvis, sternum, and jaws.
• Maxilla is affected twice as frequently as the mandible.
Genomics
• Defective function of the osteoprotegerin/ TNFRSF11A or
B/RANKL/RANK pathway, a molecular regulator of osteoclastogenesis
• Chronic osteomyelitis---
It is, therefore, imperative that all anterior teeth undergo pulp testing a
nd that serial radiographs are taken over time.
• During the mixed radiolucent‐radiopaque and completely radiopaque
phases,
1. Odontoma,
2. Sequestrum from a case of chronic osteomyelitis,
3. Ossifying fibroma,
4. Osteoblastoma
HISTOPATHOLOGY
Describe one additional feature that had not been reported previously:
the simultaneous occurrence of simple bone cysts in approximately
40% of their series of 34 cases of florid osseous dysplasia.
The suggested cause for the occurrence of these cysts is obstruction of
the normal interstitial fluid by the fibroosseous proliferation.
DIFFERENTIAL DIAGNOSIS
• Odontoma,
• Cementoblastoma,
• Osteoblastoma,
• Ossifying fibroma,
• Osteosarcoma.
• Condensing osteitis, a residual tooth root, or a bone scar from a
previous injury
FCOD OF
1. Hyperparathyroidism
2. Osteopetrosis
3. Ossifying fibroma
a) Introduction
b) Etiology/Pathogenesis
c) Clinical features of each type
d) Radiological features
e) Histological features
f) Differential diagnosis
g) Treatment and prognosis
Primary Hyperparathyroidism
Primary Hyperparathyroidism
• Caused by hypersecretion of parathyroid hormone (PTH)
• Hyperplastic glands
• Identified by hypercalcemia ≥10.5 mg/dl [2.6 mmol/L], after correction for serum
albumin (8.5 to 10.2 mg/dL)
• <5% of cases are recognized by the presence of an osteolytic defect with giant cells,
a condition referred to as a brown tumor.
• Hypercalcemia,
• Efforts should be made to keep serum calcium levels above 8.0 mg/dL
(2 mmol/L).
PROGNOSIS
Hypocalcemia
• These symptoms are usually coupled with the signs and symptoms of
their existing renal failure.
DIAGNOSTIC WORK‐UP
• Secondary hyperparathyroidism is suspected when a patient presents
with a history of dialysis and/or renal failure. It is confirmed by
demonstrating hypocalcemia, hyperphosphatemia, and elevated PTH
levels.
Histopathologic Features
Same spectrum of changes seen in florid cemento-osseous dysplasia,
and the two cannot be distinguished microscopically.
Massive mixed radiolucent/
radioopaque expansile lesions in both jaws.
Fibro-osseous pattern
with cementicles and boney trabecuae, the former oftern appearing
much larger that those seen in cemento-ossifying fibroma
Treatment and Prognosis
• Before the final sclerotic stage, attempts to improve aesthetics by
shave-down surgical procedures have not been successful because
the dysplastic tissue rapidly regrows.
3 PATTERNS
Osteitis deformans
Expansile lesions
Alk phosphatase
Cranial neuropathies
Polyostotic
Ground glass
Cotton wool
Mosaic bone
Cemento-osseous Dysplasia MELROSE et al
Non-expansile
Painless
African descent
3 patterns
Hyperparathyroidism
Parathormone, Ca
Renal disease
Ground glass
Multilocular Brown tumors
Massive opaque jaw lesions
Trabecular
Giant cell lesions
Osteopetrosis
Ossifying fibroma
Expansile, painless
Circumscribed lucent
Floccular opacities
Similar to cemento-osseous