GIANT CELL LESIONS OF THE JAWS

DR KRISHNA KISHOR

DEPT OF ORAL & MAXILLOFACIAL SURGERY

SGT DENTAL COLLEGE

CONTENTS

DEFINITION OF GIANT CELLS

ORIGIN

CLASSIFICATION OF GIANT CELL LESIONS

GIANT CELL GRANULOMA / TUMOR

ANEURYSMAL BONE CYST

TRAUMATIC BONE CYST

OSTEOID OSTEOMA / OSTEOBLASTOMA

CHERUBISM

BROWN TUMOR OF HYPERPARATHYROIDISM

HISTIOCYTOSIS

DEFINITION

Giant cells are very large, multinucleate, modified macrophages which may be formed by coalescence of mononuclear cells or by nuclear division without cytoplasmic division of monocytes, particularly in response to the presence of a foreign body.

ORIGIN

Giant cells are derived from the cells of mononuclear

phagocyte system.

Originate from precursor cell in the bone marrow and also

closely related cells of bone marrow origin.

They are transported in blood as monocytes, which are

pool of immature cells.

Monocytes invade areas of damage & inflammation, where

they differentiate into macrophages.

When the macrophages fail to deal with particles to be

removed they fuse together to form multinucleated giant

cells.

CLASSIFICATION OF GIANT CELL LESION

Inflammatory / Reactive:

• Peripheral Giant Cell

Granuloma

• Central Giant Cell

Granuloma

• Aneurysmal Bone Cyst

• Traumatic Bone Cyst

Metabolic:

• Hyperparathyroidism

• Cherubism

• Histiocytosis

Neoplastic:

• Central Giant Cell Tumor

• Osteoid osteoma /

Osteoblastoma

CENTRAL GIANT CELL GRANULOMA

• Was 1st described in jaws by Jaffe (1953).

• Designated as Giant Cell Reparative Granuloma.

• Waldron & Shafer (1966)

• Classified on the basis of biologic behavior as:

Non-aggressive & Aggressive.

Etiology:

• Reactive lesion

• Trauma

• Origin from odontoclasts

Clinical Features:

• Male : female ratio of 1:2

• Age: 11-30yrs

• Present almost exclusively in jaws

• Mandible > maxilla

• Frequent site in mandible is the ant. region not crossing

midline.

• Non-aggressive type:

Asymptomatic, slow

expansion of the affected

bone.

• Aggressive type:

Painful, rapid growth, root

resorption, perforation of

cortical bone, paraesthesia.

Radiographic Features:

• Solitary unicystic radiolucency,

as it grows it becomes

multilocular with soap-bubble

appearance.

• Multilocular > unilocular

• Root displacement / resorption

• Loss of lamina dura

• Expansion of cortical plate

Histologic Features:• Proliferation of spindle cells in collagenous stroma.• Numerous small vascular channels.• Giant cells with 15-20 nuclei present throughout the

stroma, adjacent to capillaries.

Treatment:

Intralesional steroids:

• Triamcinolone – 20 mg/cc once per wk for 6 wks

• Suppresses inflammatory component of lesion

Calcitonin – s.c. inj.:

• Dose – 20 IU O.D.

• Antagonizes bone resorption by inhibiting Giant cells.

JOMS 61:649-53,2003

CASE REPORT (JOMS 61:649-53,2003)

α-Interferon – s.c. inj.:

• Dose – 30 lac IU O.D.

• Suppresses angiogenic component of lesion.

Surgical:

• Curettage

• Enucleation

Surgical technique(JOMS 60:756-61;2002 )

• Incisions placed (1cm away from radiographic extent of the lesion)

• mucoperiosteal flap raised

• Buccal bone overlying lesion removed

• Through curettage is done

• Peripheral ostectomy may be done Doesn’t spread via perineural spread so preserve

neurovascular bundle

Through debribment of bony cavity

• Control of hemorrhage

• Flap repositioned & sutured

Preserve teeth in area of lesion With good prognosis

(adequate bone support) Treat endodontically before surgery

Extract teeth in area of lesion With poor prognosis (poor bone support) Compromising access to the lesion

Recurrence

• Varies from 10 – 50 %• Higher for Locally aggressive lesion• Recurrent lesion respond well to further curettage, aggressive

lesions may require more radical surgery for cure.• Incomplete removal

GIANT CELL TUMOR

• Very rarely found in jaws.

