56
REVIEW ARTICLE BENIGN FIBRO‐OSSEOUS LESIONS OF JAWS‐ A REVIEW Rashi Bahl1, Sumeet Sandhu 2, Mohita Gupta3
1Reader,
Department
of
Oral
and
Maxillofacial Surgery, Genesis Institute Of
Dental Sciences & Research, Ferozepur,
Punjab, India
& Head, Department of Oral and
Maxillofacial Surgery, Sri Guru Ramdas
Institute of Dental Sciences & Research,
Amritsar, Punjab, India
Corresponding Author
Mohita Gupta (BDS)
Address: 302, Green Avenue,
Punjab. PIN 143001
Contact number: 08146182200
E-Mail: [email protected]
Amritsar,
2Prof.
Access this Article Online
www.idjsr.com
3Genesis
Institute of Dental Sciences and
Research, Ferozepur, Punjab, India
Use the QR Code scanner to
access this article online in
our database
Quick Response Code
Article Code: IDJSR 0030
Abstract
Benign Fibro-osseous Lesions is a group of lesions in which normal bone is replaced initially by
fibrous connective tissue and over a period of time, the lesion is infiltrated by osteoid and cementoid
tissue. This is a benign and idiopathic process. Fibro-osseous lesions of the maxillofacial bones
comprise a diverse group of pathologic conditions that include developmental lesions, reactive or
dysplastic diseases, and neoplasms. The concept of fibro-osseous lesions has evolved over the last
several decades and now includes two major entities: fibrous dysplasia and ossifying fibroma. The
less common lesions include florid osseous dysplasia, periapical dysplasia, focal sclerosing
osteomyelitis, proliferative periostitis of Garre, and osteitis deformans. It represents a diverse
group of pathological conditions that includes developmental lesions, reactive or dysplastic diseases,
and neoplasms. Owing to substantial overlap of the histopathologic findings, sub classification of
Benign Fibro-osseous Lesions may be problematic. Despi te the advances i n the
u nde r st and ing of the se co ndi tion s , fibr o-o sseo u s l es ion s c on tin ue t o p re sent
problems in classifi cation, di agno si s, an d ma nage men t due to mul ti ple hi sto logi cal
a nd r a d i o g r a p hi c si mil a ri tie s . The objective of this article is to review the most current clinic
pathologic, radiographic, and molecular studies of Benign Fibro-osseous Lesions to aid the surgical
pathologist in the recognition and diagnosis of this diverse group of maxillofacial lesions.
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Introduction
The fi bro -o s seou s les ion s rep re sen t a
l arge group of di so rde rs that may
have
c o m mo n
c ha ra c teri s ti c s
i ncl udi ng cli ni cal , r adiog rap hi c and
mi cro scopic fe at ure s. Alt hough mo st
a re of unk now n e tiolog y, some are
be lieved to be neo pl asti c an d ot hers
a re rela ted to met abo lic i mbal an ces.
I t is no t un u su al to s ee the se le sio ns
p re sen tin g
wi th
a
ran ge
of
radiographic
appearance s,
ca using
conside rable di agnosti c co nfusio n 1 .
Benign fibro-o sseo u s lesion i s a well k now n ,
de script ive
te rm
t h at
en co mp asse s
a
wi de
ran ge
of
c ond itio n s, t he diag nose s of w hi ch
may be challeng ing . In part, the
c ha llenge
a ri se s
be ca u se
the
h istop at hologi ca l appe aran ce s of all
fi bro-o sseous le sions are very simil ar,
if no t ide ntical , maki ng cl ini cal
d iag nosi s
dif fic ul t
ba sed
on
mi cro scopic
feature s
alone.
The
max illofaci al fibro-osseous le sions are
the le sio ns that are diffe rent (ex cept
fibrous dysplasia) to t ho se fou nd in
t he res t of t he skele ton .
Charles Waldron wrote “In absence of
goo d
cli nic al
a nd
rad iologi c
i nformation a pa thologi st can o nly
st ate th at a given biop sy is con si s ten t
Classification
Charles A Waldron i n 1985 classifie d
fi bro osseous le sions i nto main groups
on t he ba si s of cli ni cal beh avio r,
histopathology
and
radiographi c
fi ndi ng s :
wi th f ibr o os seo us le sion s . Wi th
adequate
clini cal
&
radiologi c
i nfor mation , mo st le sion s c an be
a s sig ned wi th rea son able cert ain ty
i nto one of the seve ral catego rie s 2
owi ng to the ir si mil ar hi stology.
Radiog raphically ,
fi bro-o sseo u s
le sio ns vary co nside rably from a
si mple radiolucent le sio n to mixed
radiolucent/ radiopaque or radio paque
le sio n . The se c an be well defi ned or
ill -defi ned
ble ndi ng
i mpe rceptibly
i nto the su r rou ndi ng bone . The re may
o r may no t b e e x pan si o n o f bone , wit h
o r wit ho ut di spl ace men t o f too th .
Histologi cally,
the
f ibro-o s seous
le sio ns
mai nly
consi s t
of
two
c o m p o ne nts - h a r d t i s sue a n d sof t
t is s ue compo nen t . The tre atmen t of
fi bro-o s seous le sions varie s depending
on the nature of the le sion. It may
v a r y f r o m s i mpl e s urgi cal excision o r
curettage
in
cemento
ossifyi ng
fi bro ma to a su rgical ex ci sion and
rese ction of the involve d jaw i n cases
of
juvenile
o s sif ying
fi bro ma,
o steo geni c
s ar co ma
and
c ho nd ro s a r co ma .
