FIBRO OSSEOUS LESION

INTRODUCTION:
FIBRO-OSSEOUS LESIONS ARE A POORLY DEFINED GROUP OF LESIONS AFFECTING THE
JAWS AND CRANIOFACIAL BONES. ALL ARE CHARACTERIZED BY THE REPLACEMENT
OF BONE BY CELLULAR FIBROUS TISSUE CONTAINING FOCI OF MINERALIZATION THAT
VARY IN AMOUNT AND APPEARANCE. CLASSIFICATION AND, THEREFORE, DIAGNOSIS
OF THESE LESIONS IS PROBLEMATICAL, PARTLY BECAUSE OF A LACK OF AGREEMENT
ABOUT TERMINOLOGY, BUT ALSO BECAUSE OF A SIGNIFICANT OVERLAP IN
HISTOLOGICAL FEATURES. THE GROUP INCLUDES DEVELOPMENTAL AND REACTIVE OR
DYSPLASTIC LESIONS AS WELL AS NEOPLASMS. A NUMBER OF WORKERS HAVE TRIED TO
CLARIFY THE CLASSIFICATION OF THESE LESIONS1, 2, 3, 4, 5, 6, 7 AND ALTHOUGH THEY
MAY NOT HAVE AGREED ON AN EXACT TERMINOLOGY, A CONCEPT HAS EMERGED
WHICH HAS CULMINATED IN THE LATEST WHO CLASSIFICATION.8 THE CORE OF THIS
CLASSIFICATION IS THE CONCEPT OF A SPECTRUM OF CLINICOPATHOLOGICAL
ENTITIES IN WHICH THE DIAGNOSIS CAN ONLY BE MADE ON THE BASIS OF A FULL
CONSIDERATION OF CLINICAL, HISTOLOGICAL AND RADIOLOGICAL FEATURES.
•
Definition:
Fibro-osseous lesions (FOL) are a poorly defined group of lesions affecting the jaws and craniofacial bones. All are
characterized by the replacement of bone by cellular fibrous tissue containing foci of mineralization that vary in
amount and appearance

Fibro-osseous lesions refer to a collection of nonneoplastic intra-osseous lesions that replace normal bone and
consist of a cellular fibrous connective tissue within which non-functional osseous structures form.
• Classification :
. Fibrous dysplasia

. Periapical cemento – osseous dysplasia

.focal cemento – osseous dysplasia
.florid cemento –osseous dysplasia

.familial gigantiform cementoma

.fibro –osseous neoplasm:
-cementifying fibroma
-ossifying fibroma
-cemento – ossifying fibroma

-juvenile ossifying fibroma

.Cherubism
 cherubism
.Cherubism is a skeletal dysplasia characterized by bilateral and symmetric fibro-osseous lesions
limited to the mandible and maxilla. In most patients, cherubism is due to dominant mutations in
the SH3BP2 gene on chromosome

.Hereditary disease
. It is a autosomal dominant fibro-osseous benign hereditary condition which affects only the jaw bones
and it is characterized by "bilaterally symmetrical enlargement" of mandible sometimes maxilla
PATHOGENESIS
 classification
The lesions of cherubism can be classified according to their extent:

. grade 1: bilateral involvement of the ascending ramus of mandible

.grade 2: bilateral involvement of the ascending ramus of mandible and maxillary tuberosity
. grade 3: complete involvement of the maxilla and mandible compromising the coronoid processes and
condyles
Clinical Features :
 RADIOGRAPHIC FEATURES
Photograph of a 7 year old boy with cherubism showing
bilateral swelling at the mandibular angles.

Orthopantomograph of the same patient as in Figures 1. Note the bilateral swelling caused by expansion
related to multilocular bone lesions of the angle and ascending ramus of the mandible and coronoid
process
Photograph of a 9 year old boy with cherubism showing bilateral swelling of the angle and ascending ramus of the
mandible, stretching the skin in the nasogenial region

Orthopantomograph of the same patient as in Figures 3. The image reveals bilateral swelling of
the angle, ascending ramus of the mandible and coronoid process caused by multilocular bone
lesions. Note the lack of involvement of the condyles
Orthopantomograph of the same patient, revealing radiographic alterations characterized by
greater radiopacity resulting from bone deposition and confirming the lack of involvement of the
condylar regions. Note the malpositioning and retention of teeth
Histopathological features

 The microscopic appearance of cherubism is similar to that of giant cell

lesions.
 It consists of cellular and vascular fibrous tissue containing many giant
cells that may be focally arranged or scattered.
 In older resolving lesions of cherubism, the tissue becomes more fibrous,
the number of giant cells decreases, and new bone formation is seen.
 Eosinophilic cuff-like perivascular deposits which appears to be specific to
cherubism but not a consistent finding.
Multinucleated giant cells with eosinophilic perivascular cuffing. Eosinophilic cuffing
appears to be specific to cherubism, yet not a constant finding
LABORATORY FINDINGS :ALONE ARE NOT TYPICALLY USED FOR
DIAGNOSING CHERUBISM. HOWEVER, IMAGING STUDIES AND CLINICAL EXAMINATION ARE THE
PRIMARY TOOLS FOR DIAGNOSIS
DIFFERENTIAL DIAGNOSIS:
. FIBROUS DYSPLASIA

