‫الر ِح ِيم‬

َّ ‫من‬
ِ ْ‫الرح‬
َّ ‫هللا‬
ِ ‫س ِم‬
ْ ‫ِب‬

Bone Pathology
1

Dr. Arsalan Malik

Assistant Professor (Oral Pathology)
 Osteogenesis Imperfecta
2

 It is a heterogeneous group of heritable diseases characterized by impairment of

collagen maturation.

 Collagen forms a major portion of bone, dentine, sclerae, ligaments and skin

 Osteogenesis imperfecta imparts problems in every structure made by collagen

 Abnormal collagen maturation results in thin cortex, fine trabeculation and diffuse
osteoporosis

 upon fracture, healing will occur but may be associated with excessive callus
formation
 3 Clinical & Radiographic Features

 Blue sclerae, altered teeth, hypoacusis, long bone, spine deformities and joint
hyperextensibility

 Radiographs show deformation of the long bones, multiple fractures and

wormian bones in the skull.
4 Clinical & Radiographic Features
5 Oral Manifestations

 Both dentitions are involved showing blue translucence

 Premature pulpal obliteration

 Class III malformations

 Multifocal radiolucencies

 Mixed radiolucencies and radio-opacities

6 Oral Manifestations
7 Types of Osteogenisis Imperfecta

 Type I

 Type II

 Type III

 Type IV
8 Type I Osteogenisis Imperfecta

 Most common and mildest form

 Mild to moderate bone fragility

 Most fractures occur during preschool time

 Hearing loss develops before 30

 Sclerae is blue and help in diagnosis

 Hypermobile joints and easy bruising

9 Type II Osteogenesis Imperfecta

 Most severe form

 Exhibits extreme bone fragility and frequent fracture

 many patients are still born and 90 % die before 4 week of age

 Opalescent teeth may be present

10 Type III Osteogenisis Imperfecta

 Moderately severe to sever bone fragility

 Sclerae is pale yellow or blue and fades as the child grows

 Hearing loss and ligament hyperextensibility present

 Many have normal teeth and some have opalescent teeth

11 Type IV Osteogenisis Imperfecta

 Mild to moderately severe bone fragility

 Sclera may be blue but fades with age

 In 50 % of patients fractures are present at birth

 Many have normal teeth and some have opalescent teeth

 Frequency of fracture decreases with age

12 Histopathological Features

 Bone architecture remains immature

throughout life

 Failure of woven bone to become

transferred to lamellar bone

 Teeth show abnormalities of dentine

similar to dentinogenisis imperfecta

 Teeth appear opalescent

13 Treatment

 There is no cure for this, symptomatic treatment is the only choice

 Management of fractures is a major problem

 Mainstay of treatment is physiotherapy, rehabilitation and orthopaedic surgery

 Medical treatment with Bisphosphonates provides relief from pain but its long term

use is not recommended

 In patients with severe malocclusion, orthognathic surgery is recommended

14 Osteopetrosis (Marble Bone Disease)

 It is a group of hereditary skeletal disorders characterized by marked

increase in bone density resulting from a defect in remodelling caused
by failure of normal osteoclast formation.

 Number of osteoclasts is normal but function is defective

 Continuous bone formation and defective resorption results in

thickening of bone.
 Clinical & Radiographic Features
15

Infantile Osteopetrosis
 Patients having disease at birth or early infancy usually have severe
disease called malignant osteopetrosis

 Facial deformity, broad face, hypertelorism, snub nose, frontal bossing.

 Narrowing of arches due to delayed eruption is common

 In dental radiographs roots of teeth are often invisible due to increased
density of bone
 Clinical & Radiographic Features
16

 Adult Osteopetrosis
 It is discovered later in life and exhibits less severe manifestations

 Inherited as autosomal dominant trait and termed benign osteopetrosis

 Approx 40 % of patients are symptom free and long bones are not
effected.

 Often It is diagnosed by increased bone opacity in dental radiographs

 Frequent fractures and cranial nerve compression are common

 Clinical & Radiographic Features
17
18 Histopathological Features

 Tortuous lamellar trabeculae

replacing the cancellous bone

 Globular amorphous bone

deposition in the marrow space

 Osteophytic bone formation

19 Treatment & Prognosis

 Adult Osteopetrosis is mild in severity and is associated with long term

survival as compared to infantile type where patients die during 1st decade of
life.

