Giant cell lesions in maxillofacial region from cawson essential of oral pathology

Contents
INTRODUCTION
FORMATION OF GIANT CELL
TYPES OF GIANT CELLS
CLASSIFICATION OF GIANT CELL LESIONS
GIANT CELL LESIONS IN THE ORAL CAVITY
CONCLUSION
REFERENCES

A giant cell is a cell
that is larger in
dimension than the
cells that are routinely
encountered in
histology.
These cells are involved
in many physiologic and
pathological processes.
The giant cells may be
mono-nucleated or
multi-nucleated .
They are easily
recognized under light
microscopy and hence
provide a vital clue in
arriving to a diagnosis.
Introduction

Concepts on the fusion
of giant cells;
1.Immune-mediated
concept –
lymphokinesand cell
surface changes
facilitate fusion of
macrophages
2. Young and old
macrophage concept –
recognition of old
macrophages, which
shows chromosomal
abnormality and
altered cell surface
which stimulates the
fusion process
between old and
young macrophages
3.Endocyticactivity
concept – two or more
macrophages try to
ingest or engulf the
same antigen the
resulting phagocytosis
causes fusion of
endosome margins
between two cells.[1]

Osteoclasts
 Named by KOLLIKER are bone resorbing cells that play a
pivotal role in bone homeostasis and remodeling.
 Osteoclast precursors are derived from bone marrow as
easily mononuclear macrophages , circulate in blood and bind
to surface of bone.
 Osteoclast formation driven mainly by two cytokines ,
 Receptor Activator of Nuclear Factor Kappa b Ligand [
RANKL]
 Macrophage – colony stimulating factor [ M-CSF]
 Systemic hormones and growth factors influence formation
and function of osteoclast 8

Tumor giant cells
 Many epithelial and mesenchymal
neoplasm's contain tumor giant cells.
 The nuclei of these giant cells are
pleomorphic, often diploid, shows
abnormal mitosis and resemble those
of mononuclear tumor population.
 Tumor cells are known to possess an
abnormal surface and are predisposed
to fusion in different ways
9

Touton giant cells
 Touton giant cells are characterized by
multiple nuclei that cluster together in the
cell and are surrounded by foamy cytoplasm.
 These cells were originally known as
xanthelasmatic giant cells and are formed by
fusion of macrophage derived foam cells.
 These MGCs are most frequently found in
lesions containing cholesterol and lipid
deposits, and are associated with xanthomas
and xanthogranulomas. 10

Langhans’ giant cells
 Langhans’ giant cells are characterized by
the presence of few nuclei (< 20) arranged
peripherally, within the giant cell.
 They are commonly found in immune
granulomas and granulomatous inflammations
in the presence of indigestible particles of
organisms, eg: the tubercle bacillus.
 The presence of MGCs in the tuberculous
granuloma was first described by Langhans in
1868.
11

Foreign body giant cells
 Unlike osteoclasts, which adhere to bone,
FBGCs, together with their macrophage
precursors, adhere to markedly different
synthetic surfaces that display distinct
differences in hydrophilic/hydrophobic
character as well as chemical and physical
properties.
 FBGCs contain many nuclei (up to 100 200)
that are arranged in a diffuse manner
throughout the cytoplasm. 12

13
Lesions where giant cells in the
concerned background are
pathognomic
Lesions where giant cells are
characteristic but not
pathognomic
Lesions associated with presence of giant cells
Hodgkins lymphoma
Peripheral/central giant cell
granuloma
Giant cell fibroma
Tuberculosis
Herpes Simplex Virus
infection
Measles
Xanthoma
Orofacial granulomatosis, fungal infection, foreign
body reaction, neoplasm, syphilis, leprosy, fibrous
dysplasia, cherubism, ossifying fibroma, aneurysmal
bone cyst, paget’s disease of bone, wegners
granulomatosis actinomycosis, odontogenic giant cell
fibromatosis
Ranjan V, Chakrabarty S, Arora P, Rastogi
T. Classifying giant cell lesions: A review.
Journal of Indian Academy of Oral
Medicine and Radiology. 2018 Jul
1;30(3):297.
Classification of giant cell lesions

14
1;30(3):297.