.GIANT CELL GRANULOMA

. ANEURYSMAL BONE CYST

. MULTIPLE ODONTOGENIC KERATOCYSTS

. AMELOBLASTOMA

.ODONTOGENIC MYXOMA
 FAMILIAL GIGANTIFORM CEMENTOMA

.FAMILIAL GIGANTIFORM CEMENTOMA IS A RARE FIBRO-CEMENTO-OSSEOUS LESION THAT CAN CAUSE MARKED EXPANSION
OF FACIAL SKELETON WITH AN AUTOSOMAL DOMINANT PATTERN OF INHERITANCE AND VARIABLE PHENOTYPIC EXPRESSION

. IT IS A DISORDER OF GNATHIC BONE THAT LEADS TO FORMATION OF MASSIVE SCLEROTIC MASSES OF DISORGANISNIZED

MINERALIZED PRODUCTS .
CLINICAL FEATURES:
• ANTERIOR MANDIBLE.

• NO SEXUAL PREDILECTION.

• NO RACIAL PREDILECTION.

• BEGINS DURING 1ST DECADE OF LIFE AND CAN GROW RAPIDLY.

• INVOLVEMENT OF BOTH MAXILLA AND MANDIBLE.

• GNATHIC ENLARGEMENT RESULT IN FACIAL DEFORMITY, AS WELL AS IMPACTION, MALPOSITION

AND MALOCCLUSION OF THE INVOLVED DENTITION.
 RADIOGRAPHIC FEATURES:
.MULTIPLE RADIOLUCENCIES IN PERIAPICAL REGIONS .

.EXPANSION OF AFFECTED SIDE AND DEVELOP MIXED RADIOLUCENT AND RADIOPAQUE

PANORAMIC RADIOGRAPHY SHOWING PERIAPICAL LESIONS AND MISSING TEETH.

Histopathological
features:

characterized by cemental-like
deposits in abundant fibroblastic
tissue, often accompanied by
irregular bone formation.

Although familial gigantiform

cementoma is defined as a genetic
inherited disorder, single cases of
gigantiform cementoma without a
family history have been reported
LABORATORY FINDINGS :ALONE ARE NOT TYPICALLY USED FOR
DIAGNOSING FGC. INSTEAD, DIAGNOSIS IS PRIMARILY BASED ON CLINICAL AND
RADIOGRAPHIC FINDINGS. LABORATORY TESTS MAY BE ORDERED TO ASSESS OVERALL
HEALTH STATUS AND RULE OUT OTHER CONDITIONS, BUT THEY ARE NOT SPECIFIC TO
FGC.
 DIFFERENTIAL DIAGNOSIS :
• PAGET'S DISEASE

• CHRONIC SCLEROSING OSTEOMYELITIS

• SCELEROTIC CEMENTAL MASSES

• CHRONIC PRODUCTIVE OSTEITIS

• OSSEOUS DYSPLASIA
 PERIAPICAL CEMENTO- OSSEOUS DYSPLASIA
. PERIAPICAL CEMENTO-OSSEOUS DYSPLASIA (COD) IS A VERY RARE BENIGN LESION ARISING FROM A GROUP OF
DISORDERS WHICH ARE KNOWN TO ORIGINATE FROM UNDIFFERENTIATED CELLS OF THE PERIODONTAL LIGAMENT TISSUE.

. ALSO KNOWN AS CEMENTOMA, OSSEOUS DYSPLASIA AND PERIAPICAL CEMENTAL DYSPLASIA.

• CLINICAL FEATURESINVOLVES PERAPICAL REGION OF ANTERIOR MANDIBLE
• ASYMPTOMATIC

. PERIAPICAL COD OCCURS MORE FREQUENTLY IN WOMEN OF BLACK RACE (, AND ABOVE 40 YEARS OF AGE. THE LESIONS
MAY BE SINGLE OR MULTIPLE, ASYMPTOMATIC AND DO NOT INVOLVE ALTERATIONS TO THE PERIODONTAL TISSUE. THE
PREVALENT LESION SITE IS THE ANTERIOR REGION OF THE MANDIBLE, IN THE VICINITY OF THE ROOT APEX OF THE MANDIBULAR
INCISORS AND CANINES, AND THE TEETH INVOLVED REMAIN VITAL . TYPICALLY, THE LESION DEVELOPS THROUGH THREE
PHASES: OSTEOLYTIC; CEMENTOBLASTIC; AND MATURE
RADIOGRAPHIC FEATURES:
• 3 RADIOGRAPHIC FEATURES INDICATES STAGE FROM EARLY FORMATION TO MATURATION:

• 1. OSTEOLYTIC STAGE: IN THIS EARLY STAGE, LESION ARE WOLL DEFINED RADIOLUCENCIES AT APEX OF ONE OR MORE
TEETH.