1 ) Fib rou s Dy sp la sia
2 ) R e a c tiv e
( dy sp l a st i c)
l e sio n s
a ri si ng in t he tooth bearing
a re a :
Pe ria pi c al
c e ment o
osseous dysplasia
Focal cemento o sseous dysplasia
Florid
ce mento
o sseous
d y s pl a si a
3 ) Fibro – o s seous neoplasms
Ce mentifying fibroma
Ossifying fibro ma
Ce men to o s sifyi ng fi bro ma
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It i s one of the mo st pe rplexing
d ise ase s o f o s seou s tis s ue s & h as been
de scribed a s a lesion of un kno wn
e tiology, uncertai n pathogenesi s and
d iverse
h istop at holog y.
It
is
a
conge nital,
metaboli c,
no n-fami lial
d i s tu rb an ce tha t p ro d uce s 2 .5% o f al l
bo ny tu mor s a nd o v e r 7 % o f a l l no n
mal ign an t t umo rs of bo ne 3 . I t i s a
be nign
fibro
o sseous
le sion
c ha ra c teriz e d by f o rma ti o n o f f i bro u s
c on nec tive t i ssue wi th in t he spon gio sa
of the affecte d bo ne and ofte n by the
painle ss ex pansio n of that bone to
c a u se
d e f o r mi t y 4 .
The re
is
the
repl ace men t
of
n o rmal
bony
archi tecture wi th fibrous and o s teoid
tissue 5 . It m ay al so con tai n i s l an d s o f
cal cifie d t issue , t he appe aran ce of
whi ch i s depe nde nt on the age of the
le sio n . The re i s p rolife ra tion o f
fi bro blast li ke cell s that have feature s
of o steoblasts in so me areas and tho se
of cho nd rob la st s i n o the rs . It o cc ur s
be cause of maturation a rre st of bone
f o r ma ti o n a t t he stage o f wo v e n / f i b re
bo ne 6 . The re sul tan t fib ro o s seou s
t is s ue i s poo rly fo rmed , el as ti c a nd
structurally inadequate . It can i mpai r
c osme ti c & st ru ct ur al fu nc tio n of bo ne
le ading
to
o steol yti c
le sio n s,
f ra ct ure s, & deforma tion s . I t may
i nvolve one or more bo nes of the
crani al o r extra cranial skeleton. It
has
two
basic
clinical
fo rms:
mono stoti c a nd polyo sto ti c 4 . It may
a l so be a s so cia te d wi th e ndo c ri ne
d ysfun c tion , ab nor ma l pig me nt at ion ,
a nd pre cocio us pu bert y in g irl s 7 .
It
wa s
fi rst
repo rte d
by
Von
Re ckli ng hausen
in
1891.
Fibrous
d yspla si a i s def ined a s a di sea se of
bo ne ,
cha ra c teri zed
by
lo cali zed
areas,
usually
in
a
unil ate ral
d istr ibu tion sho wing a ma tu ra tio n
a r rest of bo ne fo rma tion at the stage
of wo ven bone 8 .
E tiolo gy and Pathoge ne si s
d yspla si a pe rh ap s becau se of est roge n
receptors in the fibrous tissue 3 .
FIBROUS DYSPLASIA
Fib rou s dy sp la sia is po stulat ed to
o cc ur a s a r e su l t o f a l a c k o f s t re s s
alignme nt
and
insuffi cient
mine rali zation re sults i n substantial
lo ss of me chani cal strength, le ading
t o the develo pme nt of p ain , de fo rmity ,
and pathologi c f racture s 6 .Ma rie e t al
s ho we d t ha t a n ac tiv a ti ng mutation of
G s α in o steoblasti c cell s of patients
wi th McCune-Al bri ght sy nd rome and
mono s toti c
d i sea se
lead s
to
c o n st i t utiv e a ct i v a tio n of adenylate
c y c l a se , i n c re a sed c e l l p ro l i f e ra ti o n ,
a nd i n appr o p ria te cell dif fere ntiation,
resul ting i n overproduction of a
d i so rg ani ze d f i b ro t i c b o ne m at ri x i n
po lyo sto tic an d mono stoti c fi bro us
d y s pl a si a 6 , 3 .
Preg na nc y
ha s
bee n
i mp lic ate d in ex ace rb atio n of fi bro us
Cl ini c al Fe ature s
I t oc cu r s mo st co mmonl y i n se cond o r
t hi rd de cade of li fe 9 . The ave ra ge age
of o cc u rren ce i s t en ye ar s. S ome
st udie s
r evea led
no
ge nder
1
0
predilection .M ale to female ra tio in
s o me st u d i e s i s 2 :1 . So me st u d i e s a l so
sho w that sex predile ctio n i s almo st
equal . A mong the jaw bone s, max illa
i s mo re co mmonl y affected than the
mandi ble.