1;30(3):297.

Tuberculosis
Tuberculosis is a specific infectious granulomatous disease
caused by mycobacterium tuberculosis.
 Tuberculous lesions of oral cavity may be primary or
secondary to pulmonary TB
 Although tongue is the commonest site for oral
tuberculous lesions, they may also occur on gingiva, floor
of mouth, palate, lips and buccal mucosa.
 The typical oral lesions consist of a stellate ulcer with
undermined edges and a granulating floor.
16

17
Clinical features: Typical oral lesions are
chronic painless
 ulceration or swelling,
 non-healing extraction sockets,
 intrabony mandibular swelling
Histopathologic features:
Central caseous necrosis
 tubercle granuloma formation
 collection of epithelioid cells,
 histiocytes, lymphocytes, and
multinucleated Langhans
 type of giant cells are found that are
indicative of
 Tuberculosis.

Associated giant cell: Classically activated
macrophages (M1) and Langhans type of giant cell
Diagnosis
 presence of acid-fast
 bacilli in the specimen collected,
 tuberculin skin test,
 molecular techniques like PCR.
 Marker:
 They are positive for CD68+, Langhans-type DC-STAMP.
 The marker for acute tuberculosis is CD40+,
 chronic tuberculosis the marker is myeloid differentiated primary
response
Treatment-

Leprosy
Leprosy is a chronic granulomatous infection caused by
Mycobacterium leprae
Clinical features:
 Hypopigmented patches,
 partial or total loss of cutaneous sensation in the affected
areas.
 Facial paralysis, leonine facies,
 plantar ulcers and loss of fingers and toes
19

:
 Lepromatous leprosy demonstrates ill formed granuloma.
 The typical finding is sheets of lymphocytes intermixed with
vacuolated histiocytes known as leprae or Virchow cell
Associated giant cell: Langhans type of giant cells can be variably
present
Marker: They show positivity for CD68
Treatment:
20

Actinomycosis
Actinomycosis is a chronic suppurative soft-tissue infection caused by
Actinomyces israelii,
 Clinical manifestations of actinomycosis occur in three areas:
cervicofacial (50%), abdominal-pelvic (23%), and thoracic (17%).
 Suppurative reaction of the infection may discharge large yellowish
flecks that represent colonies of bacteria called sulphur granules.
 Cervicofacial actinomycosis affects the areas of prior trauma, due
to soft tissue injury, periodontal pocket, non vital tooth, extraction
socket or infected tonsil.
21

Histopathologic features
 A central abscess formation with colonies of
microorganisms floating in a sea of polymorphonuclear
leukocytes is observed,
 Often associated with multinucleated giant cells and
macrophages particularly around the periphery of the
lesion.
Diagnosis:
 Fine-needle aspiration biopsy
 Actinomycosis colonies or sulfur granules in microscopic
examination.
Treatment:
22

Sarcoidosis
 Sarcoidosis, in Greek meaning “flesh like condition” is a
systemic non caseating granulomatous disease of unknown
etiology, although genetic, infectious and environmental
factors have been postulated as possible cause.
 The most common presentation consists of pulmonary
infiltration and hilar lymphadenopathy; dermal and ocular
lesions. When the parotid glands are affected, 4-6% of cases
present as parotitis and Heerfordt syndrome.
23

Histopathologic features
 Sarcoidosis will show non caseating granulomas, the center of
which usually contains epitheloid macrophages lymphocytes.
 Langhans type giant cells inclusion bodies such as Schaumann
bodies or stellate asteroid bodies are observed.
Treatment:.
24

Central giant cell granuloma
 Central giant cell granuloma (CGCG) was classified by the World
Health Organization in 2005 as a rarely aggressive idiopathic benign
intraosseous lesion that occurs almost exclusively in the jaws.
 Non neoplastic lesion , grouped under two types
 Non-aggressive type
 Aggressive type
25

