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Giant Cell Lesions of Jaw

Contents
1. Introduction

2. Classification

3. Origin and types of Giant cells

4. Detail about each lesions

5. References
Introduction
• Presence of giant cell can be pathognomic or characteristic feature of a lesion.

• Most of time they have active participation in lesion , sometime only present in cellular
background.

• They have various source of origin which may determine their functioning.
1. Based on etiopathogenesis
Classification
A. Where giant cells are present in the concerned background
and are pathognomic:

1. Hodgkin’s syndrome
2. Peripheral giant cell granuloma
3. Giant cell fibroma

B. Where giant cells are characteristic, but not pathognomic:

1. Tuberculosis
2. Herpes simplex virus infection
3. Measles
4. Xanthoma Continued…
C. Diseases associated with the presence of giant cells:

1. Orofacial granulomatosis
2. Fungal infection foreign body reactions
3. Neoplasms
4. Syphilis
5. Leprosy
6. Fibrous dysplasia
7. Cherubism
8. Paget’s disease of the bone
9. Aneurysmal bone cyst
10. Ossifying fibroma
11. Wegener’s granulamatosis
12. Actinomycosis
13. Chronic diffuse sclerosing osteomyelitis
14. Odontogenic giant cell fibromatosis
15. Heerfordt’s syndrome.
2. Based on type of lesion
Giant Cell Lesions

Microbial Tumor and Tumor like lesions Cystic Lesion


Tuberculosis
Central giant cell granuloma Traumatic bone cyst
Leprosy
Peripheral giant cell granuloma Aneurysmal bone cyst
Actinomycosis
Giant cell fibroma
Sarcoidosis
Giant cell tumor

Osteosarcoma

Rhabdomyosarcoma
Continued…
Hodgkin’s lymphoma
Giant Cell Lesions

Metabolic lesion Osteodystrophic Lesion Miscellaneous Lesion


Hyperparathyroidism Cherubism,
Noonan-like multiple giant cell
lesion syndrome
Paget’s disease,

Fibrous
Dysplasia
3. Based on type of giant cell present

I. Epithelial-derived viral-induced multinucleated giant cell containing lesions:

1. Tzank giant cells – herpes simplex


2. Tzank giant cells – herpes zoster

II. Monocyte/MGCs containing lesions:

1. Inflammatory granuloma-associated giant cells


2. Langhans giant cell containing pathologies
3. Infections – tuberculosis, leprosy, late syphilis, deep fungal infections
4. Unknown antigenic stimuli – sarcoidosis and orofacial granulomatosis
5. FBGC containing lesions
6. Foreign body granuloma
7. Osteoclastic giant cell containing lesions
8. Lesions with osteoclastic giant cells being the primary pathological cells – Paget’s
disease

Continued…
III. Lesions with reactive osteoclastic giant cells formed secondarily by the activation of
primary lesional stromal cells

1. Peripheral and central giant cell granulomas,


2. Cherubism
3. Aneurysmal bone cyst
4. Fibrous Dysplasia
5. Brown tumor of hyperparathyroidism
4. Based on Pathology involved:

I. Giant cell lesions of Bone

A. Reactive: B. Benign: C. Malignant:

• Brown tumor • Giant cell granuloma • Osteosarcoma

• Hemophiliac pseudo tumor • Non-ossifying fibroma • Clear cell chondrosarcoma

• Intraosseous haemorrhage • Giant cell tumor • Metastatic carcinoma

• Aneurysmal bone cyst

• Chondroblastoma

• Chondromyxoid fibroma

• Langerhans cell histiocytosis

• Pigmented villonodular synovitis


Continued…
II. Mucosal lesions with giant cells

A. Peripheral giant cell granuloma

B. Giant cell fibroma

C. Oral granulomatosis
• Specifc: Fungal, bacterial, viral infection
• Non-specific: Sarcoidosis, Wegener ’s granulomatosis
Giant cell
• Giant cells are those cells which are having larger dimension (usually 40
µm to 120 µm diameter) then cells present in a region.

• They are seen in both physiological and pathological conditions.

• Classification of giant cells:

A) Physiologic giant cells: B) Pathologic giant cells :

a. Osteoclast a) Langhans giant cells found in TB


b. Odontoclast b) Reed–Sternberg cells in Hodgkins lymphoma
c. Megakaryocytes c) Giant cell of granuloma
d. Syncytiotrophoblast d) Epithelial giant cells as in HSV infection
According to origin :

1. Macrophage derived:

a. Langhans giant cells


b. Foreign Body multinucleated giant cells
c. Touton giant cells or Xanthelasmatic giant cells

2. Epidermal cell derived:


a. Tzank giant cells
b. Multinucleated epidermal giant cells.

3. Melanocyte derived:
a. Starburst giant cells
b. Giant cells in melanocytic nevus
i. Balloon cells
ii. Giant nevus cells

4. Other giant cells:


Floret-like multinucleated giant cells
Theories of giant cell formation

1. Immune mediated mechanism:


• Poorly removable antigen.
• Production of lymphokines
• Promote fusion of macrophages to form giant cells.

2. Fusion of “young” and “older” macrophages.


• Older macrophages undergo changes on their cell surface
• Recognized by newer macrophages
• Altered macrophage cell surface act as stimulus for cell fusion.

3. Simultaneous fusion
• Two or more macrophage try to ingest the same particle
• Simultaneous fusion occurs
• Leading to formation of multinucleated giant cells.
4. Viral induced
A) Attachment of viral envelope
• When viral envelope attach to a cells
• Cell surface coat thickness is reduced
• Approximation of lipid bilayer of adjacent cell
• Leading to fusion

B) Incorporated virus
• Proteins coded by virus are coated on surface of cell
• Thus infected cell has modified surface
• Promote fusion with uninfected cells.
5. Tumor giant cells
• Tumor cell release extracellular enzymes.
• Reduce surface coat thickness of adjacent cell.
• Causing close approximation of lipid bilayers of cell.
• Leading to fusion.
Microbial giant cell lesions
Oral Tuberculosis

• Infectious granulomatous disease.


• Commonly caused by Mycobacterium tuberculosis.
• Tuberculous lesions of oral cavity can be primary lesion or
secondary to pulmonary tuberculosis.
• Common site for oral Tuberculous lesion : Tongue
• Can also occur in gingiva, floor of mouth, palate, lips and buccal
mucosa.
• Typical oral lesion : ulceration with undermined edge and
granulating floor.
• Cervical lymphadenopathy: matted lymph nodes.
Pathogenesis
Acute inflammation fail to remove bacilli

Macrophages engulf bacilli

When antigen is poorly degradable, macrophage undergo morphological


change

Epitheloid cells

Some of epitheloid cells fuse with each other and form


giant cells
Both epitheloid cells and giant cells are:

1. Weak phagocytic cell


2. Active in destruction secretions
3. Antigen presentation

Histopathological

Granuloma consisting of caseating


necrosis surrounded by epitheloid
cell, lymphocytes and Langhans’
giant cells
• Investigation:
1. Sputum for acid fast bacilli (pulmonary)
2. Tissue biopsy (Common for extrapulmonary)
3. Culture (Definitive diagnosis )
4. Mantaux or tuberculin skin test

For screening of tuberculosis


• Purified protein derivative of antigen (0.1ml) is injected
intradermally.
• Positive: Exposure to antigen
Previous exposure or active lesion cannot be differentiated.
• False negative: Immunocompromised state or widespread
tuberculosis.