The mo st common sign is painless
ex pan sio n o f the affe cte d area a nd
de formi ty of the affected si te . The
fo ra mi na of cr ani al ne rve s , may be
encroached upon p rod uc ing ne rve
p a l si e s , t h e d i s figure ment may be
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59
extre me
justifyi ng
the
term
“ leontiasis ossea” . Diff u se polyo sto ti c
le sio ns i n l arge weigh t- bea ring bone s
a re
p rone
to
lea d
to
bo wing
de formi ties that increase wi th age and
skele tal g rowth. Unlike defo rmities in
p atie nt s wi th mono stoti c d ise ase ,
wi th
de formi ties
in
patie nts
po lyo sto tic di sea se may con tin ue to
progre ss af ter skeletal ma tu ri ty 1 0 .
Oral Manife stations
P ai n o r p ar ae st hesi a i s a n u nu s ual
complaint. Di splacement of the teeth
wi th
resultant
ma locclusion
and
i nte rfe renc e wi th no r mal er up tion
p at ter n s may o c cu r . I n chil dren tee th
i n t he affec te d pa rt may fail to e ru pt.
De nti nal dy sp la sia i s a di sorder t hat
o cc ur s in pa tie nt s wi th inhe ri ted
fibrous
d y s p l a si a .
Patho logically,
fi bro u s ti ssue th at is fi r m, rub ber y,
a nd g ri tty 7 .
Histologi cally,
f ib rou s
dy sp la sia
consi sts of varying amounts o f s pi ndle
cell
bu nd les
a nd
t rabe cul ae
of
i mma tu re wove n bone . The fi bro us
tissue in fibrous dysplasi a i s well
v as cu la ri ze d a nd o f ten sho w nu me rou s
small vessels in the centre and large
pe ri phe ral
si n u so i ds 7 .
Th ree
si te
specific patterns of hi stop at holog y
have been i dentified. C h ine se wri ting
t ype ; scle roti c/p aget oid type; a n d
sc lero ti c / h ype rcel lul ar type.
a s a spe c tr u m of fou r p at ter ns in a
p ano ra mi c r ad i o g r ap h : g ro u nd g l a ss
( condensed/granul ar) ,
radiol uce nt
(l yti c) , mixe d radioluce nt/radiopaque
( mixe d
den si ty)
a nd
radiop aq ue
( s clero ti c) . Vari atio ns in the co rti cal
t hi ck ne ss
a re
ca u sed
by
slo w
r e so rp tio n o f t he en do ste al su rf ace ,
c ommo nly refe r red to a s "en do ste al
scalloping ." The per io stea l su rf ace
remains smooth 1 0 .
T h ree v arie ti e s o f a ppea ra n ce s a re
seen o n C T scan: gro und glass
pattern,
ho moge neously
dense
p at te r n , an d cy s ti c v arie ty 6 . Magnetic
r eso na nce i magin g i n a ddi tio n c an
he lp di sti nguish f i bro u s d y s p l a si a
f ro m
men ingio ma ,
os teo ma ,
or
mu co cele an d def ine t he extent of sof t
tissue invol veme nt, parti cularly if
c e n tr a l ner v o u s sy ste m st r uc tur es a re
i mp inge d on 7 .
Single pho ton e mi s sion computed
tomo graphy has been repo rted to be
more sensitive i n dete cti ng the are as
i nvolved
in
case s
of
f ibrous
6
d y s pl a si a . A slight elevatio n of serum
a l kal i ne pho s pha ta se ma y be s e e n i n
so me ca ses bu t may no t alwa ys be
rai sed.
Calci u m,
p ho sp ha te
and
vario u s othe r hormone s are seen in
no r mal ran ge 4 .
R adio graph i c Pict ure
The le sions o f fi bro u s d y s p l a s i a a r e
usuall y poo rly circumscribe d , wi th the
le sio ns de monstrating a blending
m a rgi n an d a re r ad i o pa que ( g rou nd
gl a ss
appe aran ce)
a lt hough
ea rly
le sio ns ma y be l arge l y r adio l uce nt.
A cco rdi ng t o Aki nto ye , i t ca n prese nt
I) Polyostotic Fibrous Dysplasia
Invol vement of two o r more bone s i s
calle d
as
polyo sto ti c
fi bro us
d y s pl a si a . T wo app are nt type s o f
po lyo sto tic
f ib rou s
d y spl a sia
a re
de scribed :
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a ) J a f f e – L i ch te n stei n sy nd rom e
b ) M c C u ne –Alb ri ght syn d ro me
I t mo st com m o nl y o cc u r s i n c hi l d ho o d .
Medi an age of o n set of sy mptoms i s 81 0 y e a r s , wi th mo s t o cc ur ri ng be f o re
the
age
of
ten 1 1 .
The
dise ase
a ppa re ntly h as a di st in ct tenden cy to
o cc ur i n w o me n w i t h a m a l e : f e mal e
r a tio o f 1 :3 1 1 . Lo ng bo ne s of ex tre mi ty
are mo st of ten affecte d in follo wing
o rde r of fre que ncy : fe mu r , ti bia ,
h u me r u s & r adi u s . N e x t i n o rde r o f
f req uen cy a re bone s of t he sk ull
( crani al vau lt & jaw bo ne s) 1 1 .