Clinical features
 60% case occur below 20 years of age
 predominantly males
 70% cases occur in mandible . Mandibular lesions often crosses the
midline
 Most lesions occur in anterior portion of jaws.
 Central giant cell granuloma reported to be associated with
pregnancy, the involved teeth may be non vital and maxillary
tension may show nasal obstruction.
 Pain may or may not be present
26

Radiographic features
 Centrally a destructive lesion produces radiolucent area with either
relatively smooth or ragged borders.
 Occurs mainly in tooth bearing area,
 Causing marked resorption of cortical plates.
 Thin layer of sub periosteal bone formation
 .Multilocular cross hatched appearance particularly in maxilla.
 In sub periosteal type larger bulk on external to normal contour.
 .Displacement of the teeth is frequently root resorption and loss of
lamina of lamina dura seen.
27

Differential diagnosis
O Small unilocular lesions can be confused radio graphically
with periapical cyst / granuloma
O Large multilocular lesions should be differentiated from
other multilocular radiolucencies like – ameloblastoma
O Aneurysmal bone cyst, pindborg tumor etc
28

Histological features
 Multinucleated giant cells are present throughout the
connective tissue stroma, may be seen in patches or
evenly distributed around areas of haemorrhage.
 The giant cells contain up to 30 nuclei. Foci of osteoid
may be present, particularly around the peripheral
margins of lesion.
 Histopathologically, hemosiderin laden macrophages and
extravasated erythrocytes along with small inconspicuous
capillaries.
29

Peripheral giant cell granuloma
 Peripheral giant cell granuloma (PGCG) is one of the most frequent
giant cell lesion of the jaws and originates from the periosteum or
periodontal membrane.
 It is not a true neoplasm but rather a benign hyperplastic reactive
lesion occurring in response to local irritation such as tooth extraction,
poor dental restorations, ill-fitting dentures, plaque, calculus, food
impaction and chronic trauma.
30

Clinical features
 Occurs exclusively on the GINGIVA usually n the area between first
permanent molars and incisors
 Red or reddish – blue nodular mass [sessile or pedunculated]
 Clinical appearance is similar to pyogenic granuloma of the gingiva [
PGC=more bluish –purple compared with bright red of a typical pyogenic
granuloma]
 Prevalence = 5th and 6thdecades
 Females are more prone 60% . Either anterior or posterior regions of gingiva
or alveolar mucosa 31

 Mandible is more prone than maxilla
 If arising from periodontal ligament or periosteum – bone
resorption
 X-ray shows Cuff- shaped radiolucency at entrance to the alveolus
32

Histopathological features
 Fibroblasts are the basic element of peripheral
giant cell granulomas.
 Other features include a nonencapsulated highly
cellular mass with abundant giant cells, inflammation,
interstitial hemorrhage, hemosiderin deposits,
mature bone or osteoid.
33

 Scattered among the plump, young fibroblasts are numerous
multinucleated giant cells with abundant eosinophilic cytoplasm which
appear to be non-functional in the usual sense of phagocytosis and
bone resorption.
 Management of this gingival lesion is surgical excision and
elimination of any local contributing factors.
34

Giant cell tumor of bone
Giant cell tumors are benign bone tumors arising from bone marrow,
which account for about 5% of all biopsied primary bone tumors.
The head and neck region constitute approximately 2% of all GCTs,
with the majority occurring in sphenoid,ethmoid, or temporal bones.
Radiologically, it is usually lytic and expansile without prominent
peripheral sclerosis and periosteal reaction.
The histopathology of GCTs is characterized by frank and marked
haemorrhage, numerous giant cells and stromal cells.
35