• Aggressive – variant of low grade osteosarcoma

• H/F: similar to CGCG, except that the giant cells are larger with more nuclei and are more evenly spread.

• Treatment: Resection

( high recurrence rate)

PERIPHERAL GIANT CELL GRANULOMA

• Common tumor like growth in the oral cavity.

• Does not represent a true neoplasm but a reactive lesion.

• Arising from periosteum or PDL membrane.

• Often called as peripheral giant cell reparative granuloma.

C/F:

• Age: 5th 0r 6th decade of life.

• Common in females.

• Mandible is affected more often.

• Occurs exclusively on

gingiva, edentulous alveolar

ridge.

• Reddish or bluish nodule,

most lesions smaller than

2cm in diameter.

• May be ulcerated due to

trauma.

H/F:• Fibroblast are the basic element.• Giant cells are scattered throughout the stroma.• Foci of hemorrhage, liberation of hemosiderin pigment.

R/F:

• May or may not be present

• Larger lesion may exhibit superficial erosion of the

cortical bone

• May demonstrate widening of adjacent PDL spaces

Treatment:

Local surgical excision down to the underlying bone

Differential diagnosis• CGCG• Pyogenic granuloma

ANEURYSMAL BONE CYST

• First described by Jaffe & Lichtenstein in 1942.

• First case in jaw was reported by Bhaskar & Bernier in

1958.

• Is a non-neoplastic, reactive lesion of bone generally

consisting of several cavities filled with blood and

deprived of an endothelial lining.

• Not a true cyst, sinusoidal blood filled space.

• Word “Aneurysm” was used to describe the blown out

appearance of the contour of the affected area.

• Exist in two clinico-pathologic forms:

1. Primary

2. Secondary – arising in other osseous conditions like

Fibrous Dysplasia, Ossifying Fibroma, CGCG,

Osteoblastoma, Osteosarcoma, Ameloblastoma

Etiology:

Cause is controversial, the theories put forth regarding the

pathogenesis of the lesion are:

- Trauma

- Local changes in haemodynamics

- Reparative response to the hematoma

C/F:

• Occurs below 20 yrs of age.

• Predilection towards female.

• Mandible > maxilla, molar region is the most common site.

• Slow growing, may expand the cortical plate, does not

destroy them.

• Aggressive lesions may perforate cortical plate with soft

tissue extension.

• Teeth may be tender, missing or displaced.

• Pain is occasional complaint.

• On palpation: egg-shell crackling, non-pulsatile.

• On surgical exploration: ‘welling up’ of blood.

• Gross examination: blood-soaked sponge.

R/F:

• Well defined radiolucency

• Extreme expansion of cortical

plates - honey - comb or

soap - bubble appearance

• Can displace teeth

• Cortex may be destroyed

H/F:• Consist of a fibrous

connective tissue stroma containing of many sinusoidal blood filled spaces.

• Fibroblast are numerous as well multinucleated giant cells.

• Vascular spaces lack any endothelial lining, and giant cells form part of their walls.

Treatment:

• Surgical curettage & partial resection are primary means of

treatment.

• Cryotherapy.

• Recurrence rate is high, ranging from 19% to about 50%.

• Follow up is necessary.

• Differencial diagnosis• CGCG• Hyperparathyroidism• Cherubism

TRAUMATIC BONE CYST

Etiology:

• Trauma → Failure of organization of blood clot →

Degeneration of clot → Empty cavity within bone.

C/F:

• Young persons (<20 yrs).

• Male predilection.

• Mandible frequently affected.

• Asymptomatic, pain or tenderness rarely present.

• Rarely expansion of jaw.

• Teeth - vital, No tipping / migration.

• Aspiration – negative.

• On surgical exploration – usually empty cavity, sometimes

filled with little straw colored fluid.

R/F:

• Well defined, scalloped

margin.

• Oval / round shape.

• No root resorption.

• Lamina dura intact.

• Usually located above

mandibular canal.

H/F:

• Thin connective tissue

membrane.

• No epithelial lining.

• Presence of giant cells.

Treatment:

• Curettage

• Re-establish bleeding into the lesion.

