NONODONTOGENIC TUMOURS

#Tumours arise from the tissues other then the tooth or tooth-forming tissues
#comprises of tumours which are solely made up of bone or cartilage and bro-osseous lesions



*OSTEOMA

#benign tumours , consist of mature , compact , cancellous bone
#common in mandible
#characterised by proliferation of either compact or cancellous bone usually in an endosteal or
periosteal location
#1)COMPACT osteomas are slow growing and consist of dense lamellae of bone arranged like
layer of an onion with occasional blood vessels
#2)CANCELLOUS osteomas consist of trabecular of bone with marrow spaces in between and are
surrounded by a lamellated cortex
#most common in young adults
#2th-5th decades
#males=females


CLINICAL FEATURES

#Classied into two types
1)CENTRAL (ENDOSTEAL) OSTEOMAS
2)PERIPHERAL (PERIOSTEAL) OSTEOMAS
#that arise on the surface of bone from the periosteum or from the bone itself are called peripheral
osteomas
#contain either cancellous or compact bone
#occurs in maxilla (hard palate and sinus) and the mandible (angle more common)
#slow growing , asymptomatic , bony hard masses
#those develop from inner surface of the cortex of bone or centrally within the medullary bone are
called endosteal
#may cause localised expansion of the jaw

GARDNERS SYNDROME

#its an important marker of internal malignancy
#multiple peripheral osteomas of the jaws or sino-orbital region give rise to possibility of gardners
syndrome
#inherited as an autosomal dominant trait


the syndrome comprises :-

1)adenomatous polyps of the large intestine
2)multiple osteomas of the jaws , skull or long bones
3)multiple desmoid tumours
4)epidermal and trichilemmal cysts of skin
5)compound odontomas
6)impacted supernumerary & permanent teeth


RADIOGRAPHIC FEATURES

#well-circumscribed radiopaque mass surrounded by a radiolucent line


HISTOPATHOLOGIC FEATURES

#composed of either relatively dense mature , lamellar , compact bone with sparse marrow tissues
or lamellar trabecula-e of cancellous bone with abundant marrow tissues b/w the trabecula-e
#osteons and haversian canals are seen in the compact type


TREATMENT

#conservative excision


OSTEOID OSTEOMA

#Rare benign osseous tumour
#mandible>>maxilla
#more common in age less then 30 yrs
#males=females
#classical symptom is pain (worse at night)
#radiographically appear as a small radiolucency surrounded by densely sclerotic bone
#treatment is by excision or curettage

FIBRO-OSSEOUS LESIONS


#lesions of mesenchymal tissue origin

WLADRONS CLASSIFICATION


1)FIBROUS DYSPLASIA
*polyostotic
*monostotic

2)REACTIVE (DYSPLASTIC) LESIONS IN THE TOOTH BEARING AREA
*periapical cemento-osseous dysplasia
*focal cemento-osseous dysplasia
*orid cemento-osseous dysplasia

3)FIBRO-OSSEOUS NEOPLASMS
*cemento-ossifying broma
*ossifying broma
*cementifying broma



CEMENTO-OSSIFYING FIBROMA


#its a benign bro-osseous neoplasm arising from the undifferentiated cells of the periodontal
ligament tissue


CLINICAL FEATURES

#slow-growing , painless expansile lesions that replaces normal bone
#rst symptom - facial asymmetry
#commonly appear b/w 20-40 yrs
#females:males = 5:1
#common in the mandibular premolar or molar region
#juvenile presentation of the lesion are extremely aggressive


RADIOGRAPHIC FEATURES

#well-circumscribed
#appearence is variable
#fully mature lesiob appear as radiopaque mass surrounded by a thin radiolucent rim



HISTOPATHOLOGIC FEATURES

#Well-dened capsules , consist of spindle shaped broblast
#collagen bres are arranged in whorled or storiform pattern
#spicules of bone


TREATMENT

#readily enucleated by intra-oral approach
#good prognosis




FIBROUS DYSPLASIA

#replacement of normal bone by an excessive proliferation of brous connective tissue intermixed
with irregular bony trabecula-e
#congenital dysplastic disease of bone , that may occur one or multiple bones


PATHOGENESIS

#exact aetiology is unknown
#lichtenstien and jaffe indicated an aberrant activity in the bone -forming mesenchymal tissues as
the cause
#mutation in the gene that code for Gs alpha membrane associated protein in patient with brous
dysplasia may result in altered proliferation and differentiation of osteoblastic cells
#develop as a result of post zygotic mutation in the GNAS gene
#painless , slow enlargement of the involved bone
#lesion is recognised in the early childhood , grow slowly and stabilise in early adult life
#two forms -
*MONOSTOTIC-WHERE ONE BONE IS INVOLVED
*POLYOSTOTIC -SEVERAL BONES ALONG WITH CUTANEOUS AND ENDOCRINE
ABNORMALIES


MONOSTOTIC FIBROUS DYSPLASIA

#more common than polyostotic
#accounts for 80% cases
#characterised by focal but poorly circumscribed bro-osseous replacement of an area of bone
#maxilla (most affected site)
#frontal bone (commonly involved bone)