The haemorrhage gives rise to the characteristic grossly lytic picture.
The giant cells are considered reactive while stromal cells are considered “true”
neoplastic cells.
The giant cells are thought to be originating from circulating monocytes which
then transform into osteoclasts.
Treatment includes simple or aggressive curettage. Tumor resection and
reconstruction with prosthesis or a large segment allograft has a low rate of
local recurrence. 36

Giant cell fibroma
Giant cell fibroma (GCF) is a relatively rare fibrous hyperplastic lesion
that is considered to occur due to chronic irritation.
It is characterized by functional changes in the fibroblastic cells.
Giant cell fibroma occurs as an asymptomatic sessile
orpedunculatednodule, usually less than 1 cm in size affecting
mandibular gingiva twice as common as maxillary.
37

 The lesions are characterized by a diffuse,
somewhat immature, rather avascular collagenic
stroma with small bipolar and slightly stellate
fibroblasts scattered throughout in moderate
numbers.
 Occasional fibroblasts will be quite large and
angular, and may have more than one nucleus.
 GCF is characterized by the presence of
numerous large stellate and multinucleated giant
cells in a loose collagenous stroma. 38

Giant cell Angiofibroma
 Giant cell angiofibroma (GCA) is a distinctive benign, mesenchymal
tumor commonly encountered in the orbit.
 It has been reported to occur in submandibular region,
parascapular area, posterior mediastinum and in the oral cavity.
 It presents as a slow growing nodule or mass with normal
overlying mucosa.
39

 Characterized by a richly vascularized, patternless spindle-
cell proliferation containing pseudovascular spaces.
 Multinucleated giant cells (often of floret type) and cells
with large, rounded nuclei are present both in the cellular
areas and also lining the pseudovascular spaces.
 The stroma is variably collagenized or sometimes myxoid.
Immuno-histochemically, the spindle and giant cells are
positive for both vimentin and CD34.
40

Osteosarcoma
 Highly malignant tumor arising from primitive msenchymal bone
forming cells
 2nd most common primary malignant bone tumor after multiple
myeloma
 Any bone can be involved but the most common sites are femur,
proximal tibia , proximal humerous
 Most common metastasis to the lungs through blood stream
 More prevalent in males than female
 Involves any age but highest occurrence in adolescence 12-25 yrs 41

Etiology
 1. Exact cause is unknown
 2. Risk factors
 3.Rapid bone growth
a. Environmental
 Radiation
 Oncogenic virus
b. Genetic- Mutation of RB gene
c. Pre existing lesions – EX : fracture of bone , infarcts, Paget's disease
etc
42

Clinical features
 Pain, swelling, loosening of teeth, paresthesia nasal obstruction
 CODMAN'S TRIANGLE : formed at the angle between the
elevated periosteum and underlying surface of cortex
 SUNBURST APPEARANCE : due to osteogenesis within tumor
 SYMMETRIC WIDENING OF PERIODONTAL LIGAMENT :
due to tumour infiltration
43

 Sarcoma cells – undifferentiated mesenchymal stromal
spindle shaped cells with hyper chromatic nuclei
 Osteogenesis – osteoid matrix and bone is found in the
area of tumour cells
 Conventional type which is more predominant in adults
and childhood the predominant features are
osteoblastic, chondroblastic , fibroblastic cells.
44

Treatment plan
 Radiological staging
 Biopsy to confirm diagnosis
 Pre-operative chemotherapy
 Repeat radiological staging
 Surgical resection with wide margins
 Reconstruction using one of many techniques
 Post-op chemo based on preoperative response
45

Hodgkin’s lymphoma
 Hodgkin’s lymphoma is a malignant lymph proliferative
disorder which affects primarily lymph nodes with
secondary extra nodal spread.
 Hodgkin lymphoma (HL) most frequently presents as
cervical lymphadenopathy 80% and rarely involves extra
nodal sites.
46

 The seldomly reported lesions include different locations: palate,
tonsil, floor of mouth, buccal alveolar mucosa, buccal vestibule, and
mandibular bone.
47

 The more commonly described clinical presentations are ulcerations
and swellings. For the diagnosis of Hodgkin’s lymphoma the presence
of Reed Sternberg cells must be established.
48