• Treatment
Antitubercular drugs
World Health Organization recommendation, 4th Edn

Intensive phase Continuation phase


2 months HRZE 4 months HR
3 months HRZE 4 months HRE

When isoniazid resistance is there

Intensive phase Continuation Comment


phase
Daily Daily Optimal
Daily 3 times in a week Accepted alternative for new patient
receiving DOTS
3 times in a week 3 times in a week Accepted alternative for new patient
receiving DOTS and is not associated
with HIV

H : Isoniazid R: Rifampicine Z: Pyrazinamide E: Ethambutol


DOTS : Directly observed treatment short course
Oral Leprosy (Hansen’s Disease)

• Chronic infectious disease produced by Mycobacterium leprae.

• Exact route of transmission is unknown can be direct contact,


maternofeatal transfer.

• High number of organism present in nasal secretion

• Skin, nasal and oropharnygeal mucosa are common sites of involvement.


Two type of clinical presentation are seen: (Polar forms of leprosy)

Tuberculoid Lepromatous
High immune response Reduced immune response
Localized Diffuse
Inoculation period 2 – 5 years 8-12 years
Lepromin test positive Lepromin test negative
Granuloma formation with less Significant number of
number of microorganisms microorganism in cells
Erode basal layer of epidermis. Does not erode basal layer of
Predilection for dermal nerves epidermis
• Leprosy can be also classified according to bacterial load as:

1. Paucibacillary leprosy : Tuberculoid leprosy


2. Mutibacillary leprosy: Lepromatous leprosy

1. Paucibacillary leprosy
(Pauci: few)

• Well circumscribed hypopigmented macule.


• Nerve involvement results in anesthesia of skin
• Loss of sweating
• Oral lesions are rare
2. Mutibacillary leprosy

• Face is common site of involvement

•Ill defined hypopigmented macule and papules


that become thickened and form nodule.

•Skin enlargements gives characteristic


facial appearance (Leonine facies)

•Collapse of nasal bridge is pathognomic.

•Facial and maxillary nerve are commonly


affected leading to loss of sensations in area
supplied.
In Nasal cavity

• Floor, septum, bridge can be affected.


• Collapse of bridge of nose is pathognomonic.
• Loss of sense of smell can be there.

Facies Leprosa

Destruction of
•Anterior maxilla and alveolar ridge.
• Anterior nasal spine
•Nasal complex destruction
Infection of lip can lead to macrocheilia

Oral Lesions

• Common in Hard and soft palate, labial


maxillary gingiva, tongue, lips, buccal
maxillary gingiva, labial mandibular gingiva
and buccal mucosa

•Sites which are cooled by passage of air is


frequently affected.

•Affected soft tissue appear yellowish to red


•Sessile firm papules are formed followed by
ulceration and necrosis.
•Healing by fibrosis is attempted but recurrent
infection causes significant scaring and loss of
tissue
• Investigation:
1. Cytological smear
2. Tissue biopsy

Histopathology:
• Tuberculoid leprosy
Granuloma consisting of caseating necrosis surrounded by epitheloid
cell, lymphocytes Langhans’ giant cells . Erosion of basal layer of
epidermis. Invasion of epitheloid cells, Giant cells and lymphocytes
into peripheral dermal nerves.
• Lepromatous

Foamy macrophages known as “lepra cells” or Virchow cells


are seen heavily laden with bacilli
This aggregation of macrophages is present near blood vessel,
nerves or dermal appendages.
Erosion of basal layer of epidermis is absent. Macrophages are
separated from epidermis by clear zone.
Epidermis show thinning, may ulcerate .
Differential Diagnosis

Melkersson – Rosenthal Syndrome (Orofacial granulomatosis)

Facial paralysis
Swelling of face and lips Cheilitis granulomatosa
Fissured tongue
• Treatment

Multidrug therapy

Paucibacillary:
Rifampin (600mg once a month)
Dapsone(100mg daily) for 6 months.

Mutibacillary:
Rifampin(600mg once a month)
Dapsone(100mg daily)
Clofamizimine (300mg once a month + 50 mg daily) for 12-24 months.
Syphilis
• It is a Sexually transmitted disease (STD)
•Caused by Spirochaetes Treponema pallidium.

• Mode of transmission
1. Sexual intercourse
2. Intimate person to person contact
3. Materno foetal transmission

• Stages of syphilis
According to period after which lesion is appeared:

1. Primary syphilis
2. Secondary syphilis
3. Tertiary syphilis
Primary Syphilis
•Primary syphilis is characterized formation of chancre at site of inoculation.

•It is formed in 3 to 90 days after the initial exposure.

•Chancres are usually solitary, can be multiple occasionally.

•The external genitalia and anus are the most common sites.
•Initially a papule is formed followed by central ulceration.

•Oral cavity is the most common extragenital site.


•Oral lesions are seen most
commonly on the lip, but other sites include the tongue, palate, gingiva, and tonsils.

•The oral lesion are painless ulceration.

•Regional lymphadenopathy which is usually bilateral in most patients.


If untreated, then the initial lesion heals within 3 to 8 weeks.
Secondary Syphilis
Disseminated Syphilis
• It can be seen clinically 4 to 10 weeks after the initial infection.
• Systemic symptoms seen most commonly are

1. Painless lymphadenopathy
2. Sore throat
3. Malaise
4. Headache
5. Weight loss
6. Fever
7. Musculoskeletal pain.

• Diffuse, painless, maculopapular cutaneous rash,

• The rash may also involve the oral cavity and appear as red, maculopapular areas.
Mucous Patches formation
• Some patients may show focal areas of intense exocytosis of
inflammatory cell and spongiosis (intracellular edema)
leading to zones of sensitive whitish mucosa known as mucous
patches

• Some adjacent patches can fuse and form a “snailtrack pattern”.

These may appear on any mucosal surface but are


found commonly on the tongue, lip, buccal mucosa,
and palate.
• In Secondary syphilis condylomata lata is formed
Although these lesions typically occur in the genital or
anal regions.

• Rarely in oral cavity if present they are present as multiple indurated


papillary nodules

• Contrast to the isolated chancre noted in the primary


stage, multiple lesions are typical of secondary syphilis.

• Spontaneous resolution usually occurs within 3 to 12


weeks; however,

• Chances of relapse are there.


Lues Maligna

•Malignant syphilis.

•Widespread form of secondary syphilis.

•Formation of multiple necrotic ulceration which


commonly involve face and scalp

•Found in immunocompromised states like Acquired


immunodeficiency syndrome .
Latent syphilis

•After the second stage, patients enter latency phase.

•In this there is absence of any lesions and symptoms, known as latent syphilis.

•It may last for 1 to 30 years.

•Following this some patient enter into third stage known as tertiary syphilis.
Tertiary Syphilis
Systemic complications of tertiary syphilis

The vascular system can be affected significantly

1. Arteritis
2. Aneurysm of the ascending aorta
3. Left ventricular hypertrophy
4. Aortic regurgitation
5. Congestive heart failure

Involvement of the central nervous system (CNS)


1. Tabes dorsalis, : Loss of coordination of movement due to syphilitic infection of
spinal cord.
2. General paralysis
3. Psychosis,
4. Dementia
5. Paresis
6. Death
Ocular lesions such as
1. Inflammation of iris, choroid and retina of eye.
2. Argyll Robertson pupil
(Pupil fails to react to light but responds to accommodation)

Gumma

•It is foci of granulomatous inflammation.


•It is indurated, nodular and ulcerated .
•It causes excessive tissue destruction
•Common oral site are palate and tongue.
•Palatal lesion may cause perforation into nasal cavity.
Interstitial glossitis

Diffuse gumma on tongue


That appear as large, lobulated, and irregularly shaped.
Lobulation can be the result of contracture of the lingual
musculature after healing of gummas.

Luetic glossitis

Diffuse atrophy and loss of the dorsal tongue papillae.