Cl ini c al Pre se ntati on
A l imp , pa in i n leg a nd fr ac tu re i s
the initi al sy mp tom. It pursue s a
p ro tr a c te d
c l i ni ca l
c o u r se
c ha ra c teriz e d by pai n , def o r mi t y & a
t e nde nc y t o p at ho l o g i cal f ra ctu re o f
t he af fect ed bo nes 1 1 . Le g length
d isc repa ncy i s ve ry commo n a s a
resul t o f i nvolve ment of the uppe r
po rtion of the femu r 1 2 . Fre que nt ly
i dentified de for mi tie s i nc lude cox a
v ar a, the s he phe rd ' s c rook defo rmity ,
bo wing of the ti bia etc 1 0 . The o bje ctive
fe ature s seen i n roentge nograms o f
bo ne s affecte d by polyo stoti c fi bro us
d yspla si a
in cl ude :
b roa deni ng
or
ex pan sio n of bone , th in ning o f c or tex,
c ha ra c teristi c ra refied & a ppa ren tly
t r abe cula ted ap pearan ce , second ary
de formi ties
of
affe cte d
bo ne s 1 1 .
Pre mature
se cretion
of
pituitary folli cle stimul ating ho rmone
has
been
r e p o r te d
as
well
as
mode ra tely ele vate d b a sal me tabol ic
rate. Most surgical ti s sue i s obt ai ned
b y c u ret ta ge . The s pe ci me n h a s a
distinct gritty feeling refle c ti ng the
o steoi d
le sio n.
trabeculae
i nhe rent
in
the
T he typ i ca l mi c ro sc o p i c f i n d i ng s o f
fi bro u s dy sp la sia s ho w i r reg ula rly
shaped trabeculae of i mma ture bo ne
i n a ce llular, loo sely arranged fi bro u s
s t ro ma . The bo ny tr abe cu lae a re not
c on nec ted to e ac h ot he r 1 2 . Stellate
o steo blasts are seen parti cularly in
a c tive le sio ns an d a ppea r t o arise
f ro m fib rob la st s .
II) Monostotic Fibrous Dysplasia
It
is
more
common
than
the
po lyo sto tic type . It mo st commonl y
o ccurs a t t he age of 20 to 30 yea r s
with some case s be co min g do rma nt by
t he
t hi rd
de ca de
a nd
ho rmo nal
c ha nge s
l ike
in
p re gna nc y
r e a ctiv at i n g a dor ma nt l e sio n 7 . It can
a lso o c cu r i n inf anc y 5 , o cc u rs wi th
appare ntly
equal
predilection
for
male s
and
fe male s.
Ri bs
and
craniof acia l bo ne s are mo st commonl y
affected 7 .
O t her
b one s
a ffe cted
i ncl ude , cla vicle , t ibia , fe mu r et c. The
p atie nt m a y be a sy mp to ma ti c a nd
le sio n
dis co vere d
in cide nta lly
or
p atie nt ma y pre se nt wit h a p ainle s s
swell ing caused by a slow growi ng
le sio n causing expansio n of the jaw
a nd p ro du ci ng a no n ten de r f aci al
a sy m me try 1 3 . In children the teeth
may f ail to erupt 1 3 . Fibrous dysplasia
of the maxill a is an e spe cial ly serious
fo r m of the di sea se si nce it h a s a
m a rke d p re d i l e c ti o n f o r o cc ur re n ce i n
chil dre n . Seve re malocclusion and
bulgi ng of the canine fo ssa or extre me
p ro mine n ce of the zygo mati c p ro ces s ,
producing a marked f aci al deformity,
a re typ ic al se qul ae of t his di sea se in
max illa.
Seru m
al kali ne
pho s pha ta se
an d
u ri na ry h y d ro x y po l i ne are examples of
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u sef ul ma rke r s and a re u sed to
moni to r respo nse i n t he no n su rgi ca l
t re at men t of di sea se ra the r th an fo r
d i ag nosi s 1 3 . A ground glass or o range
pee l appearance i s see n when there
are
areas
of
co ndensatio n
i nte r spe rsed
w ith
are as
of
radiolucency.
The
le sio n
causes
reso rptio n
of
roots
of
erupted
t e e t h 1 3 .I t m ay sho w fo cu s o f g rit ty
t is s ue in the bone 5 .
The t ra becul ae may be devoi d of
o steo blastic
ri mming ,
the reby
a ppea ri ng t o be f o r me d by f i b ro b l a sti c
o s seous me tapl asia 1 3 .
S y nd ro me s A sso cia te d wi th Fi bro u s
D y spl a sia
M cCune-Albrig ht
syndro me
is
an
endocri nopathy o c curri ng mainly i n
gi rl s, co nsi sti ng of t he t ria d of
p re co ci o u s p u ber ty , po l y o sto tic fi bro u s
d yspla si a
a nd
ch ara c teri stic
c u taneo u s
p ig men ta tion .
Th e
c u taneo u s le sion s ar e fla t pig men ted
macule s, of ten re ferred to as "café au
l ait " spot s Ma za braud sy nd rome i s the
r a re combin at ion of fibrous dysplasia
and soft- ti ssue myxo ma s. There are
three
mode s
of
treatment
i .e .
ob se rva tion ,
medi ca l
the rap y
&
s u rgi cal t re at me n t . C o r ti so ne ha s
bee n re por te d to p rod uce so me relief
i n pai n of bo ne le sion s. Impo rtant line
of
me di cal
treatment
is
with
b ispho sp hon ate s
w hi ch
i nh ibit
o steo cla stic ac tivi ty 6 . Young patients
r e cei v i ng
p a mid ro n ate
s ho u l d
be
moni to red w it h se ria l radio gra ph s to
che ck fo r a transie nt mi ne ralization
de fect , wh ich pre sen t s a s in crea sed
g ro w th pla te t hi ck ne s s , t hi c ken i ng o f
corti ces
and/o r
o ssifi cation
of
radiolucent are a s 1 0 .