 This cell of lymphocytic origin is characterized by its LARGE SIZE
and BILOBED NUCLEUS; each containing a large amphophilic or
eosinophilic nucleolus.
 The nuclear chromatin pattern is vesicular and condensed at the
periphery. Reed Sternberg cells may be lacunar, polyploid or
pleomorphic
49

Treatment
 Treatment includes cytotoxic drugs, immunotherapy and
radiotherapy.
 High dose chemotherapy and autologous stem cell transplantation
has also become an established mode of treatment.
 The prognosis of the disease can be related to the number of giant
cells.
 The lymphocyte depletion type of Hodgkin’s lymphoma which has
the most abundant Reed Sternberg cells shows the least favorable
prognosis. 50

Traumatic bone cyst
 The traumatic cyst is a pseudo cyst and uncommon 1% of all jaw
cysts seen in mandible , rare in maxilla.
 It occurs in other bones of the skeleton as well.
 Solitary bone cyst, hemorrhagic cyst, extravasation cyst, unicameral
bone cyst, simple bone cyst, idiopathic bone cavity
51

 According to the traumatic theory, the clot breaks down and leaves
an empty cavity within the bone. Steady expansion of the lesion
occurs secondary to altered or obstructed lymphatic or venous
drainage.
52

Other theories of origin, reviewed by Whinery, have included:
 Cystic degeneration of primary bone tumors.
 A result of faulty calcium metabolism such as that induced by
parathyroid disease.
 Ischemic necrosis of fatty marrow.
 The end result of a low-grade chronic infection.
 A result of osteoclasis resulting from a disturbed circulation caused by
trauma creating an unequal balance of osteoclasis and repair of bone.
53

Clinical features.
 Young persons, the median age being 18 year or 10-20 yrs
 The posterior portion of the mandible [body and symphysis] is more
commonly involved than the anterior, numerous cysts are seen in
incisive canal .
 The maxilla has been known to develop the solitary bone cyst, but
only on extremely rare occasions.
 Enlargement of the mandible, the lesion is discovered during
routine radiographic examination of the patient
 .
54

Signs and symptoms
 Asymptomatic
 Rarely , swelling , pain and paresthesia may be seen
 Common in premolar and molar region of mandible
55

Radiographical features
 Appears as a radiolucency with irregular but well defined edges
and slight cortication
 When many teeth involved – radiolucency scallops between roots
 Teeth involved in lesion – usually vital , no root resorption seen.
56

Histological features.
 Solitary bone cyst may reveal a thin connective tissue
membrane lining the cavity.
 Wall shows loose fibro vascular CT, Hemorrhage and
hemosiderin pigment usually present
 Multinucleated giant cells scattered within the CT
 Adjacent bone shows osteoclastic resorption on inner
surface
57

Aneurysmal bone cyst
 Aneurysmal bone cysts are non-neoplastic benign bony
lesions with multilocular appearance.
 When present in the jaw and long bones ,these manifest
as a swelling which develops rapidly.
 Pain is often reported; paraesthesia, compressibility, and
crepitus.
58

Clinical features
 First 3 decades
 Mainly females
 Molar regions of mandible and maxilla
Signs and symptoms
59
 Hard rapidly growing swelling which can
cause malocclusion
 Mobility of teeth
 Migration of teeth
 Root resorption
 Pain is often present
 Paresthesia is present
 If lesion perforates cortical plates , can
cause EGG SHELL CRACKING

 Classically seen as a unilocular , ovoid / fusiform radiolucency which
balloons the cortical plates
 Teeth displacement and root resorption also observed
60

 Microscopically, numerous cavernous, sinusoidal spaces filled
with blood are surrounded by loose, fibrous connective tissue
and not lined by endothelium
 The connective tissue septa contain small capillaries,
multinucleated giant cells, inflammatory cells, extravasated
erythrocytes, and hemosiderin
 The multinucleated, osteoclast-like giant cells often
aggregate adjacent to the sinusoidal spaces.
61