Previously it was thought to be precancerous lesion
Congenital syphilis
Sir Jonathan Hutchinson in 1858 described

Hutchinson tried

1. Hutchinson’s teeth
2. Ocular interstitial keratitis
3. Eight nerve deafness

Hutchinson’s incisors has greatest Mulberry molars taper toward the


mesiodistal width in the middle third of the occlusal surface.
crown.
The occlusal anatomy is abnormal, with
Incisal third tapers to the incisal edge numerous disorganized
tooth resembles a straightedge screwdriver globular projections that resemble the
surface of a mulberry
Manifestations of Congenital syphilis

Features Number of Patients Percentage


Frontal bossing 253 86.7
Short Maxilla 227 83.8
High Arched Palate 207 76.4
Saddle shaped nose 199 73.4
Mulberry molars 176 64.9
Hutchinson’s incisors 171 63.1
Enlargement of clavicle adjacent to sternum: 107 39.4
Higoumenaki’s sign
Relative Prognathism of mandible 70 25.8

Interstitial keratitis 24 8.8

Cohort study n 271


Premature perioral fissuring : Rhagades 19 7.0
Anterior bowing of tibia : Saber shin 11 4.1
8th cranial nerve deafness 9 3.3
Concavity in vertebral border of scapulae : 2 0.7
Scaphoid scapulae
Painless synovitis and enlargement of joints, 1 0.3
usually knee : Clutton’s joint

Fiumara NJ, Lessel S: Manifestations of congenital syphilis: an analysis of 271 patients. Arch
Dermatol.1970;102-78.
Investigations

1. Smear or biopsy
Demonstration of microorganism by specific immunofluorescent antibody
or
Serological test VDRL (Venereal Disease Research Laboratory )
and RPR (Rapid Plasma reagin)

2. Culture in artificial medium is not possible.


Treatment
1. Drug of choice : Penicillin

2. Patient allergic to penicillin: Doxycycline

3. Tetracycline and erytheromycin also have antitreponemal activity

Jarisch Herxheimer reaction

• This process occurs secondary to release of endotoxin when antibiotics kill


large numbers of organisms.

• Clinical evidence of this reaction occurs about 8 hours after the first injection
of penicillin

• Most commonly includes mild fever, malaise, headache, and an exacerbation


of skin or mucosal lesions.

• These signs and symptoms rapidly fade.


Actinomycosis
It is chronic suppurative disease caused by Actinomycetes israelii and vicosus.

Organism is normal commensals of oral cavity can invade and proliferate in


condition like break in continuity of epithelium.

Depending on anatomic location it is 4 types:

1. Cervicofacial 50%

2. Abdominal: liver appendix and caecum 25%

3. Thoracic: lungs, pleura, ribs and vertebrae 15%

4. Pelvic: infected IUCDs and skin 5%


Cervicofacial Actinomycosis
Common site: 1. Soft tissue area over angle of mandible
2. Submental, submandibular and buccal
The classic description is of a “wooden”
indurated swelling which ultimately forms a
central, softer area of abscess.

The infection may extend


to the surface, forming a sinus tract.

Suppurative reaction of the infection may


discharge large yellowish flecks that represent
colonies of
the bacteria called “sulfur granules”

“Lumpy jaw”

Pain often is minimal.


• Major Salivary gland can be involved followed by abscess formation in
submandibular and masseter space.

•Common intraoral site: Tongue

• Tonsillar crypts : Tonillar hyperplasia.

•Osteomyelitis of maxilla and mandible is also reported.


Radiological findings

large osteolytic region(arrows),


with a floating tooth (arrowhead), in the right mandibular body crossing the midline

Y. Sasaki,et al .Actinomycosis in the Mandible: CT and MRI findings. Am J Neuroradiol. Feb 2014;35:390–94 .
Investigation

1. Culture and staining.

2. Lesional Biopsy

3. Fine needle aspiration


Treatment

1. Drug of choice : Penicillin Amoxicillin

2. Case of allergy or resistance: tetracycline

3. Adequate debridement for ostomyelitis

4. Tonsillectomy may be required for tonsillar hyperplasia


Sarcoidosis
• Sarcoidosis is a multisystem granulomatous disorder of unknown cause.

• Jonathan Hutchinson initially described the disease in 1875

• Boeck coined the term sarcoidosis which in Greek means “fleshlike


condition”.

• It is characterised by noncaseating granulomatous inflammation.

• Antigen is unknown.

• Possible antigen could be

Infectious agents e.g.


Mycobacterium, Epstein-Barr virus

Environmental agents e.g.


Wood dust, pollen, clay, and silica etc.
• Unusual immune response can be associated with:

1. Prolonged or heavy antigenic exposure.


2. Immunodysregulation (genetic or secondary to other factors).
3. Defective regulation of the initial immune reaction.

•Combination of all three of these factors

•Resent investigators have confirmed genetic predisposition also.


Clinical Features:

•There is a slight female predominance.

•The disease exhibits a bimodal age distribution,

first peak between 25 and 35 years of age


second peak between 45 and 65 years of age

• Mostly lymphoid, pulmonary cutaneous and ocular lesions are most common
but any organ in body can be affected.

•Common clinical symptoms include


1. Dyspnea
2. Dry cough
3. Chest pain
4. Fever
5. Malaise
6. Fatigue
7. Arthralgia,
8. Weight loss.
• Predominant sites:
1. Lungs
2. Lymph nodes
3. Skin
4. Eyes
5. Salivary glands
All cases show lymphoid tissue involvement

Lungs

•Multiple Granuloma formation.


•Pulmonary symptoms like dry cough dyspnea and chest pain.
•Along with Mediastinal, hilar and paratrachial lymph nodes.

Skin

•Violaceous, indurated lesion known as Lupus Pernio is formed.


•Site : Face, limbs, back and buttocks.
•They are symmetrical, elevated indurated violet-purplish plaques
Erythema nodosum formation
Scattered, nonspecific, tender erythematous
nodules, known as erythema nodosum,
frequently can occur on the lower legs.

Ocular

• Most often anterior uveitis (inflammation of


iris, ciliary body and Choroid).
• Conjunctiva and retina and lacrimal glands
may also be involved.
• It leads to Keratoconjuctivitis sicca .

Salivary glands

• Enlargement
• Xerostomia
Intraosseous

• Intraosseous lesions may occur and most commonly involve the


phalanges, metacarpals, and metatarsals.

•Less frequently, the skull, nasal bones, ribs, and vertebrae are affected.
Oral lesions

•Isolated papule or macules are formed with


an area of granularity or an ulceration.

•The mucosal lesions may be normal in color,


brown-red, violaceous, or hyperkeratotic.

•The most frequently affected intraoral soft tissue


site is the buccal mucosa, followed by the
gingiva,lips, floor of mouth, tongue, and palate.
Syndromes associated with Sarcoidosis:

Löfgren’s syndrome

1. Erythema nodosum
2. Bilateral hilar lymphadenopathy,
3. Arthralgia

Heerfordt’s syndrome (uveoparotid fever)

1. Parotid enlargement
2. Anterior uveitis of the eye
3. Facial paralysis
4. Fever
Differential Diagnosis

1. Sjögren Syndrome

Rheumatoid arthrirtis
Systemic Lupus Erythematosus
Collagen disorder

Sarcoidosis shows noncaseating granulomas

Sjögren syndrome shows focal chronic inflammatory aggregation adjacent to normal


appearing - acini

and epimyoepithelial islands (proliferation of salivary gland ductal epithelium and


myoepithelium.)
Histopathology
• Classic picture of a granulomatous inflammation
seen.

•Tightly clustered aggregates of epithelioid


histiocytes are present, with a surrounding rim of
lymphocytes.

• Intermixed with the histiocytes are scattered


Langhans’ or foreign body type giant cells.

•These giant cells may show following


cytoplasmic inclusion:
1. Laminated basophilic calcifications known as
Schaumann bodies which are degenerated
lysosomes.