Surgical Treatment
A cco rdi ng
to
El
Dee b
M,
t he
t re at men t
of
choi ce
is
su rgi ca l ,
de pending upo n the si ze of the l esion
a s a s cer ta i ned by t he r adio g ra phi c
p i c tu re
an d
by
b i o p sy .
In
the
o steol yti c t ype radi cal cu re tt age i s
i ndi ca ted ,
w he re a s
in
t he
m o re
ma tu re , soli d type su rgi cal shav ing
a nd re con tou ri ng i s in di ca ted . Fibrous
d y s pl a si a i s t re ate d b y cu ret tage an d
p ac ki n g wi th ca ncello us chip g raf ts,
by
subpe rio steal
excision
and
c an ce l l o u s
b o ne
g r aft ,
by
extrape rioste al
exci sion
and
can cello u s bone g raf t, co rt ica l graf t o r
bo th. In ma xillof aci al are a , a co mmon
pro cedure i s to del ay surge ry until
bo ne g rowth cea se s an d to con tou r the
b ulge d por tio n o f the bo ne fo r an
e st he ti c ap pea ra nce . I n c ase o f v i su al
d i s tu rb an ce cau se d by co mp ress i o n o f
op ti c ne rve , i mmed iate s urge ry i s
nee ded 5 . A cco rdi ng t o Edge rt on the
su rgi cal tec h niq ues used a re :
1 ) Si mple bo ne co ntou ri ng
2) Rese ction and acryli c i mpl ant
3)
Re sectio n,
replantation
re model ing
and
Re cu rre n ce
of
fibrous
dysplasia
follo win g cu re tt age i s mo re common in
c hi l d re n th an i n a du lt s . Thi s “ re move ,
resha pe , an d repl ant ” te chn ique h as
excelle nt
bone
heali ng,
good
po s tope ra tive
co ntou r s ,
and
no
c lin ic al evi den ce of re cu rre nce of bone
enlarge ment.
Mal ignant
t r an sfo rma tion of fi bro u s dy sp la sia
o cc ur s
v e ry
i nf re q uen tl y ,
w i th
r epo rte d p rev alen ce’ s ra ngi ng f rom
0 .4% to 4% wi th ave rage incide nce
be ing
1% 1 0 .
The
mo st
commo n
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mali gna nt t u mo rs we re o steo sa r coma ,
fi bro sarcoma, a nd chon dro sar coma .
Diffferential Diagnosis
O the r e ntit ies wh ic h ma y be con fu sed
wi th fi brou s dy splasia are ossifying
fi bro ma, c eme nto o s seu s dys pl as ia ,
Page t s
di sea se ,
c eme nto ma ,
c he ru bi sm ,
h y per pa ra th y roi di s m ,
c h roni c
sc lero si ng
o steo myeli ti s,
o steo geni c s a r coma e tc . Le sion s th at
may suggest fibrous dysplasi a incl ude
simple
bone
cy sts,
nono ssifyi ng
fi bro mas,
o steo fib rou s
dy sp la sia ,
a da ma nti no ma ,
low-grade
i nt ra med ull ary
o steo sa rco ma ,
and
P a g e t d i sea se 1 0 .
CEMENTO-OSSEOUS
DYSPLASIA (COD)
Ce mento-osseous dysplasia i s the
mo st co mmon f ibro-o sseous le sion
o ccurri ng i n the tooth be ari ng areas
of the jaws. COD i s a group of no n
neo plastic proce sse s usually confine d
t o the too th be ar ing a rea s of the ja ws
o r e den tulo us alveol ar p ro ce s ses 1 4 .
Ma ny te rms h ave been u se d to refe r to
ceme nt-osseus dyspla si a : Peri api cal
ceme nt-osseous
dysplasia
(PCD),
Flo rid o sseus dy splasia ( FOD) , Flori d
c eme nto osseu s dy sp la sia ( FLCOD ) ,
Focal
cemento
osseus
dysplasia
( FCO D) .
Ro binso n a tt ri bu te d the ca u se as
i n ju red bone rea ct s in an a bno rmal
w ay to low- gr ade o r c h roni c in ju ry by
reso rb ing fo rmed bone trabe cul ae and
r epl aci ng i t wit h c ell ula r f ib rou s
connective tissue , in which i mmature
bo ne an d a ceme nt um-li ke subst an ce
a re de posit ed .
Pe ria pi cal
Ce me nto –O s seo u s
D y spl a sia a l so k no wn as cementoma ,
osseous
dysplasia
a nd
periapical
cemental
dysplasia.
The
first
compre hensive clini cal, radiographi c ,
a nd
hi st opa tholo gi c
st udy
w as
r e po rte d by Sta f ne i n 193 3 . B l um i n
1 930 and Tho ma in 193 7a nd 194 4
de fine d i ts h i sto pa tholog y . PCD i s not
a tr ue ne o p l a s m b ut a dys pl as ti c
conditio n in whi ch multi ple fo cal
a rea s of bo ne an d ma rrow are
repl ace d by cellular connective tissue
le sio ns wi th li mi ted g row th po ten tia l.
