Bone pathology

1

Bone Pathology

Dr. Arsalan MalikAssistant Professor (Oral Pathology)

حمن الر الله بسمحيم الر

2 Osteogenesis Imperfecta 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.

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.


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


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


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


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


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


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


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


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


The defects contain cellular hematopoitic or fatty marrow

Lymphoid aggregates may be present No abnormal osteoblastic /

osteoclastic activity


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


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


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


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


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


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


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


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


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


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


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 FeaturesIrregularly 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

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.


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

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


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


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


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


Osteomas are compact type

Individual lesion cannot be differentiated from solitary

osteomas


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


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.

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


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


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


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

Bone pathology

Health & Medicine

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