 Bone marrow transplant is the only hope for treatment

 Supportive measures include transfusion, antibiotics, drainage and surgical

debridement
20 Cleidocranial Dysplasia

 It is best known for its dental &

clevicular abnormalities

 It is caused by the mutation in gene

that is responsible for osteoblastic
differentiation and bone formation
21 Clinical & Radiographic Features

 Mainly the clevicles and skull are effected however other bones may be
involves.
Clevicles show varying degree of hypoplasia and malformation

 In 1 % of patients, clevicles are absent

 Patient’s appearance is diagnostic (Large head and frontal bossing)

 skull radiographs show delayed suture closure or wide open throughout life
22 Clinical & Radiographic Features
23 Histopathological Features

 Insufficient bone resorption is the

reason for impaired tooth eruption

 Some theories suggested impaired

cementum formation as a main
factor but it is not proved
24 Clinical & Radiographic Features

Patients often have a narrow high arched palate and increased chances of
cleft palate

Abnormal spacing in the lower incisors and delayed eruption of teeth

Supernumerary teeth are present

increased density of mandible and narrow maxillary arch

25 Clinical & Radiographic Features
26 Treatment & Prognosis

 No specific treatment exists for skull, clevicles and bone abnormalities

 Regarding dental problems, full mouth extraction with denture

construction, auto-transplantation of impacted teeth with prosthetic
restoration

 removal of the primary and supernumerary teeth followed by

exposure of permanent teeth
27 Focal Osteoporotic Marrow Defect

 An area of hematopoitic marrow that is sufficient in size to produce

an area of radiolucency which is confused with an intraosseous
neoplasm.

 It does not represent a pathological process

 Reasons may be

 Aberrant bone regeneration after tooth extraction

 Persistence of fetal marrow

 Marrow hyperplasia in response to increased demand for erythrocytes

28 Clinical & Radiographic Features

 Asymptomatic and purely an

incidental finding

 It appears a radiolucent area varying

in size

 Defect have ill defined borders and

fine central trabeculae

 No expansion of jaw is observed

29 Histopathological Features

 The defects contain cellular hematopoitic

or fatty marrow

 Lymphoid aggregates may be present

 No abnormal osteoblastic / osteoclastic

activity
30 Treatment & Prognosis

 Incisional biopsy often is necessary to establish diagnosis

 No treatment is necessary as it is not a pathological condition

31 Paget’s Disease of Bone

 It is characterized by abnormal and anarchic resorption &

deposition of bone resulting in weakening and distortion of
affected bones

 Inflammatory, genetic and environmental factors may play a role

in this disease
32 Clinical Features

 Men > Women

 Whites > Blacks

 Older > younger

 Asymptomatic disease is usually diagnosed in radiographs taken for

other reasons or serum alkaline phosphatase levels.

 Its frequency increases with age

33 Clinical Features

 Disease may be monostotic (limited to one bone) or polyostotic (multiple

bones involved)

 Bone pain is a frequent complaint

 Pegetic bone often forms near joints and promotes osteoarthritic changes with
associated joint pain and limited mobility
34 Clinical Features

 Jaw involvement is present in almost 17 % of cases

 Maxillary involvement results in mid face enlargement

 Nasal obliteration, enlarged turbinates, obliterated sinuses and

deviated septum

 Alveolar ridges become enlarged and create problems for denture

wearers
 35 Radiographic Features
 Initial stages show decreased radiodensity of bone and alteration of the trabecular pattern.

 During bone formation patchy areas of sclerotic bones formed giving a cotton wool
appearance in radiographs

 Teeth often exhibit hypercementosis

36 Histopathological Features

 Apparent uncontrolled alternating resorbtion and

deposition of bone.