The le sion attains a f ixed si ze and
l ate r un der goe s a ma tu ra tion p ro cess
t ha t c ul mi na tes in t he for mat ion of
mul ti ple dense cal cifie d intrao sseous
no dule s 1 2 . P C D i s a n a sy m pt o m ati c
le sio n of ten di scove red o n r ou tine
radiographi c examination .Multi ple
le sio ns a re ofte n pre sen t . B u cc al a nd
l i ngu al e x p an si o n o f the co rti ce s i s
of ten absent.
The age of occurre nce has bee n
v ari ably repo rted by va rio us au tho r s
f ro m 3 r d to 5 t h de ca de wi th a ra nge of
1 4-8 2 ye ars and mea n of 42 .5 yea r s
wi th ca se s r a rely o cc u rr ing befo re 20
ye ars of age 1 2 . Mandible ( 6 8 .15 %) 5 i s
more
commonl y
affe cted
than
1
4
max illa .The
lesio n
princi pally
i nvolve s the api cal a rea of one o r
more
vital
mandibul ar
teeth,
p ar ti cul arly t he i nci so rs . F emale to
male ratio has been vari ably repo rted
be twee n10:1 to 14 :1 . Mo st le sion s a re
l e s s t han 0 .5 c m i n si ze . M a x i mu m
si ze ra rely excee d s 1.5 cm. Pe ri api cal
ceme nto -osseous dysplasia has bee n
c la s si cally de sc ri bed a s p rog re ssing
t h roug h 3 r a d i o g r a p hi c sta g e s .
1) Osteoloytic stage
2) Ceme ntoblasti c stage .
3 ) The thi rd o r mat u re stage
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Differential diagnosis
R adio lu cen t s tage
¾
Apical periodo ntal
o r a rad i c ul a r cy st
¾
Pr imo rd ial odo ntoge ni c c ys t
¾
Ea rly
ph a se
fi bro ma
¾
Chro nic o steomyelitis( if 4 to 6
a nte rio r tee th a re i nvolve d)
of
g r anuloma
o s sif yin g
Mi xed stage an d radio paq ue stage
¾
O do n to ma
¾
Chro nic o steomyelitis
¾
Ossify ing fi bro ma
¾
Osteoblasto ma
Treatment
Only pe riodic obse rvation i s necessary
d uring whi ch o ne wou ld ex pect t o see
t he radiog ra phi c ch ange s a ssociate d
wi th mat ur a tion of the lesion .
Focal Cemento-Osseous
Dysplasia
T hi s
c on di tion
d e rive s
histoge neti call y f rom ele me nts of the
pe rio don tal
l igamen t .
O ther
e tiologi cal theo rie s consi der it to be a
rea ctive le si o n an d i t i s mo re co mmon
i n wo men 2 .
Cl ini c al Fe ature s
FCOD
is
al mo st
in va riab ly
an
asympto mati c di scove red le sion o n a
radiographic examination an d o c cu rs
i n per iapi ca l are as of tee th wi th v ita l
p ulp s o r i n r egio ns o f ex tr ac tio ns . The
conditio n rarely pro duce s expansion o f
t he bone . La rge r le sio ns may ho weve r
c au se sl igh t jaw enl arge me nt . FCOD
to 6th
u s ual l y o cc u r s b e twe e n 3 r d
de ca de s, fe ma le: male ra tio bei ng
Mandible
is
more
8 : 1to
1 0: 1 .
commo nly the site of occurrence wi th
around 77% of le sions bei ng in
mandi ble ; particularly too th be ari ng
areas of po ste rio r ma ndi ble , and 1123% o f lesions occurri ng in max illa.
The si ze ra nge va ries fro m 0 .2 -1 1 c m
wi th ave rage of 1 .8 cm 2 . Re gardless of
st age , a n impo rt an t d iag nosti c fe ature
i s it s clo se a sso ci atio n wit h t he
pe ri apex or previous ex tr ac tion si te .
Focal ce mento-o s seous dyspl asia te nds
t o be well de marca ted wit h or wi tho ut
c or ti cat ion. The re is no bowi ng of
i nfer ior ma ndi bul ar bo rde r .
A t t he time of s u rgi cal explo ra tion ,
t he su rgeon usually fi nd s gr it ty
he mo r rha gi c mate ria l. The se gro s s
fi ndi ng s con t ra st sha rp ly wi th t ho se
of ce me nto-o s sifyi ng fibro mas, which
s h are m an y f e a tu re s hi sto l o g i cal l y .
The
la tte r
neopla sms
ten d
to
enucleate i n o ne piece and are ofte n
w hite , gl ist eni ng an d ho moge neou s on
cut surface . Radiology was of central
i mpo r tan ce to the det ec tion of a t lea st
64%
of
f o cal
c e me nt - o s se o u s
dysplasi as
fo und
i n cide ntally
to
radiography 1 5 .
On
the
basi s
of
histopathologic study 3 progressi ve
stage s can be i dentified: The early
(o steo lytic) , the inte rme diary ( fibroo s seous) , the late (Osteoscle rotic) .
Be cau se fo cal ce men to-o s seous
dysplasi a ge nerally ex hibi ts l ittle or
no ten denc y to e nla rge eve n a fte r
partial removal of the le sion, these
l e sio ns do no t requ i re a ny t re a tme n t .