2. Stellate inclusions, known as Asteroid bodies


which are entrapped fragments of collagen.
• In lymph nodes, small yellow-brown structures called
Hamazaki-Wesenberg bodies which are large lysosomes may
be noted in the subcapsular sinus.

• Special stains for fungal and bacterial organisms are negative.


• No polarizable, dissolvable, or pigmented foreign material can be detected.
• In lymph nodes, small yellow-brown structures called
Hamazaki-Wesenberg bodies which are large lysosomes may
be noted in the subcapsular sinus.

• Special stains for fungal and bacterial organisms are negative.


• No polarizable, dissolvable, or pigmented foreign material can be
detected.
Investigation
1. Biopsy

Showing granulomatous inflammation without caseating necrosis.


Negative findings with both special stains and culture for organisms.

2. Kveim test

Sterilized suspension of human sarcoid tissue is injected intradermally.


In positive test, nodular lesion appear in 3-6 weeks at inoculation site
Its microscopic examination show presence of non-caseating granulomas.

The procedure is no longer widely used because of difficulty in obtaining material


for test.
Treatment
1. In most of patients symptoms may resolve spontaneously within 2 years
without treatment.

2. Active intervention is required for progressive disease and significant


systemic involvement.

3. First line therapy: Oral Corticosteroid


Prednisone at a dose of 0.5 mg per kilogram of body weight .
For at least 6 months, with a gradual reduction of the dose over the
following 6 months.(12 month therapy)

4. If sarcoidosis is reactivated while the dose is still above the maintenance


level of 7.5 mg per day, immunosuppressive combination therapy with
methotrexate or azathioprine should be considered.
Tumor and tumor like giant cell
Central giant cell granuloma
•Giant cell lesion or Giant cell tumour or Giant cell reparative granuloma

•Clinical features

•Most of casses occurs before 30 years of age.


•Mostly seen in females

•Site: anterior region of jaw


• Mandible is commonly affected then maxilla
• Lesion frequently crosses midline.

•Mostly giant cell granuloma of the jaws are asymptomatic


• Paresthesia can be there.
• There is painless expansion of the affected bone and thinning of cortical pates.
•Sometime perforate to soft tissue.
• Release of hemosiderin may gives bluish cast to lesion.
•Covering mucosa appear normal unless traumatised.
Based on clinical and radiographic findings CGCG is of 2 types :

Non Aggressive lesions Aggressive lesions


•Asymptomatic • Pain

•Slow growth • Rapid growth

•Absence of cortical perforation • Cortical perforation

•Absence of adjacent root • Root resorbtion of adjacent teeth


resorbtion
Histopathology

1. Vascular granulomatous tissue stroma consisting of

• Spindle shaped mesenchymal cells.


• Round monocyte-macrophages.
• Giant cell which can osteoclast, present focally or diffusely.

2. These mesenchymal cells give rise to moncyte-macrophage and promote


formation of ostoclast like giant cell form macrophages.

3. Areas of erythrocyte extravasations, destruction and hemosiderin


deposition often are prominent.

4. Older lesions may show considerable fibrosis of the stroma and foci of
osteoid formation.
Radiographic findings

Location :

Mandible > Maxilla.

The epicentre of the lesion to be anterior to the first


molar in the mandible and

anterior to the canine in the maxilla.

In older individuals
this lesion can occur in greater frequency in the
posterior aspect of the jaws.
Periphery

• It usually produces a well-defined radiographic margin in the mandible.

• Lesions in the maxilla may have ill-defined, almost malignant-


appearing,
borders.

• In most cases the periphery shows no evidence


of cortication.
Internal structure

• Mostly small lesions are unilocular and


completely radiolucent.

•Some lesion can be mixed radiopaque and


radiolucent multilocular.

•With presence of granular pattern of


calcification.
• Or organized into ill-defined, fine septa.

If present which characteristically , emanate at right angles from the periphery of the
lesion. A small indentation of the expanded cortical margin is seen at the point where
this right-angled septum originate.
Effect on surrounding structure

•Displacement and root resorbtion can be there teeth.


The lamina dura of teeth within the lesion usually is missing.

•The inferior alveolar canal may be displaced in an inferior direction.

•Expansion of the cortical boundaries are present.

•The expansion usually is uneven in nature, which may give the


appearance of a double boundaries

•Sometimes cortical plate perforation can be there


Commonly buccal cortical plate of bone
Common in the maxilla.
Differential diagnosis

A) Unilocular , completly radiolucent


1. Cystic lesion
2. Brown tumor of hyperparathyroidism
3. Cherubism

B) Multilocular radiolucency
1. Ameloblastoma
2. Aneurysmal bone cyst
3. Odontogenic myxoma
Treatment and Prognosis

• Surgical : Curettage

•For large aggressive lesions :


(1) Intralesional corticosteroids
(2) Calcitonin, and
(3) Interferon alfa-2a

• Recurrence rate : 11% to 20% reported


Peripheral giant cell granuloma
•Giant cell epulis. Giant cell hyperplasia

•Most common giant cell lesion in oral cavity.

•They are tumour like growth of oral cavity.

•They are not true neoplasm but reactive lesion caused by local irritation or
trauma.

•Source of giant cell in lesion

1. Osteoclast
Some researchers suggest it is soft tissue counterpart of CGCG.
That eroded through cortical plate into gingival soft tissues.

2. Mononuclear phagocyte system


Clinical features
1. Can occur in any age from 1st to 6th decades of life .

2. Common in females

3. Exclusively on the gingiva or edentulous alveolar


ridge,

4. It may develop in either the anterior or posterior


regions of the gingiva or alveolar mucosa.

5. Mandible is commonly affected then maxilla.

6. Presenting as a red or red-blue nodular mass

7. Most lesions are smaller than 2 cm in diameter,


although larger ones are seen occasionally.

8. The lesion can be sessile or pedunculated .


Histopathology

1. Vascular granulomatous tissue stroma consisting of

• Plump ovoid spindle shaped mesenchymal cells.


• Round monocyte-macrophages.
• Giant cell which can ostoclast. which can be present
focally or diffusely.

2. These mesenchymal cells give rise to moncyte-


macrophage and promote formation of ostoclast like giant
cell from macrophages.

3. Areas of erythrocyte extravasations, destruction and hemosiderin deposition


characteristically present in entire mass

4. Dense fibrous connective tissue separate giant cell proliferation from mucosa.
Radiographic findings

“Cupping resorbtion” or “saucerisation” of alveolar crest.

Can be seen in underlying alveolar bone.


Differential Diagnosis

1. Pyogenic granuloma

2. Fibroma

3. Hemangioma

4. Lymphangioma
Treatment

1. Removal of chronic source of irritation.


2. Surgical excision.
Giant cell fibroma
It is a fibrous tumour.
Arise from fibroblast
Clinical features
1. The giant cell fibroma is typically an asymptomatic sessile
or pedunculated nodule, usually less than 1 cm
in size.
2. The surface of the mass often appears papillary.
3. The lesion usually occurs at a younger age.
4. Mostly diagnosed during the first 3 decades of life.
5. Show female predilection.
6. Common site is gingiva.
7. The mandibular gingiva is affected twice as often as the
maxillary gingiva.
The tongue and palate also are common sites
Retrocuspid papilla

•It occurs on lingual gingiva in mandibular canine region.

•Commonly Bilateral.

•Appear as pink papule, measuring 5mm in diameter.

•They appear in Children and young adults and disappear in old age.

•They represent normal anatomic variation that disappears with age.

•Histologically it is similar to fibroma.


Histopathology

•Mass of vascular fibrous connective tissue which is usually


loosely arranged .

•The hallmark is the presence of numerous large, stellate


fibroblasts within the superficial connective tissue.