Treatment
62
Percutaneoussclerotherapy,
Diagnostic andtherapeutic embolization
curettage,
Block resection and reconstruction,
radiotherapy
Systemic calcitonin therapy

Brown tumor of hyperparathyroidism
 Bone tumor composed of non neoplastic reactive tissue that
occurs in setting of primary, secondary and tertiary
hyperparathyroidism
CAUSES:
 Adenoma
 Chief cell hyperplasia
 PTH Receptor on OB  active RANKL  RANKL binds to
RANK on OC  oc activation
63

Clinical features
 3rd and 4th decades of life
 Females
 May be solitary or multiple
 SITE : pelvis , ribs , clavicles and extremities
 Painful mass
64

Biochemical changes
Hypercalcemia
Hypophosphatemia
Hypercaliuiria
Lowering of renal phosphate
threshold
Elevated PTH
Elevated VIT D
65

O Well –circumscribed , reddish – brown hemorrhagic mass with lobular
architecture thins and expands cortex
O Large , blood filled cysts may develop [ osteitis fibrosa cystica]
O Peripheral shell of reactive bone may be present
67

OSTEODYSTROPHIC LESION
Noonan like mutiple gaint cell lesion
syndrome
68

Cherubism
 Also known as familial fibrous dysplasia of the jaws , disseminated
juvenile fibrous dysplasia , familial multilocular cystic disease of
the jaws and hereditary fibrous dysplasia of the jaws
 It was first described by W.A . Jones in 1933 as familial
multilocular cystic disease of the jaws. He coined the term
cherubism
 Disease is of developmental origin
 Point mutations in SH3BP2 gene on chromosome 4p16
69

Clinical features
 Affects in early childhood – mainly 1-5 yrs
 The characteristic feature is that it is strictly bilateral
 Painless bilateral firm swelling involving the posterior mandible
usually
 Maxilla if involved either tuberosity or entire maxilla
 Eyes turned up to heaven which is mainly due to wide rim of
exposed sclerae below the iris
 Impaired mastication, speech difficulties, loss of normal vision
70

 Progressive , extensive bone involvement causes widening and distortion
of alveoli
 As a result , developing teeth displaced , fail to erupt
 Numerous dental abnormalities have been reported , such as agenesis of
the 2nd and 3rd mandibular molars , displacement of the teeth, delayed
eruption of the permanent teeth , and transposition and rotation of the
teeth
 The permanent dentition is often defective
 It is been connected to NOONAN’S SYNDROME
71

 Bilateral multilocular radiolucent lesions of the mandible
more often than lesions of the maxilla. Cortices are
frequently paper thin focally.
 Complete erosions and concomitant soft tissue
extension
 Involvement of the coronoid process is common,
Condylar involvement is rare
 Initially fibrous replacement of the bone give rise to
SOAP BUBBLE 72

 The presence of numerous unerupted teeth and the destruction of
alveolar bone may displace the teeth, producing FLOATING TOOTH
SYNDROME.
 With adulthood, the cystic areas in the jaws become re-ossified ,
which results in irregular patchy sclerosis
73

Multinucleated giant cells are
positive for tartrate resistant acid
phosphate
 Vascular fibrous stroma is loosely arranged
 Multinucleated giant cells are small , numerous and usually
aggregated focally with fibrous connective tissue stroma
 Peculiar perivascular eosinophilic cuffing
 An increase in osteoid and newly formed bone matrix id found in
the peripheral region of the fibrotic stroma in patients above the
age of 20 years
74

Fibrous dysplasia
 Fibrous dysplasia is a skeletal developmental anomaly of the bone –
forming mesenchyme that manifests as a defect in osteoblastic
differentiation and maturation
 It is a non hereditary disorder of unknown cause
ETIOLOGY
 Idiopathic
 Non hereditary
 Caused by mutation in GNAS1 gene
75