Florid Cemento-Osseous
Dysplasia
Ce mento-osseous
dysplasia
has
a
pattern of expre ssion that i s often
mul tifo cal a n d co mmonl y a ffe ct s all
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64
quadrants
of
the
max illa
and
mandi ble . Thi s multifocal e xpre s sion
i s kno wn as f lori d ce mento-o s seous
dysplasi a. It is cl inically the mo st
extensive fo rm of ce mento-o s seous
d y s pl a si a a nd he nce t he ter m f l o ri d.
Mel ro se
et
al
initi ally
repo rte d
FLCOD as florid osseous dysplasia.
T he di sea se a ppea r s t o ha v e a f a mi l i al
d is tr ibu tion ; i t i s more co mmon in
wo me n 2 . The di sor de r i s s t ri ctl y
lo cali ze d to the too th bearing areas
a nd not a s so cia te d wi th any o the r
skeletal deformity. When the le sio ns
a re la rge , ja w expa n sion ma y be
no ted , p ar ti cu l a rl y o f t he m a ndi bl e
le ading sometime s to facial defo rmity,
sy mp to ms
such
as
du ll
p ain,
discharging sin use s o r seq ue str a tion s .
O c ca sio n al l y , pat i e nt s wit ho ut si g n s
of
infe ction
compl ain
of
an
i nte rmittent, dull , achi ng sensation in
the ma ndibular molar area. All Tee th
h av e no rma l spo nt ane o u s p ai n a nd a re
vi tal . It is see n mo re co mmonly in
fe ma les 2 . They ha ve st ri king ten den cy
t owa rd s
b ila tera l,
often
quite
sy mmet ri ca l, lo cat ion , a nd i t i s not
u nu s ual to f i n d e x ten si v e l e sio ns i n
a ll fou r qu ad ra nt s, p arti cul arly t he
po sterio r
region
( mol ar-pre molar
region) . The y affe ct onl y the alveolar
p ro cesse s a nd see m t o be in depe nde nt
of tee th. Le sio ns have been f ound
more c ommonl y i n ma nd ible an d
s o meti me s i n t he maxil la .
Radiog raphically , a wi de spe c trum is
seen. R adiographs usuall y di splay
d i f f u se
d i s tr i bu tio n
of
l o b ul a r ,
i rre gul arl y
sh ape d
r a diopa ci ties
t h roug hout t he alveol ar p rocess. The
lo bular densi tie s are of ten enme shed
i n poo rly de fine d area s of decrea sed
radiodensity, of ten havi ng a groundg l a ss a ppea ra nce . The l e sio n s a ppea r
as multiple sclero ti c masse s, lo cated
i n two o r more quadrants usually in
t he too th be ari ng a rea s . Bio psy i s no t
ne ce ssa ry .
Manage ment of FLCOD i s of ten
d iffi cul t an d not ve ry sat isfa cto ry . In
t he
a sy mp to ma tic
p atie nt ,
it
is
p rob ably wi se to kee p the p a tien t
u nde r o b se rva ti o n wi tho ut su rgi ca l
i nte rvention be cause the radiologi c
fe at ure s are diag no st ic. Ma nage men t
of the sympto matic patient is mo re
d iffi cul t .
Se que st r atio n
of
t he
c eme nt u m-li ke
ma ss e s
wil l
o cc ur
slowl y and he ali ng will follow thi s.
S au ce ri za tio n o r su rgi cal ex ci sio n of
t he scle ro tic ma sse s is of ten not
s u c ces sf ul a nd may ma ke ma tte r s
wo rse 1 2 .
D iffe re ntia l Di ag no si s
The se
in cl ude
c h roni c
d iffu se
sclero si ng
o s teomyeli ti s,
Page t’s
dise ase of bo ne , the o steomas of
G ar dne r’ s
sy nd rome ,
G iga nt ifor m
c eme nto ma , o s teogene si s i mpe rfe ct a
a nd pol y o st o ti c f i b ro u s dy spl a si a.
Malig nant Po tential
Deve lopment of ma lig nant spindle cell
t u mor ha s b e e n rep o r te d i n a p a tie nt
wi th FLCO D b ut i t is a r are
o cc ur re n ce.
Hereditary
Cemnto
Dysplasia/Gigantiform
Cementoma
Osseous
In 1953, Ag azzi & Bello ni repo rted a
conditio n that was clini cally and
r ad iogr ap hi call y
simil ar
to
flor id
ceme nto -osseous dysplasia but was
i nhe rite d a s an au to so mal domin ant
trait.
They
proposed
the
name
Gi g an ti f o rm
c e men to ma .
This
c ond itio n
is
r are .
The
g nat hi c
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enlarge ment i n mo st patients resul ts
i n sig nifi ca nt fa cia l de for mi ty , a s well
as
i mpactio n,
ma lpo sition
and
malo cclusion
of
the
i nvolved
de nt itio n . I f not trea ted the o sseo u s
enlarge ment eventually cease s during
t he 5 t h de ca de 1 2 . Usual ly develop
d ur i ng 1 s t de ca de of l ife a nd by
adole sce nt typi cal obvio u s le sions are
no ted a nd followe d by a rap id an d
expansi ve
growth
p at te rn .