 In active resorptive stage numerous osteoclasts

surround the trabeculae

 ‘’Reversal lines’’ mark the junction between

alternating resorptive and formative phase

 A highly vascular fibrous connective tissue replaces

the bone
37 Diagnosis

 Patients show raised levels of serum alkaline phosphatase but

normal levels of calcium and phosphate

 Serum alkaline phosphatase is considered the most specific marker

of bone formation

 Urinary hydroxyproline levels often are markedly elevated

 Lab parameters and clinical examination is sufficient for diagnosis

38 Treatment & Prognosis

 Asymptomatic patients usually do not need treatment

 In symptomatic cases, bone pain is controlled by acetaminophen or

NSAIDs

 Neurological complications are due to pressure of bony structures on

cranial nerves. Use of Parathormone (PTH) antagonists can reduce bone
turnover and improve the biochemical parameters.

 Dental patients may require new dentures due to increased size of

alveolar ridges.
39 Central Giant Cell Granuloma

 It is considered to be a non neoplastic lesion

 60 % cases arise before 30 years

 Females > males

 70 % cases arise in mandible

 Anterior portion > posterior

40 Clinical Features

 Some cases are asymptotic and diagnosed in routine radiographs

 Symptomatic cases represent pain, paraesthesia or perforation of cortical

plate resulting in ulceration of overlying mucosa

 Nonaggressive lesion
 Exhibit few or no symptoms, slow growth, no cortical perforation / root resorption

 Aggressive lesions
 Pain. Rapid growth, cortical perforation, tendency to recur
41 Radiographic Features
 Radiolucent defects which may be unilocular / multilocular

 Range from 5 mm to 10 cm in diameter

 D/D of unilocular lesions

 Perioapical granuloma

 Periapical cyst

 D/D of multilocular lesions

 ameloblastoma
42 Histopathological Features

 Multinucleated giant cells in back ground of ovoid mesenchymal cells &

round monocyte-macrophages

 Stroma is loosely arranged and edematous

 Areas of erythrocyte extravasation and hemosiderin deposition are

prominent

 Foci of osteoid and newly formed bone are occasionally present in lesion
43 Histopathological Features
44 Treatment & Prognosis

 Usually treated by thorough curettage

 Aggressive lesion show more tendency to recur

 They require radical surgery for cure

 Three alternatives to surgery are

 Corticosteroids

 Calcitonin

 Interferon alfa-2a
45 Cherubism

 Rare developmental disorder

generally inherited as autosomal
dominant trait

 Males > females

46 Cherubism

 It occurs due to spontaneous mutation in gene that codes for

protein responsible for activity of osteoblasts & osteoclasts during

bone formation

 The term Cherubism is used because of pulmp-cheeked little angles

depicted in some paintings

47 Clinical Features
 Usually occur between 2-5 years of age

 Clinical alterations progress till puberty then stabilize and slowly regress

 Bilateral involvement of the posterior mandible that gives chubby cheeks

 ‘’Eye upturned to heaven’’ appearance due to wide rim of sclera noted

below the iris

 Marked cervical lymphadenopathy

48 Clinical Features

 Mandibular lesions typically appear as painless, bilateral expansion of

the posterior mandible

 Bilateral expansion is symmetrical

 Maxillary tuberosity area may be involved

 Widening and distortion of alveolar arches

 Failure of eruption, impair mastication, speech difficulties, loss of

normal vision
49 Radiographic Features
 Multilocular, expansile radiolucencies

 Appearance is virtually diagnostic because of bilateral location

 It typically involves the jaws but ribs & humerus may be involved
 50 Histopathological Features
 Lesional tissue consist of vascular fibrous tissue
containing variable number of multinucleated
giant cells

 Giant cells are small and arranged focally

 Foci of extravasated blood is common

 Stroma tends to be loosely arranged

 In older resolving lesions, tissues become more

fibrous, the number of giant cells decreases and
new bone formation is seen.
51 Treatment

 In many cases lesion is self limiting after puberty

 In some patients the recovery is very slow and in some the deformity
may persist

 In some cases surgical curettage may result in early recovery and in

some cases it worsens the situation

 Radiation therapy is contraindicated because of risk of development

of postirradiation stroma
52 Fibro-Osseous Lesions of the Jaws
 It is a diverse group of processes that are characterized by replacement of normal
bone by fibrous tissues containing a newly formed mineralized product.