• 2. CEMENTOBLASTIC STAGE: DISPLAYS SIMILARLY SIZED LESIONS AND A DEMARCATED BORDER WITH IDIOLUCENCIES
CONTAINING NODULAR RADIOPAQUE DEPOSITS.

• 3. MATURE STAGE: WELL DEFINED, DENSE RADIOPACITIES EXHIBIT SOME NODULARITY. EACH RADIOPAQUE NODULE HAS
THIN RADIOLUCENT ZONE AROUND ITS PERIPHERY THAT SEPARATES IT FROM THE SURROUNDING BONE AND NEARBY TEETH.
PERIAPICAL RADIOGRAPH SHOWING MULTIPLE AND RADIOPAQUE RADIOLUCENT PATTERN AT THE APICES OF MANDIBULAR TEETH.
Histology at a later stage of the lesion
showing sclerotic mass of cemento-osseous
material (hematoxylin-eosin stain)
All types are fibro-osseous in nature with
cellular fibrous tissue containing woven bone
trabeculae and islands of dense cementum-
like bone. Progressive calcification leads to
the formation of a solid, bony mass with
prominent resting and reversal lines.

The arrows illustrate

acellular segments of
cementum-like substances
in the loose fibro-
collagenous stroma. No
osteoclastic activity is
shown in the unmarked
lamellar bony formations
LABORATORY FINDINGS : ARE NOT TYPICALLY INVOLVED IN THE
DIAGNOSIS OF PCOD. INSTEAD, DIAGNOSIS IS USUALLY MADE BASED ON CLINICAL AND
RADIOGRAPHIC FINDINGS.
DIFFERENTIAL DIAGNOSIS
RADIOLUCENT STAGE:
• APICAL PERIODONTAL GRANULOMA OR A RADICULAR CYST
• PRIMORDIAL ODONTOGENIC CYST
• EARLY PHASE OF OSSIFYING FIBROMA
• CHRONIC OSTEOMYELITIS IF 4 TO 6 ANTERIOR TEETH ARE INVOLVED) MIXED STAGE AND RADIOPAQUE STAGE:
• ODONTOMA
• CHRONIC OSTEOMYELITIS
• OSSIFYING FIBROMA
• OSTEOBLASTOMA
.
FIBRO-OSSEOUS LESIONS SHARE CLINICAL, RADIOLOGICAL, AND PATHOLOGICAL FEATURES, PARTICULARLY IN THE
CRANIOFACIAL COMPLEX, A LOCATION WHERE DIAGNOSING THESE LESIONS IS CHALLENGING. SOME MOLECULAR
MARKERS INVOLVED IN THE PATHOGENESIS OF THESE LESIONS OFFER SIGNIFICANT DIAGNOSTIC SUPPORT. GUANINE
NUCLEOTIDE-BINDING PROTEIN/ALPHA SUBUNIT (GNAS) MUTATIONS ARE SPECIFIC TO FIBROUS DYSPLASIAS AND ARE NOT
ENCOUNTERED IN OSSIFYING FIBROMAS (INCLUDING JUVENILE VARIANTS), OR IN LOW-GRADE AND HIGH-GRADE
OSTEOSARCOMAS OF THE JAW. LOW-GRADE AND DEDIFFERENTIATED OSTEOSARCOMAS SHOW MURINE DOUBLE-MINUTE 2
(MDM2) AMPLIFICATION, WITH OVEREXPRESSION OF MDM2, ABNORMALITIES NOT ENCOUNTERED IN FIBROUS DYSPLASIA, OR
IN OSSIFYING FIBROMA. NO RECURRENT CYTOGENETIC OR MOLECULAR ABNORMALITY HAS BEEN REPORTED TO DATE IN
CONVENTIONAL OSSIFYING FIBROMAS. THE JUVENILE VARIANTS OF OSSIFYING FIBROMA EXHIBIT MDM2/RAS PROTEIN
ACTIVATOR LIKE-1 (RASAL1) CO-AMPLIFICATION, WITHOUT MDM2 OVEREXPRESSION, ABNORMALITIES ALSO OBSERVED IN
CERTAIN AGGRESSIVE
 PRESENTED BY:
.MAHMOUD NAGAR 200036723
.ESRAA SALAH 200037027
.NADA AHMED 200037093
.DINA GALAL 200037349
.MANAR KASSEM 200045137
.MOHAMED TAREK 200045575
.YOUSSIEF ABDELLATTIF 200045557
.SARA IBRAHIM 200045892
.AMNA MAHMOUD 200048378
.MOHAND KAMAL 200048406