CLINICAL FEATURES

#1ST-2nd decades of life
#males=females
#diffuse painless , smoothly rounded swelling
#usually unilateral
#facial asymmetry as it grows
#buccal cortical plates is most commonly expanded
#interuption with normal occlusion and eruption
#maxillary lesions involves adjacent bones such as zygoma , sphenoid , maxillary sinus and oor
of orbit
#in mandible -mandibular body area is most commonly affected


RADIOGRAPHIC FEATURES

#homogenous radiopacity with numerous trabecula-e of woven bone (GROUND GLASS /
ORANGE PEEL TEXTURE)


HISTOPATHOLOGIC FEATURES

#loose cellular brous tissue interwoven with irregular trabecula-e and newly formed bone
#chinese characters trabecula-e
#lamellar bone formation is not seen (classical microscopic picture )



POLYOSTOTIC FIBROUS
DYSPLASIA


#uncommon
#several or many bones involved
#females:males=3:1
#McCune-Albright syndrome = polyostotic +brous dysplasia +skin pigmentation+endocrine
abnormalities
#more common in childhood



TREATMENT

#Radiotherapy is contra-indicated because of its potential for malignant transformation \
#biopsy for conrmation
#surgical intervention
#osseous contouring
#complete excision is not possible
CHERUBISM

#rare developmental jaw condition
#inherited as an autosomal dominant trait


CLINICAL FEATURES

#Onset b/w 6months and 7 years
#severely affected cases are manifested before 1 year of age
#males >females
#painless , slow expansion of affected areas of the jaw
#mandible(angle)is frequently involved
#involvement of the anterior maxillary segment produces the most characteristic deformity
#inverted V shape of the palate , due to maxillary expansion
#regional lymphadenopathy
#eyes appear to be turned upwards


GRADING OF VARIABLE CLINICAL PRESENTATIONS OF
CHERUBISM

1)GRADE 1 - bilateral involvement of ascending rami of mandible
2)GRADE 2 - bilateral maxillary tuberosity involvement along with ascending rami of the mandible
3)GRADE 3 - extensive involvement of anterior and posterior regions of the maxilla and mandible
4) GRADE4 - extensive involvement of anterior and posterior region of the maxilla and mandible
along with involvement of coronoid and condylar process


RADIOGRAPHIC FEATURES

#Well-dened multilocular radiolucencies
#expansion and thinning of cortical plates


HISTOPATHOLOGIC FEATURES

#characterised by the presence of cellular and vascular brous tissue containing multinucleate
giant cells


TREATMENT

#Curettage or de-bulking with recontouring

CENTRAL GAINT CELL
GRANULOMA


#nonneoplastic lesion
#formely known as gaint cell reparative granuloma


CLINICAL & RADIOGRAPHIC FEATURES

#Age around 2 to 80 years
#females >males
#70% in mandible
#more common in the anterior portion of the jaws

1) NONAGGRESSIVE LESIONS -make up most of the cases , slow growth , no cortical
perforation
2) AGGRESSIVE LESIONS -characterised by pain, rapid growth , cortical perforation

#radiograhically , central giant cell lesions appear as radiolucent defects (may be unilocular or
multilocular)


HISTOPATHOLOGIC FEATURES

#many irregularly disturbed multinucleate-d giant cells , in the background of spindle-shaped
mesenchymal cells


TREATMENT AND PROGNOSIS

#curettage
#patients with aggressive lessons three alternative to surgery are -
1)intralesional corticosteroids
2)exogenous calcitionin
3)interferon alpha-2

PAGETS DISEASE OF BONE
(OSTEITIS DEFORMANS)

#Characterised by abnormal and anarchic resorption and disposition of bone , resulting in
distortion and weakening of the affected bones
#causes of pagers disease are unknown , but inammatory , genetic and endocrine factors may
be contributing agents


CLINICAL FEATURES

#mostly older ppl
#men>women
#most common sites - lumbar vertebrae , pelvis , skull and femur
#generally leads to increase in the circumference of the head
#maxillary >>>>mandible (results in enlargement of middle third of the face)
#LION LIKE FACIAL (LEONTIASIS OSSEA)
#nasal obstruction , enlarged turbinates
#enlargement causes spacing of the teeth


RADIOGRAPHIC FEATURES

#Early stage of pagets reveals a decreased radiodensity of the bone and alternation of the
trabecular pattern
#large circumscribed areas of radiolucency
#PATCHY SCLEROTIC AREAS (COTTON-WOOL APPEARENCE)


HISTOPATHOLOGIC FEATURES

#apparent uncontrolled alternating resorption and formation of bone
#numerous osteoclasts surround bone trabecula-e
#JIGSAW PUZZLE OR MOSAIC appearance of the bone


DIAGNOSIS

#pt shows high elevations in serum alkaline phosphatase levels and normal blood calcium and
phosphorus levels along with elevated hydroxyproline levels
#N-telopeptides and pyridinoline cross-link assays are available


TREATMENT AND PROGNOSIS

#Use of parathyroid hormone antagonist (calcitonin and biphosphonates) can reduce bone
turnover
#newer biphosphonates such as , etidronate , pamidronate , alendronate , risedronate
#osteosarcoma (complication of pagets )