 Fibrous Dysplasia

 Cemento-Osseous Dysplasia

 Focal Cemento-Osseous Dysplasia

 Periapical Cemento-Osseous Dysplasia

 Florid Cemento-Osseous Dysplasia

 Ossifying Fibroma
53 Fibrous Dysplasia

 A developmental tumour like condition characterized by replacement

of normal bone by an excessive proliferation of cellular fibrous
connective tissue intermixed with irregular bony trabeculae

 It may involve one bone, multiple bones with or without cutaneous &
endocrine manifestations

 Degree of severity depends upon time of mutation

54 Clinical Features

Monostotic Fibrous Dysplasia

 80 – 85 % of cases

 Limited to single bone

 Male = females

 Jaws are most commonly effected

 Maxilla > mandible

 Painless swelling of jaws

55 Radiographic Features
 Ground glass appearance due to poorly calcified bony trabeculae arranged in a
disorganized pattern

 In mandible, superior displacement of inferior alveolar canal is observed

 In maxilla, sinus floor shifts superiorly

56 Clinical Features

Polyostotic Fibrous Dysplasia

 Relatively uncommon

 Combined with café au lait pigmentation

is termed as jaffee-Lichtenstein
syndrome

 Combined with café au lait pigmentation

and endocrinopathies is termed McCune-
Albright syndrome
57 Clinical Features

Polyostotic Fibrous Dysplasia

 Pathological fractures are common

 Pain and leg length discrepancy is common

 In Mc-Cune-Albright Syndrome, sexual disturbances are

observed in females as endocrine manifestations
58 Histopathological Features

Irregularly shaped trabeculae of immature bone in

a cellular, loosely arranged fibrous stroma

Bony trabeculae are not connected to each other

and assume a Chinese Script writing appearance

Lesional bone directly fuse to normal bone

without any capsule

Jaw and skull lesions undergoes maturation with

time
59 Treatment

 Smaller lesions are easily resected without difficulty

 Larger lesions are difficult to resect completely

 Some lesion tend to regress with time but cosmetic

problems are addressed through surgery
60 Cemento-Osseous Dysplasia

 Occurs in tooth bearing areas of the jaws and most common fibro
osseous lesion in clinical practice

 Some believe it arises from periodontal ligament and some believe it

arises from defect in extra-ligamentry bone remodelling.

 Three types are observed

 Focal

 Periapical

 Folrid
61 Focal Cemento-Osseous Dysplasia

 Single site of involvement

 3rd – 6th decade

 90 % in females

 Asymptomatic , smaller than 1.5 cm in

diameter

 Occur in posterior mandible

 Lesion varies from completely

radiolucent to dense radio-opaque
62 Periapical Cemento-Osseous Dysplasia

 Periapical region of anterior mandible

 Female > Males

 3rd – 5th decade

 Asymptomatic radiolucency covering the

roots of several teeth

 Lesions tend to mature with time

 Does not expand the jaws

63 Florid Cemento-Osseous Dysplasia

 Multifocal involvement not limited to anterior mandible

 Bilateral symmetrical involvement

 May remain asymptomatic or manifest as dull pain and bony expansion

 Both edentulous and dentulous areas are involved regardless of

presence of teeth
64 Florid Cemento-Osseous Dysplasia
65 Histopathological Features
 All three patterns of cemento osseous dysplasia demonstrate similar
histopathological features

 Tissues consist of fragments of cellular mesenchymal tissues composed of

spindle shaped fibroblasts and collagen fibres

 Free haemorrhage is typically noted interspersed throughout lesion

 Within this fibrous connective tissue background is a mixture of woven bone,

lamellar bone and cementum like particles

 As the lesion matures, the amount of mineralized material increases

66 Histopathological Features
67 Diagnosis

 In most instances, the distinctive clinical & radiographic picture of periapical &
florid types allow a strong preseumptive diagnosis without need of biopsy

 In case of focal type. Features are less specific and often require surgical
intervention

 Findings from surgery are helpful in discrimination between focal cement-

osseous dysplasia and ossifying fibroma
68 Treatment

 They are not neoplastic so they usually do not need treatment

 The best management option for asymptomatic patient is regular follow

up & maintaining better oral hygiene to avoid periodontal disease.