OSTEOBLASTOMA

• It is a benign neoplasm of bone that arises from osteoblasts.

• Osteid osteoma is thought to represent a smaller version of the same tumor.

C/F:• Occurs mostly in young persons (<30yrs).

• Slight male predilection.

• Affects both the jaws, more in post region.

• Characterized by Pain and swelling at tumor site.

• Size: 2-4cm ,but may be larger than 10cms,

measuring <1.5cm is osteoid osteoma.

R/F:

• Well circumscribed.

• Radiolucent - mixed -

radiopaque pattern.

• A thin radiolucency may

be surrounding calcified

central tumor mass.

• Occasional sun-ray

appearance - resembling

osteosarcoma.

H/F:

• Irregular trabeculae of

osteoid & immature bone

within the stroma.

• Prominent vascular network.

• Actively proliferating

osteoblasts & moderate no.

of multinucleated giant

cells.

Treatment :

• surgical excision.

• Recurrence is rare.

• Malignant transformation into osteosarcoma has also been

reported.

• Differential diagnosisCementoblastomaOssifying fibromaFibrous dysplasia

CHERUBISM

• Rare developmental jaw condition.

• The condition is named due to the facial appearance

-plump cheeked little angels with upward directed look

depicted in renaissance painting.

Etiology:

• Inherited as an autosomal dominant trait.

• Gene for cherubism present on chromosome 4p16.3.

• Disturbance in development of permanent II & III molars.

• Dysregulation of mesenchymal bone formation and development of giant cell granulomas.

C/F:

• Occurs between the age of 2-5yrs.

• Progressive, painless, symmetric

swelling of the jaws- mandible or

maxilla.

• Cherub like facies arise from

bilateral involvement of posterior

mandible that tends to include the

angles & rami region - angelic

chubby cheeks.

• Eyes ‘up turned to heaven’

appearance – due to b/l maxillary

involvement.

• Lesion grows slowly but no perforation of cortex.

• Marked cervical lymphadenopathy.

• Premature shedding of deciduous teeth.

• Permanent dentition – teeth missing, failure of teeth

eruption, teeth displacement.

• Speech difficulty.

• Bony lesions regresses after puberty.

R/F:

• B/L expansion & thinning of cortical plates.

• Multilocular cystic appearance – teeth floating in spaces.

• Numerous unerupted & displaced teeth.

H/F:

• Consists variable number

of multinucleated giant

cells.

• Foci of extravasated blood

are commonly present.

• Cuff like deposits

surrounding small blood

vessels throughout the

lesion.

Treatment:

• The lesions tend to show varying degree of remission after

puberty.

• By 4th decade facial feature approach normalcy.

• Early surgical intervention for cosmesis has given good

results.

• Some studies showed the use of calcitonin, but still not

proved.

BROWN TUMOR OF HYPERPARATHYROIDISM

• Parathormone (PTH) is normally produced by parathyroid

glands, which regulates the Ca+ metabolism.

• Excessive production of PTH results in a condition known

as Hyperparathyroidism.

• It is an exaggerated form of ‘Osteitis fibrosa cystica’

discovered by von Recklinghausen in 1891.

Three types:

• Primary hyperparathyroidism

• Secondary hyperparathyroidism

• Tertiary hyperparathyroidism

C/F:

• Incidence: 1 in 500

• Predilection for females.

• Describes the features - ‘Stones , Bones, Abdominal

Groans & Psychic Moans’

• Metastatic calcifications - Nephrocalcinosis, blood vessels.

• Jaw - not as frequent as in long bones and skull.• Vague aches, severe bone pain, tenderness following

fractures.

• Swelling develops, firm in consistency, tender.• Mobility of teeth.

R/F:

• Sub-periosteal erosion of

middle phalanges is the

hallmark.

• Very rarely jaw affected

first.

• Generalized loss of lamina

dura.

• Ground glass appearance.

• Cortical plate may be

thinned or lost.

• There is a cystic type

of radiolucency.

• On gross examination-Vascularity, hemorrhage & deposits

of haemosidrin imparts a dark reddish brown color to the

lesion –”Brown Tumor”

H/F:

• Giant cells of osteoclastic origin scattered over the fibrovascular stroma in which foci of hemosiderin are present.