•These cells may contain several nuclei.

•The covering epithelium often is thin and atrophic.

•Although the rete ridges may appear narrow and elongated .


Differential Diagnosis

1. Fibroma

Causative factor : Chronic irritation


Age: 4 to 6 decades of life

Treatment

Surgical excision

Recurrence is rare

Retrocuspid papillae does not require surgical excision.


Osteosarcoma
• Malignancy of mesenchymal cells that have ability to produce osteoid or immature
bone .

•After hematopoietic neoplasms, osteosarcoma is most common malignancy of bone.

Clinical features:
Extragnathic
• Bimodal age distribution.
•Most arise in patients between10 and 20 years, with a lesser
number diagnosed in adults older than age 50.

•Common site :
Distal femoral and proximal tibial metaphysis.
•In older patients, the axial skeleton and flat bones are
involved most frequently.

•Risk factors : Paget’s disease and previous irradiation


Osteosarcoma of Jaw
• 7% of ostosarcoma

•Common Age: third and fourth decades of life.

•Slight male predominance is noted.

• Mandible is commonly effect than maxilla.

•Mandibular tumors arise more frequently in the posterior body and horizontal ramus
Maxillary lesions are discovered more commonly in the inferior portion
(alveolar ridge, sinus floor and palate) than the superior aspects (zygoma, orbital
rim).

•Swelling and pain are the most common symptoms

• Loosening of teeth or paresthesia can be there.


•Osteosarcoma metastasize exclusively by hematogenous spread .

•Pulmonary metastasis being most common.

•Lymph node involvement is rare.

•Osteosarcoma is most common postirradiation sarcomas.

•As jaws are situated closely to tissue that commonly receive therapeutic radiation.
thus they are common site for postirradiation bone sarcomas.

•Features and prognosis is similar to Osteosarcoma de novo.


Peripheral Osteosarcoma
Juxtacortical Osteosarcoma

• The periosteal form of osteosarcoma is


a sessile lesion that arises within the
cortex and elevates the overlying
periosteum.

•It promotes significant peripheral


periosteal new bone formation.

•Often the leading edge of the tumor


mass perforates the surface of the
periosteum and extends into the
surrounding soft tissue..

•Prognosis is better than intramedullary


tumors, but poorer than the parosteal
variant.
•The parosteal type of osteosarcoma is a
lobulated
nodule attached to the cortex by a short stalk.

•There is no elevation of the periosteum and no


peripheral periosteal reaction.

•It is a low-grade sarcoma that has a small risk


of recurrence and metastasis.
Radiographic findings

There can be dense sclerosis, mixed sclerotic and radiolucent lesion or completely
radiolucent lesion

Periphery and shape


• illdefined
•No peripheral sclerosis or encapsulation.
Periosteum can be displaced or partially destroyed or disorganised

Many thin irregular spicules of new bone formed


project outward and perpendicular to surface of lesion
forms “sunburst appearance”

Destruction of cortical bone followed by periosteal


reaction at periphery forms two triangle radiopacites
project from cortex at lateral extremities of lesion.
This is known as “Codman’s triangle”

When periosteum
is raised,
breached in
centre. But it
maintains its
osteogenic
potential at
periphery.
Internal Structure
• Completely radiolucent, mixed radiolucent- radiopaque or radiopaque.

•Areas of bone destruction(radiolucent)

• Area of Bone formed that can give appearance of


• Granular- appearing bone.
•Sclerotic appearing bone.
•Cotton bolls
•Wips
•Honeycombed
“Cumulus -Cloud” appearance

•Normal trabecule of bone is lost


Effect on surrounding structure

• Band like widening of periodontal ligament.


•Usually limited to one side of root.
•Infiltration into periodontal ligament space

•The antral or nasal wall cortices may be lost in maxillary lesions.

• Mandibular lesions may destroy the cortex of the mandibular canal


Garrington’s sign
OR
the mandibular canal may be symmetrically widened and enlarged.
Histopathology
• Malignant mesenchymal cells are present which directly form osteoid.

•There is also presence of small cells, epitheloid cells histeocytoma-like and


giant cell.

The tumor cells may have uniform round or spindle-shaped cells or highly
pleomorphic cells with bizarre nuclear and cytoplasmic shapes.
These malignant cells of tumour may also form chondroid or fibrous connective
tissue

Depending on predominance pathologist classify Osteosarcoma into


 Osteoblastic osteosarcoma
 Chondroblasstic osteosarcoma
 Fibroblastic osteosarcoma
These histological subtypes has similar prognosis
Differential Diagnosis

Completely radiolucent Spicules periosteal new bone

•Metastatic carcinoma •Metastatic tumor from breast and prostate


•Fibrosarcoma •Ossifying subperiosteal hematoma

Mixed

•Chondrosarcoma
•Peripheral fibroma with calcifications
•Chronic osteomyelitis
Treatment and prognosis

• Preoperative chemotherapy followed by radical surgical excision

• Osteosarcoma of jaw is less aggressive then extragnathic osteosarcoma .

•Distinct metastasize by hematogenous spread to lungs.

• Lymphatic spread is rare.

• Several studies indicated survival rate upto 30% to 70%.


Rhabdomyosarcoma
•It is malignant tumor of striated muscle.
•Derived from primitive mesenchymeal cells.

•This sarcoma is common in early age of life.


Most cases reported within 10 to 20 years of age

Clinical features:
•Age : Young age
Various reviews reported mean age of 6 to 16 years.

•Chief presenting complaint : Rapid swelling


Painful if nerve involvement

• Head and neck sarcoma may show


Divergence of eye Dysphagia
Abnormal phonation Aural discharge
Deviation of jaws
•Common site: Head and neck region and genitourinary tract.
• Most of head and neck lesions are common in embryonal and alveolar types.
•Pleomormpic rhabdomyosarcoma is common in extremities.

•Head and neck common site: facial muscle orbital muscles.


•Intraoral site : Soft Palate
Tongue, floor of mouth and buccal mucosa

•Gender predilection : Males

•Extensive infiltration and invade into bone

• Show Distant metastasis


Histological types with distinct clinical features :

1. Embryonal
2. Botryoid
3. Alveolar
4. Pleomorphic.
1. Embryonal

• Common in first decade of life.

• Head and neck

•Better prognosis

• Histolopathologically:

Well diffentiated type


Eosinophilic spindle cells with presence of cross striation.
Some of them can show interlacing arrangement.
Present in loose to dense fibrous tissue with presence of myxoid zones.

Poorly differentiated type may be difficult to diagnose


and consist of small round or oval cells with hyperchromatic nuclei and indistinct
cytoplasm.
2. Botryoid

•Subtype of embryonal rhabdomyosarcoma.


•Common site is vagina, prostate and base of bladder
•Head and neck region involving maxillary sinus, masopharynx middle ear also seen

•Histopathological:
•Less cellular(mesenchymal ) with prominent myxoid stroma.
•Presence of peripheral zone of rich cellularity known as “cambium layer”.
Expansile osteolytic lesion of right maxilla

Embryonal rhabdomyosarcoma
3. Alveolar
•Common in extremities
•Can be seen in head and neck region.
•Poor prognosis

•Histopathological:
•Aggregates of poorly differentiated round to oval cells separated by fibrous septa.

• These cells in centre demonstrate loss of cohesiveness, which results in appearance


of cell floating freely within the alveolar spaces.

•The peripheral cells of these aggregates adhere to the septal walls in single layer.

•There is presence of multinucleated giant cells.

•Mitotic figures are common.


4. Pleomorphic
•Least common type of rhabdomyosarcoma .
• Usually seen in extremities
•Seen in older individuals.

•Histopathological:

•It is composed of spindle shaped cells arranged in haphazard pattern.