 In symptomatic patients, antibiotics are given but not effective because of

dead sclerotic bone.

 Saucerization of the dead bone may speed healing.

69 Ossifying Fibroma

 It’s a true neoplasm with significant growth potential

 Composed of fibrous tissue containing variable mixture of bony

trabeculae and cementutum like spherules.

 The cementum like material in the lesion is variation of bone.

70 Clinical Features

 Majority of cases are present in 3rd & 4th decade

 Female predilection

 Mandible > maxilla

 Most common in peremolar & molar area

 Smaller lesions are asymptomatic but large lesions cause painless swelling
of involved bone

 Pain & paraesthesia are rarely present

71 Radiographic Features

 Well defined and unilocular

 May appear completely radiolucent because of calcified material

produced in tumour

 True lesions are completely radio-opaque with thin radiolucent

border.

 Root resorption or resorption may be seen

72 Radiographic Features
73 Histopathological Features
 Lesion is well demarcated from
surrounding bone

 Some are encapsulated others are not

 Tumour is usually submitted in one large

or few small pieces

 It consists of fibrous tissues of varying

cellularity and mineralized material hard
tissue portion consist of bone or
cementum like material

 Intra-lesional haemorrhage is unusual

74 Treatment

 Circumscribed nature of lesion allows enucleation of tumour with

ease

 Large tumours may need surgical resection and bone grafting

 Chances of recurrence are very rare

75 Osteoma

 Benign tumours composed of mature compact or cancellous bone

 These are strictly restricted to craniofacial region

 Common palatal & mandibular tori are not considered as osteomas

76 Clinical Features

 Osteoms of the jaws arise from surface of the bone or they may be present
in medullary bone

 Asymptomatic and present in younger age

 Body of the mandible, condyle are common locations

 Condylar osteoms are considered to be true neoplasms

 They disturb the occlusion by deviating the jaw to one side

 Facial swelling, pain and limited mouth opening are other signs
77 Radiographic features

 They appear as circumscribed sclerotic masses

 Periosteal osteomas may exhibit uniform sclerotic pattern or sclerotic

margins with central trabeculae

 Smaller osteomas are difficult to differentiate from foci of sclerotic

bone

 The true mature osteomas can be confirmed by observing continued

growth
78 Radiographic features
 79 Histopathological Features

 Compact osteomas are composed of

normal appearing dense bone showing
minimal marrow tissue

 Cancellous osteomas are composed of

trabeculae of bone and fibrofatty
marrow.

 Osteoblastic activity may be fairly

prominent
80 Treatment

 Larger osteomas of the mandibular body causing symptoms or

cosmetic deformity are treated by surgical excision

 Smaller asymptomatic osteomas present endosteally do not need

treatment but should be observed periodically

 Condylar osteomas are surgically removed

 Osteomas are completely benign and patients do not experience

malignant change
81 Gardner Syndrome

 A rare disorder that is inherited as an autosomal dominant

trait. Approximately 1/3rd cases represent spontaneous

mutations

 it is considered to be the part of a spectrum of diseases

characterized by colorectal polyposis

82 Clinical Features

 The associated colonic polyps develop in 2nd decade of life.

 They are adenomatous, they transform into adenocarcinomas.

 Common skeletal abnormalities include osteomas

 Skull, paranasal sinus and mandible are the commonly affected sites

 Patients demonstrate 3 – 6 lesions

 Osteomas appear as areas of increased radiodensity

 Large osteomas will limit mandibular opening

83 Clinical Features
84 Clinical Features

 Dental abnormalities include multiple osteomas, multiple impacted teeth and

supernumerary teeth

 Frequency of extra teeth is low as compared to cleidocrania dyaplasia

 Patients show epidermoid cysts of skin

 Increased prevalence of thyroid carcinomas is also noted

85 Histopathological Features

 Osteomas are compact type

 Individual lesion cannot be differentiated from solitary

osteomas
86 Treatment & Prognosis

 The major problem is malignant transformation of colonic polyps

in adenocarcinoma

 Prophylactic colectomy is recommended

 Removal of jaw osteomas & epidermoid cysts are advised for

cosmetic reasons
87 Osteoblastoma & Oteoid Osteoma

 These both are closely related tumours that represent identical

histopathological features but different clinical behaviours

 Osteoid osteoma contain a concentration of peripheral nerves not seen in

osteoblastoma

 It produces prostaglandins that results in pain which is relieved by

prostaglandin inhibitors like asprin.