Lab. investigations:• Serum calcium level,

alkaline phosphatase & PTH level will be raised

Treatment:

• Primary:– Surgical excision of parathyroid adenoma.

– Bone lesions resolve spontaneously.

• Secondary:

– Management of kidney disorders.

• Tertiary:

– Oral Ca supplement

– Vit. D analogue

LANGERHANS CELL HISTIOCYTOSIS

• Results from abnormal proliferation of Langerhans cells or

their precursors.

• Langerhans cells are specialized cells of the histiocytic cell

line that normally are found in the skin.

Types :

• Eosinophilic granuloma (Solitary)

• Hand Schuller Christian disease (Chronic disseminated)

• Letterer Siwe disease (Acute disseminated)

Eosinophilic granuloma

C/F:

• Occurs in older children & young adults.

• Male > female

• May be asymptomatic – incidental finding on R/F.

• Affects skull & mandible, also long bones.

• Local pain, swelling, tenderness.

• General malaise and fever occasionally accompany.

• May cause bony swelling

and involve overlying soft

tissue.

• Gingival bleeding, pain &

ulceration.

• Loosening of the teeth

often occurs after

destruction of alveolar

bone.

R/F:

• Single or multiple irregular radiolucent lesions.

• Well circumscribed.

• Usually involving superficial alveolar bone.

• Cortex often destroyed.

• Tooth ‘floating in air’ appearance.

• Pathologic fractures may occur.

H/F:

• Sheets of Histiocytes.

• Histiocytes coalesce to

form Giant cells.

• Early lesion – large no.

of eosinophils.

• Mature lesion – fibrosis,

eosinophils decrease.

Treatment:

• Curettage

C/F:

• Occurs in early life (Age < 5 yrs).

• Widespread skeletal & extra-skeletal lesions.

• Chronic clinical course.

• Classic triad of:1. Multiple ‘punched-out’ lesions of skull.2. U/L or B/L Exophthalmos.3. Diabetes insipidus with or without Dyspituitarism.

Hand-Schuller-Christian Disease

• Oral manifestations – earliest signs of diseases.

• Stomatitis, Gingivitis, Halitosis

• Loose teeth, premature exfoliation.

• Failure of healing of post-extraction sockets.

• Loss of supporting alveolar bone mimicking advanced

periodontal disease – characteristic.

R/F:

• Individual lesions – sharply

outlined.

• Jaw lesions – more diffuse.

• Destruction of alveolar bone

with tooth displacement.

H/F:• Proliferative histiocytic phase:

eosinophils scattered throughout sheets of histiocytes.

• Vascular – granulomatous phase: histiocytes + eosinophils + Foam cells

• Diffuse xanthomatous phase: abundance of Foam cells

• Healing phase: fibrosis

Treatment:

• Spontaneous regression – approx. half of the pts.

• Curettage / excision

• Radiotherapy – inaccesible lesions

• Chemotherapy – vinblastine,

cyclophosphamide

• Prognosis – good

Letterer-Siwe Disease

C/F

• Occurs in infants (age < 3 yrs).

• Initially – skin rash of trunk, scalp & extremities.

• Low-grade fever, malaise, irritability.

• Splenomegaly

• Hepatomegaly

• Lymphadenopathy

• Nodular or Diffuse involvement of visceral organs –

Lungs & GIT.

• Diffuse involvement of skeletal system.

• Ulcerative lesions of oral mucosa.

• Gingival hyperplasia.

• Loosening & premature loss of teeth.

H/F:

• Histiocytic proliferation with or without eosinophils.

• Foam cells – not prominent.

• No fibrosis.

Treatment:

• Chemotherapy – only few pt. respond.

• Poor prognosis.

• Rapid course of disease – terminates fatally in short time.

REFERENCES

• Textbook of OMFS – Peterson• Textbook of OMFS – Laskin• Oral Pathology – Regezi• Oral Pathology – Shafer• Surgical Pathology – Cawson

• J Craniomaxfac Surg 2005: 33,61-68• Oral Surg Oral Med Oral Patho 2005: 99,464-70• J Oral Maxillofac Surg 2002: 60,1103-11• Am J Otolaryngol 2006: 27,281-86• J Craniomaxfac Surg 1998: 26,56-62