• These cells are large and show considerable variation in apperence.
Treatment
• Local surgical excision followed by multiagent chemotherapy

•Postoperative radiotherapy is required for cases where complete resection cannot be


done.
Hodgkin’s lymphoma
•Lymphoma is neoplastic proliferation of lymphoid tissue, lymphocytes or histiocytes

•Hodgkin’s disease primarily arise within lymph node followed by extranodal sites
secondarily .

Etiology
the cause of this disease is unknown, but could be related with Epstein Barr Virus.

Neoplastic cells known as Reed- Sternberg cells are B-lymphocytic in origin

They are precursors of mononuclear cells that continue division and fusion with
each other that leads to formation of multinucleated giant cells .
Clinical Features
• The most common sites of initial presentation are the cervical and supraclavicular
nodes .
Followed by axillary and mediastinal node, abdominal and inguinal lymph nodes.

• Male predilection is observed.

•Age: Bimodal pattern is noted

Peak is observed between 15 and 35 years of age;


another peak is seen after the age of 50 years of age.

• Presenting sign : persistently enlarging, nontender,


discrete mass or masses in one lymph node region

• Characteristically firm and rubbery on palpation.


• In the early stages, the involved lymph nodes are movable but as the condition
progresses, the nodes become matted and fixed to the surrounding
tissues.

• If it is untreated, then the condition spreads to other lymph node groups.

• Eventually involves spleen and other extralymphatic tissues such as bone,


liver, and lung.

• Extralymphatic (Secondarily) oral involvement is rare.

• Constitutional symptoms:
1. Weight loss
2. Low grade fever
3. Night sweats
4. Generalized pruritus (itching).
5. Malaise

Absence of these systemic signs and symptoms is assigned as Category A


Presence of systemic signs and symptoms is assigned as Category B
• Category A has better prognosis then Category B.
Staging of Hodgkin’s lymphoma
Ann Arbor System for classification of Hodgkin’s Lymphoma

Stage Features

I Involvement of a single lymph node region (I) or a single


extralymphatic organ or site (IE)

II Involvement of two or more lymph node regions on the same


side of the diaphragm (II) or
one or more lymph node regions with an extralymphatic site (IIE)
III Involvement of lymph node regions on both sides of the
diaphragm (III), possibly with an extralymphatic organ
or site (IIIE), the spleen (IIIS), or both (IIISE).
IV Diffuse or disseminated involvement of one or more
extralymphatic organs (identifi ed by symbols), with or
without associated lymph node involvement
Histopathology

Reed – Sternberg cell are neoplastic cells


They are giant cells.

Types :

1. Classic Reed – Sternberg cells


• Large cells
• Biolobed nucleus appearing mirror image of each other.
• Each having prominent eosinophilic nucleolus with a clear halo around it
Giving “Owl eye” appearance.
2. Lacunar Reed Sternberg cells
• Present in a pericellular space
• Artifact formed due to shrinkage of cytoplasm
during fixation with formalin.

3. Polypoid cells Reed Sternberg cells

They have lobulated nucleus in shape of a popcorn “Exploded kernel of corn” .

4. Plemorphic Reed Sternberg cells


They have pleomorphic atypical nuclei
Histological type of Hodgkin's lymphoma

Nodular lymphocyte predominant Classical Hodgkin's lymphoma

• Lymphocyte rich

• Nodular sclerosis

• Mixed Cellularity

• Lymphocyte deplition

• Unclassifiable
Type Reed – Sternberg cell Additional Features

Nodular lymphocyte predominant


Popcorn cells Less common
Classical Hodgkin's lymphoma
Lymphocyte rich Few Classic cells or Sheets of proliferating
lymphocytes are also
present
Nodular sclerosis Lacunar cells Broad bands of collagen
fiber present .

Most common
Mixed Cellularity Abundant Classic cells Mixture of plasma cells,
small lymphocytes,
eosinophils and
histiocytes also present
Lymphocyte Bizzare giant Reed Few lymphoctes
deplition Sternberg cells present Most aggressive type
Unclassifiable They do not fit into above subtype thus designated
as unclassifiable.
Differential diagnosis

Multiple and disseminated lymphomas in advanced cases


can differentiated form:

1. Viral lymphadenitis: lymph nodes are tender on palpation.


2. Infective mononucleosis : Paul- Bunnel heterophil test positive
(Patient’s serum is tested for presence of antibody
against EBV)
Treatment

Stage I and II with local radiation therapy.

•Combination of less extensive radiotherapy fields with multiagent chemotherapy


regimens to maximize
disease control and minimize long-term complications can be considered.

Patients with stage III or IV disease require chemotherapy and radiation therapy
Prognosis

Stage I and II with local radiation therapy.

80% to 90% relapse free 10 years survival rate

Stage III and Stage IV


55 % to 75% 10 years survival rate

Histological types
Lymphocyte predominant and Nodular sclerosis : Best prognosis

Mixed cellularity : Less favorable

Lymphocyte depletion : Poor prognosis


Cystic lesions
Traumatic Bone cyst
Also known as Simple Bone cyst Progressive bony cyst
Hemorrhagic bone cyst Blood cyst
Solitary bone cyst Extravasation cyst
Idiopathic bone cavity Unicameral bone cyst

• It is false cyst of bone

Because benign, empty, or fluid containing cavity within bone that does not
have epithelial lining .

• An SBC is a cavity within bone that is lined with connective tissue.


•It may be empty, or it may contain serosanguineous fluid.
Clinical features:

1. Lesion is usually discovered on routine radiographs and is asymptomatic.

2. Patient may give history of trauma.

3. Large lesion may cause expansion of the cortical plates without erosion.
Producing hard bulge on jaw.

4. Long bones commonly affected humerus and femur.

5. Mandible is commonly affected then maxilla.

6. Premolar and molar regions are the most common locations,


Symphysis is also frequently involved .
6. Teeth involved are vital without tipping, migration and root resorption or loss of
lamina dura.

7. Occur in patients under 25 years of age.

8. Aspiration usually is negative, but in some cases serosanguineous fluid, a small


quantity of blood. or a serumlike fluid may be obtained.
Pathogenesis
Most accepted theory

1. Trauma Hemorrhage theory :

Trauma to the bone that is insufficient to cause a fracture

intraosseous hematoma formation

hematoma does not undergo organization and repair

it may liquefy and result in a cystic defect


Other etiologic theories include:

2. Inability of interstitial fluid to exit the bone because of inadequate venous


drainage.

3. Local disturbance in bone growth.

4. Ischemic marrow necrosis.

5. Localized alteration in bone metabolism resulting in osteolysis.

6. Aberration in normal bone remodelling.


Radiographic features

Location :
Most common in the mandible.
In rare cases they develop in the maxilla.

The lesion can occur


anywhere in the mandible but is seen most often in the
ramus and posterior mandible.

Periphery and shape:


The boundary usually well defined in the
alveolar process and around the teeth
than in the inferior aspect of the body of the
mandible

It often shows domelike projections that


scallop upward
between the roots
Internal Structure :

• It is usually unilocular and completely radiolucent.

• Sometimes may appear multilocular


But does not have true septa
It is scalloping of endosteal surface of buccal or
lingual plate
Effect on surrounding structure:

•No effect on the surrounding teeth.

•Lamina dura remain intact or only partly disrupted.

•Similarly cortical boundary of the crypt around a


developing tooth is unaffected .

•It has tendency to grow along the long axis of


the bone, causing minimal expansion.

•However, expansion of the involved bone can occur and


in larger lesions.
Histopathology

1. Epithelail lining is absent.

2. Walls of the defect may be lined by a thin band of vascular fibrous connective
tissue .
3. Thickened myxofibromatous proliferation intermixed with trabeculae of
cellular and reactive bone can be present as lining.