 osteomas are smaller than 2 cm & osteoblastoma is bigger than this

88 Clinical Features (Osteoblastoma)

 Most commonly affected bone are vertebral column, sacrum,

calvarium, long bones & small bones of hand & foot
 Occur before 30 years
 Female predominance
 Posterior region of mandible
 2 – 10 cm
 Pain, swelling & tenderness may lead to tooth mobility , root
resorbtion and tooth displacement
89 Radiographic Features (Osteoblastoma)

 Well defined or ill defined radiolucent lesion often having a patchy areas of
mineralization.

 Most osteoblastomas arise within the medulary bone however it may present as bony
projections from the periosteum of bone ( periosteal osteoblastomas)
90 Clinical Features (Osteoid Osteoma)

 Occur in femur, tibia & phalanges

 Very rare in jaws & pain is nocturnal &

relieved by salicylates

 Mostly present out of the jaws

(extragnathic lesions)
 91 Histopathological Features
 Mineralized material that demonstrate reversal lines

 At the periphery of large masses there are

multinucleated osteoclast like cells and numerous
osteoblasts

 The supporting stoma consist of loose fibrous

connective tissues that contain loose vascular
channels

 Aggressive osteoblastomas are marked by large

epitheliod cells with increased mitotic activity.
92 Treatment

 Mostly cases of osteoblastoma & osteoid Osteomas are treated by

surgical excision or curettage

 Prognosis is good and some lesions will regress even after

incomplete excision
93 Cementoblastoma

 It is an odontogenic neoplasm of ameloblasts

 No gender predilection

 Mostly involves premolar & molar area of mandible

 Signs of aggressive behaviour like bony expansion, cortical

erosion tooth displacement and infiltration into pulp chamber
may be observed
94 Radiographic Features
 Radio-opaque mass fused to one or more roots and is surrounded by a thin radiolucent
rim

 The outline and roots of the involved tooth is usually obscured as a result of root
resorption & fusion of tumour with tooth
95 Histopathological Features

 It resemble osteoblastoma with the primary distinguishing feature of fusion

with the involved tooth.

 Majority of the tumour consist of sheets and thick trabeculae of mineralized

material with irregularly placed lacunae & prominent basophilic reversal
lines.

 Multinucleated giant cells are often present

 Periphery of the lesion is composed of non calcified material which appears

as radiolucent rim on radiograph.
96 Histopathological Features
97 Treatment

 Surgical Extraction of the tumour with involved tooth

 Surgical excision of the mass with root amputation and

endodontic treatment may be considered.

 Total removal of mass with tooth is closely related to chances of

recurrence.
98 Osteosarcoma

 It is a malignancy of mesenchymal cells that have the ability to

produce osteoid or immature bone

 Majority of osteosarcomas demonstrate intramedullary origin

but a small number may be justracortical
99 Clinical Features

 It shows a bimodal age distribution 10 – 20 years and after 50 years

 Initial peak occurs during the period of greatest bone growth and
involves proximal tibial metaphyses

 In older age axial skeleton and flat bones are involved.

 Peget’s disease & previous irradiation are associated with increased

prevalence
100 Clinical Features

 Maxilla = Mandible

 In mandible most tumours arise in posterior region

 Maxillary tumours usually arise in inferior portion as compared to superior

portions

 Swelling & pain are the most common symptoms.

 Loosening of teeth, paresthesia and nasal obstruction can be seen

101 Clinical Features
102 Radiographic Features

 It varies from dense sclerosis to a mixed sclerotic and radiolucent lesion

to an entirely radiolucent process

 Peripheral border of the lesion is ill defined and indistinct making it

difficult to distinguish the lesion

 The classis sunburst appearance caused by osteophytic bone production

on the surface of the lesion is noted in about 25 % of jaw osteosarcomas
103 Radiographic Features