GNAS1 gene
encodes a G-
proteinStimulate
s production of
CampContinuous
activation of the G-
protein
Overproduction
ofcAMPin affected
tissuesHyper
function of
affected endocrine
organs
Increased
proliferation of
melanocytesResul
ts in large café –au-
laitspots
cAMPaffect on
differentiation of
osteoblasts
Pathogenesis
76

 Incidence equal in males and females
 Commonly found in persons aged 3 – 15 yrs
 Polyostotic disease persons asymptomatic before 10 years
 Monostotic disease persons asymptomatic as old as 20 – 30 years
77
Clinical features

70 – 80% of fibrous dysplasia .
Occurs in rib, femur, tibia, craniofacial bones and humerus
Pain or pathologic fracture in 10-70 years
Bone deformity less severe
Painless swelling of the jaw
Swelling involves labial or buccal plate
Protuberant excrescence of inferior border of mandible
Monostotic form
78

20 – 30 % OF FIBROUS DYSPLASIA
SITES : FEMUR, TIBIA, RIBS , SKULL AND FACIAL BONES ,
UPPER EXTRIMITIES , LUMBAR SPINE , CLAVICLE AND
CERVICAL SPINE
TENDS TO OCCUR IN UNILATERAL DISTRIBUTION
INVOLVEMENT ASYMMETRIC AND GENERALIZED ON
BILATERAL LESIONS
Polyostotic form
79

Pain in involved
limb
spontaneous
fracture
Structural
integrity weak
Bowing of
weight bearing
bones ,
curvature of
femoral neck ,
proximal shaft
increase
Shepherd’s
crook deformity
80

• Involves variable number of bones,
accompanied by pigmented lesions of skin
or café – au – lait spots
JAFFE LICHENSTIEN SYNDROME
• Involves nearly all bones in skeleton ,
pigmented lesions of skin and endocrine
disturbances
MC-CUNE ALBRIGHT SYNDROME
• Hyperthyroidism , hyperparathyroidism ,
Cushing syndrome , gonadotrophin, mc-
cune Albright syndrome
ENDOCRINE DISTURBANCES
ASSOCIATED
81

CAFÉ- AU – LAIT
SPOTS
• INCREASED MELANIN IN
BASAL CELLS OF
EPIDERMIS
• CUTANEOUS
PIGMENTATION SEEN
IPSILATERAL TO SIDE OF
BONE LESION
• OCCUR AT BIRTH PRECEDES
SKELETAL AND ENDOCRINE
DISEASES
MAZABRAUDS
SYNDROME
• FIBROUS DYSPLASIA AND
INTRAMUSCULAR MYXOMA
, RISK OF SARCOMATOUS
MALFORMATION
MALIGNANCIES
• OSTEOSARCO MC-CUNE
ALBRIGHT SYNDROME
• CHONDROSARCO MC-CUNE
ALBRIGHT SYNDROME
• FIBROSRCO MC-UNE
ALBRIGHT SYNDROME
• LIPOSARCO MC-CUNE
ALBRIGHT SYNDROME
82

NO SIGNIFICANT CHANGE IN SERUM
CALCIUM /PHOSPHORUS
ELEVATED ALKALINE PHOSPHATASE
MODERATE INCREASE IN BASAL
METABOLIC RATE
83

MONOSTOTIC POLYOSTOTIC
Proliferating fibroblasts in a compact
stroma of interlacing collagen fibres
Irregular bony trabeculae scattered
throughout lesion
No definite pattern
C- shaped or Chinese character shaped
Trabeculae usually coarse woven bone
Lesions rich in spindle shaped fibroblasts
with a swirled appearance
Islands of cartilaginous tissue within
lesions
Affected bones may have cystic lesions
lined by multinucleated giant cells
Histologic features
84

 Network of fine bone trabeculae
 Increased trabeculation – lesion more opaque and mottled
appearance
 Opaque with many delicate trabeculae ; ground – glass or
‘peau d’ orange’ appearance
 Cortical bone becomes thinned
 Roots of teeth separated or moved out of normal
85