4. This lining may exhibit occasional giant cells adjacent to the


bone and often shows resorptive areas (Howship’s lacunae) indicative of past
osteoclastic activity.
Differential diagnosis

1. Radicular cyst:

Non vital tooth, absence of lamina dura, a radiolucency with epicenter at periapex of
tooth.

2. Periapical cemento osseous dysplasia

PCOD is not more than 0.7 cm in diameter ; Traumatic bone cyst are larger than 1 cm.
PCOD is seen in patients over 30 years of age
Common site mandibular central incisors
Show maturational changes (calcification)

3. Median mandibular cyst


Present in midline of 2 halves of mandible
Show separation of teeth
4. Keratocystic odontogenic tumour

usually have a more well defined cortical boundary,


resorb and displace teeth,
and occur in an older age group (10 to 30 years)

5. Malignancy

Ill defined borders suggestive of invasive peripheral bone destruction


Widening of periodontal ligament space.
Treatment

Open the area surgically

On surgical exposure bone defect will be seen which is usually empty cavity with
smooth, shiny bony walls.

Some times the cavity will contain small amounts of serosanguineous fluid.
The mandibular neurovascular bundle may be seen lying free in the cavity.

removal of tissue debris present is done,


curette the walls of the bony cavity to induce bleeding

Clot organization followed by bone formation usually occurs.

Follow up to examine bone healing.


Aneurysmal Bone cyst

• It is false cyst of bone because of absence of epithelial lining.

•Blood filled spaces surrounded by cellular fibrous connective tissue and


reactive trabeculae of woven bone.

•It is reative lesion of bone.

•Recently translocation of chromosome is reported which may suggest


neoplastic nature of disease.
Pathogenesis
The cause and pathogenesis of the aneurysmal bone cyst
are poorly understood.

Some investigators suggest :


1. Associted with traumatic event of bone.
2. Vascular malformation or
3. Neoplasm
disrupts the normal osseous hemodynamics and leads to an enlarging,
hemorrhagic extravasation.

4. Close relation to central giant cell granuloma

The exaggerated proliferative vascular reaction is similar to that of the central


giant cell granuloma.

But continually effusion with circulating blood from the injured vessels is seen
in developing ABC ,

Not in granuloma .
5. Aneurysmal bone cyst as secondary lesion

ABC is formed as a microcyst that forms as a result of intercellular edema in


a primary lesion with loose, unsupported stroma.

It is supported by presence of ABC in

Giant cell granulomas


Fibrous dysplasias
Paget's disease
Fibrosarcomas
Osteosarcomas
Clinical features
• Site : most commonly in the shaft of a long bone or in the vertebral column.
Gnathic aneurysmal bone cysts are uncommon,.
If present common in madible then maxilla.
Mandibular molar region.

• Age : under 20 years of age.

•No gender predilection.

• It is slow growing lesion causes expansion and thinning of cortical plate


“Ballooning out of cortex”
•But it does not causes cortical destruction.

•It may be slightly tender on palpation.

• Teeth may be mobile, displaced or resorbed.

•It is usually solitary.


•Gross appearance of lesion at surgical site:

•Intact periosteum with thin shell of bone


•Soft and reddish brown lesion because of rich blood supply resembles a sponge
filled with blood giving “Blood soaked sponge ” appearance.
Histopathology

•Aneurysmal bone cyst is characterized by spaces of varying size, filed with


unclotted blood.

•surrounded by cellular fibroblastic tissue containing multinucleated giant cells and


trabeculae of osteoid and woven bone.

•On occasion, the wall contains an unusual lacelike pattern of calcification that is
uncommon in other intraosseous lesions.

•The bloodfilled spaces are not lined by endothelium


Radiographic appearance:

Location :

Mandible is involved more often than the


maxilla and the molar and ramus regions
are more involved than the anterior region.

Periphery and shape :


It is well defined, and
the shape is circular or “ hydraulic. ”
Internal Structure

•Small initial lesions can be completely radiolucent.


Axial CT image
•Common it has has a multilocular appearance.

•The septa bear a striking resemblance to the wispy, illdefined septa seen in giant cell
granulomas.

•Another similar finding is septa positioned at right angles to the outer expanded border.
Differential Diagnosis

1. Gaint cell granuloma


ABC show greater cortical expansion
They are common in posterior region of jaws

2. Ameloblastoma
Common in older age (30-70 years)

3. Cherubism
Multifocal bilateral disease
Treatment

Surgical curettement is the treatment of choice.

Large lesions may require surgical resection and immediate reconstruction

Reported recurrence rate:


8% to 60%

Specially when inadequate removal is done


Or related with untreated primary lesion.
Metabolic lesion
Hyperparathyroidism
Brown giant cell lesion of hyperparathyroidism

Excessive production of parathyroid hormone.

Primary hyperparathyroidism

Parathyroid adenoma or parathyroid hyperplasia

Secondary hyperparathyroidism

In response to chronic low levels of serum calcium level

As in case of Renal osteodystrophy (Decreased synthesis of Vitamin D3)


Osteomalacia (Lack or inability to use dietary calcium cased by deficiency
of vitamin D)
Clinical features

1. Common in females

2. Age : 30 to 60 years of age.

3. Metastatic calcification is common


Most commmonly Nephrolithiasis or nephrocalcinosis.

Other soft tissues that may show ectopic calcification are the
subcutaneous tissues, walls of blood vessels, articular
cartilages, and joint capsules.
Lithiasis frequently develops in the pancreas and salivary glands.

4. Patients complain of weakness, anorexia, nausea, vomiting, constipation,


abdominal pains, muscular and
joint pains, polyuria, polydipsia, and emotional instability
Brown tumor

This lesion derives its name from the


color of the tissue specimen, which is usually a
dark red-brown because of the abundant
hemorrhage and hemosiderin deposition within
the tumor..
Histopathology

1. Proliferation of vascular granulation tissue, which serves


as a background for numerous multinucleated osteoclast-type giant cells.

2. Some lesions may also show a proliferative response characterized by a


parallel arrangement of spicules of woven bone set
in a cellular fibroblastic background with variable numbers of multinucleated giant
cells.
Radiographic features

1. Earliest and most reliable changes of hyperparathyroidism are


erosions of bone from the subperiosteal surfaces of the middle phalanges of the hands

2. Osteitis fibrosa cystica are localized regions of bone


loss leading to decrease in density of bone.

3. calvarium shows a granular appearance caused by the


loss of central (diploic) trabeculae and thinning of the
cortical tables.

Classically known as
“Salt and paper” skull
4. Bone remodelling leads to replacement of normal trabeculae by numerous small
randomly oriented trabeculae, resulting in “ground glass apperarence”.

5.Brown tumors of hyperparathyroidism

•They may appear in any bone


But are commonly found in the facial bones and jaws.
•Seen in long-standing cases of the disease.
•These lesions are solitary may be multiple
within a single bone.
•They have defined margins and can be multilocular.
•They may produce cortical expansion.

6. Loss of lamia dura that may give tappered


tooth appearance.

7. Because of decreased density of bone and


Loss of lamina dura, tooth appear more
radiopaque
Lab investigation

Normal

Serum calcium level : Increased (8.5-10.2 mg/dL)

Serum alkaline phosphate : Elevated (44 to 147IU/L)

Serum level of parathyroid hormone : Increased (10-65pg/mL)


Treatment

1. Treatment of the primary type requires excision of the parathyroid tumors.

2. Secondary
High dietary calcium and vitamin D supplementation.
Calcitriol, an inhibitor of PTH syntheses and secretion.
Miscellaneous lesion
Langerhans cell Histiocytosis
Histiocytosis X Langerhans cell granuloma
Langerhans cell disease Langerhans cell granulomatosis
Idiopathic histocytosis Self healing histocytosis
Eosinophilic granuloma

Histiocytosis X because cause of reactive proliferation of histiocytes in unknown.

Exact etiology in unkown.

Definition

Langerhans cell histiocytosis is a group of idiopathic disorders characterized by clonal


proliferation of specialized bone marrow derived, antigen presenting dendritic cells
called Langerhans cells mature eosinophils.
Clonal proliferation does not necessarily mean neoplastic process as :

1. There is no alteration in number or morphology of chromosomes in cells.

2. Survival rate of patient increases with age.

3. Healing of lesion by its own

Resent study shows there is excessive uncontrolled production of cytokine


which explain recruitment of inflammatory cells and Langerhans cells followed by bone
resorbtion, fibrosis and necrosis.
General clinical features

Age: Children 1-4 years


Mostly under 15 years of age

Gender : Common in males then females.

Clinicopathological presentation

Monostotic or Acute disseminated


Polyostotic histiocytosis
Chronic disseminated
Eosinophilic granuloma histiocytosis Letterer – Siwe disease
Hand Schüller Christian disease

Bone Skin Visceral


involvement
Mobility of teeth, Loss of alveolar bone and premature loss of teeth which mimic
advance Periodontitis.

Bone lesion may break out and produce


soft tissue mass.
Radiological findings

1. Punched out radiolucency


Well defined radiolucency without radiopaque corticated rim.

2. Scooped out appearance

Destruction of superficial alveolar bone.


Common in posterior region of mandible.
Leads to lessening of teeth or giving “teeth floating in air” appearance.
Mimic aggressive Periodontitis .
Radiological findings

1. Punched out radiolucency


Well defined radiolucency without radiopaque corticated rim.

2. Scooped out appearance

Destruction of superficial alveolar bone.


Common in posterior region of mandible.
Leads to lessening of teeth or giving “teeth floating in air” appearance.
Mimic aggressive Periodontitis .
Hand-Schüller-Christian Disease
Chronic disseminated histiocytosis

Clinical features

1. Bone : “ punchedout ” bone destruction in the skull.


2. Unilateral or bilateral exophthalmos.
3. diabetes insipidus .

4. with or without other manifestations of dyspituitarism such as dwarfism or


infantilism.

5. Other bones affected are Femur, Ribs, Vertebra, Pelvis, Humerus and Scapula.
Oral manifestation

nonspecific and include

1. Sore mouths
2. Most commonly ulcerative lesions
3. Halitosis
4. Gingivitis and suppuration
5. Unpleasant taste
6. Loose and sore teeth with early exfoliation of teeth
7. Failure of healing of tooth sockets following extraction
Histopathology

1. Initial phase: Proliferation of histiocytes and accumulation of collections of in


scattered sheets throughout.

2. Vascular-granulomatous phase with persistence of histiocytes and eosinophils,


sometimes with aggregation of lipid laden (cholesterol) macrophages(‘foam
cells’).

3. Xanthomatous phase with abundance of these ‘foam cells’.

4. Tissue cholesterol level raise significantly but not serum cholesterol.

5. Some of histiocytes coalases to form multinucleated giant cells.

6. Fibrous or healing phase.


To demonstrate Langerhans cell immunostainig is done and viewed under electron
microscope

• Langerhans cell stain positive to S-100 protein and peanut agglutinin and
demonstrate rod shaped cytoplasmic structures called as Birbeck or
Langerhans cell granules.

Birbeck or Langerhans cell granules has dilated terminal ends giving tannis racket
appearance.

Demonstration of CD1 antigen on cell surface can also be used to differentiate


Langerhans cell

Langerhans cell under electron microscope


Prognosis

Depends on extent of lesion specially number of organ involved.


Overall prognosis: Good

Treatment

• Most of time spontaneous remission is seen

• If treatment is required

If lesion is approachable : Excision or curettage.

Inapproachable : irradiation
Chemotherapy : Prednisone, Vinblastine, cyclophosphamide
Cherubism
Clinical features
•It is autosomal dominant genetic defect that affects bone
remodeling in specific anatomically confined limits of
embryologic mandible and some time mandible and maxilla.
Both.

•First begins to manifest itself by age of 2.5 yearrs and fully


expressed by the age of 5 yrs.

•Affects males slightly more then females.

•Involvement of maxilla contributes to the expansion of the


maxilla gives a child a cherubism the “Chubby face
appeareance”.
Radiographic features
The lesion is bilateral and often affects both the jaws, the mandible is
the most common location.

The epicentre is always in the posterior aspect of jaws, in the ramus of


mandible or tuberosity of maxilla.

The lesion grows in an anterior direction and in severe cases can


extends almost to mid line.

The periphery is well defined and in some instances corticated.

There is Expansion of cortices of the mandible

Teeth are displaced anteriorly as lesions expand


In some lesions tooth bud are destroyed.
Histopathology

• The microscopic findings of cherubism


are essentially similar to those of isolated
gaint cell granulomas,

• The lesion tissue consists of vascular


fibrous tissue containing variable number
of multinucleated gaint cells.
Differential diagnosis

GIANT CELL GRANULOMA: Cherubism is bilateral with an


epicenter in the ramus should provide a clear differentiation.

FIBROUS DYSPLASIA : Fibrous dysplasia is more commonly


a unilateral disease; also the multilocular appearance and
anterior displacement of teeth are more characteristic of
cherubism.

ODONTOGENIC KERATOCYSTS : Cherubism may bear some


similarity to multiple odontogenic keratocysts in basal cell
nevus syndrome. The bilateral symmetry of cherubism, along
with the anterior displacement of teeth and pronounced
multilocular appearance, help with the differential diagnosis.
Treatment
•Treatment can be delayed because the cyst like lesion usually become static
and fill in with granular bone.

• After skeletal growth has stopped , conventional surgical procedure, if


required may be done for cosmetic problem, surgery may also be required to
uncover displaced teeth,

•orthodontic treatment may be needed.


Paget disease
Clinical features
•It is a condition of excessive bone resorption followed by disorganized repair.

•An increase in vascularity develop to cope up with demand of new bone


formation.

•Patient often complain of deep bone pain as an early symptom.

•The pagetoid bone is structurally weak, leading to bowed tibia, kyphosis or


frequent fractures of long bone , depending on bone involvement.
 Paget’s disease usually occurs in individual
older than 50 years.

 Man are affected more frequently than women

 The bone most commonly affected by are


spine, femur, skull,pelvis and sternum .
•The patient with jaw involvement
present with expansion and bone pain.

•Affected area often feel warm with


visibly enlarged vein or a bluish hue
because of increased vascularity.

•Teeth will have diastema and lingual


inclination
Radiographic features

Bone appear as a mottled mixed of radio-opacities


and radioluciencies.

The ratio of these elements depends on the


duration of disease.

The radiographic pattern has been


termed as cotton wool appeareance
because it is characterized by fluffed
, radiodense, cloud like aggregation.

In jaw these bone may cause root


resorption as well as tooth
displacement.
The involvement of mandible
may occasionally reveals the
uptake of radiolucency from one
condyle to another condyle giving
rise to Lincon’s Sign or Black
Beard Sign.

The coronal view of computed


tomography shows widened diploic
space with sclerotic and lytic areas
involving inner and outer table of
bony calvarium giving cotton wool
appeareance.
• A radiopaque painfull expansion of jaws in an adult may be suggestive of
osteomyelitis, particularly chronic diffuse sclerosing osteomyeleitis.

• Fibrous dysplasia may resembles pagets disease radiographically but


would have been present from early age.

• Radio opacities of florid cemmento osseous dysplasia also mimic the


cotton wool appeareance of pagets disease and may be painfull from
secondry infection.
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