Treatment
 Conservative treatment to prevent deformity
management requires a multidisciplinary approach in
polyostotic
 Bisphosphonates therapy may help to improve function,
decrease pain and lower fracture risk in some patients
 Surgery indicated for confirmatory biopsy, correction of
deformity, prevention of pathologic fracture
86

Paget’s disease
 Paget’s disease is a chronic condition of bone
characterized by disorder of the normal bone remodeling
process
 Characterized by abnormal resorption and deposition of
bone , resulting in distortion and weakening of bone .Also
known as osteitis deformans is a bone disease
 Etiology :
 Unknown , but predisposing factors could be inflammatory,
genetic , endocrine factors 87

• Multifocal chronic skeletal disease
due to chronic paramyxoviral virus
infection
• As a result , the bone that is
formed is abnormal
INFLAMMATORY DISEASE
• Certain of genes may been
associated with Paget&apos;s
disease , including the
sequestrosome 1 gene on
chromosome5
CONGENTIAL
88

Manifestation – symptoms
BONE PAIN
FRACTURES
Skeletal deformities – bowed legs, fronto- occipital skull enlargement
Warm sensation – due to increased blood supply to abnormal bones
Nerve damage - hearing loss , visual abnormalities
Cardiac abnormalities – shortness of breath 89

Manifestations – sign
Bitemporalskull enlargement with frontal bossing, diluted scalp veins, nerve deafness.
Maxilla affected more than mandible , maxilla –enlargement of middle third of face [
LEONTIASIS OSSEA]
In DENTULOUS patients spacing of teeth is seen , while edentulous patients complains
of tightness of the dentures.
Mandible involved rarely – may cause PROGNATHISM.
Also angioid streaks in the fundus of the eyes and a short kyphotic trunk
90

Investigations
 Blood chemistry results indicate very high alkaline phosphates levels
with normal serum calcium and phosphorus
 X- ray of the skull, spine , pelvis and long bone
 CT scan
 MRi
91

Radiological appearance
 Cortical thickening with a coarse, thickened trabecular pattern
 Mixed areas of radiolucent osteolysis and radiopaque
osteosclerosis may be seen
 Dental radiographs also show the classical cotton wool appearance
 Extensive hypercementosis can be noted
92

 Alternating bone resorption and deposition seen.
 Thus, osteoclastic resorption seen surrounding the trabeculae.
 Osteoblastic activity also seen with formation of osteoid rims
around trabeculae a characteristic feature is presence of basophilic
reversal lines in the bones.
 It gives a JIGSAW OF THE BONE
 The new bone is disordered , poorly mineralized and lacks structural
integrity
Treatment
93

Conclusion
Multinucleated giant cells are commonly encountered
in various lesions of oral cavity.
They may be characteristic for the lesion or exist
just to the elimination of microbes or foreign
materials. They provide a vital clue to the diagnosis.
So, a definite criterion to identify individual giant
cells in any giant cell lesions however is required to
assist the clinician and researchers for proper
diagnosis and management.
94

References
 Shafer’s textbook Of Oral Pathology Seventh Edition Shafer.
Hine. Levy.
 Kamble KA, Guddad SS, Guddad SS, Lingappa A. Central giant cell
granuloma: A case report with review of literature. J Indian Acad
Oral Med Radiol 2016;28:98-101.
 Ranjan V, Chakrabarty S, Arora P, Rastogi T. Classifying giant cell
lesions: A review. J Indian Acad Oral Med Radiol 2018;30:297-301.
 Giant cell lesions of the oral cavity by Sreeja Chellaswamy,
Pavithra Manohar, Beeula Rajakumari, Sathish Muthukumar
Ramalingam,Vijayashree Ragavan, Nachiammai Nachiappan
 Shrestha et al., Giant cells and giant cell lesions of oral cavity
- a review, Cumhuriyet Dent J 2014;17(2):192-204.
95

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