It
de mo nstrates mul tifo cal invo lve ment
of both ma xilla and mandi ble 1 2 . The
i niti al fe atu re s re se mb le t ho se seen in
ceme nto -osseous dysplasia, appe aring
as mul tiple radiol ucencie s, in the
pe ri api cal region s. Wi th pro gressio n ,
the affe cted site s expand to repl ace
mu ch of the nor ma l bone w ith in t he
i nv o l v e d q ua dr an t a nd dev e l o p a
m i x e d rad i o l u ce n t a n d rad i o pa que
p at ter n . Wi th f ur the r ma tu ra tion , the
le sio n
be co me s
p redomin an tly
radioopaque but of te n maintain a thin
radiolucent ri m. Extensive resectio n
of t he altered bone an d re con stru ctio n
of the faci al skeleton and asso ciated
soft
ti ssue
is
re comme nded
ca n
produce acceptable f unctional and
ae sthe tic re sul t 1 2 .
D iffe re ntia l Di ag no si s
O steiti s defo r man s o r Page t’ s d ise a se
of
bone ,
ch ron ic
scle rosing
o steo mye lit is ,
S cele roti c
cemen tal
ma sse s, ch ro nic p rod uc tive o stei ti s ,
osseous dysplasia, mu lti ple enosto se s.
OSSIFYING FIBROMA (OF)
O s sify ing
fib ro ma
is
a
be nign
odo nto genic t umo r of me sen ch y mal
o rigi n . O F beh ave s li ke a be nig n bone
neo pla sm 1 6 . The t u mo r is de mar c ated
and occasionally encapsul ated le sion
c on si st ing of fi bro us t issue co nta ini ng
v ari able
a mo un ts
of
mine ra li zed
m a ter i a l
r e se mbl i ng
b o ne
a nd /o r
c e me nt u m .
M o n tg o mer y w a s f i r st to c o i n the te r m
“ o s si f y i ng f i bro m a
t i s s ue wit hi n w hi ch t he bone i s
fo rmed 1 7 .
I t accou nts fo r on ly 0 .1% of t he bo ny
l e sio ns . Os s i f y i ng f i b ro ma be l o n g s to
t he poo rly def ined g roup of fi broosseous lesions involving the jaws and
craniof acia l bone s t ha t resul t in
repl ace ment of the bo ne by fibro us
tissue
and
subsequent
1
8
,
1
9
mine rali zation
. The c au se o f the
o s sifyi ng fi bro ma re mai n s un kno wn .
OF usual ly o ccurs be tween the 3 r d a nd
4 t h de ca de of life with the a vera ge age
be ing 30 yrs 1 2 .M arked predile ctio n fo r
o ccurrence is repo rted to be seen in
fe ma les wi th fe male to male r atio
v ary i ng f ro m 1 .5 6 :1 t o 5 :1 .
Goaz and White repo rted that when
OF occurs in the ma xilla, i t i s most
commo nly lo cated i n the canine fo ssae
and zy gomati c arch. It may g row to
comple tely fil l the ma xillary sinus. It
can effe ct bo th maxill a and mandible
but the prefe rre d si te of occurrence is
r epo rte d to be mandi ble va ryin g fro m
70%- 89% of cases and max illa i n 11%26% with af fini ty fo r premolar &
molar area. The max illary le sio n s
we re fo und to be more ag gre ssive .
O s sif y i ng F i b ro mas a re a ss o c i ate d
wi th
a
slowly
progressi ng
enlarge ment of the affected bo ne .
Le sion i s asymptomati c until the
growth produce s a no table swel ling
and
mild
deformi ty
an d
fa ci al
a sy mme try. Di sp la cemen t of tee th i s
a n ea rly cli ni cal fe at u re . When ra pid
g row th doe s oc c ur , the sy mptoms a re
r ela ted to t he le sion si te a nd may
i ncl ude
painle ss
chee k
swe lling ,
u nil ate ral p rop to sis, di plopia and
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epi s tax is. De at h i s a ra re o ccu r ren ce
s e co nd ary t o i n t ra cr an i al e x ten si o n .
The se le sion s may o cc as iona lly h ave
ill -defi ned border, if rel ative ly rapid
g row th o c cu r s . A s the le sio n ma tu re s ,
m i x e d rad i o l u ce n t a n d rad i o pa que
a ppea ra n ce
may
be
se e n .
The
c ha ra c teris ti c f e a tu re s o f O F i n
r a d i o g r a p hs a re e x p a n si o n a nd l e sio n
margi nation,
demarcation,
or
.
c o r ti ca t i o n
Co rti cal
e xpansion
is
pre sent, of ten with an eg gshell- thi n
c o rte x . La rge o s sifyi ng fib roma s of
ma ndi ble
ofte n
de monst ra te
a
c ha ra c teris ti c d o w nw a rd b o wi ng o f
i nfe rio r cor te x of man di ble .
O n s u rgi ca l e xplo rat ion , the tu mo r i s
fo und to be re lat ively hy pova scul ar
and
well
de marcated
f rom
the
su r roun din g
ti ssue ,
permi tti ng
r ela tively e asy sepa ra tion f ro m the
surrounding bone . Some lesions will
h ave
a
def ini te
c ap sule.
Thi s
de mar ca tion f ro m t he s u rr ou nding
t is s ue i s a n i mpor t an t featu re i n
distinguishing OF fro m FD .
The
va sc ul ar
sp ace s
re se mble
arteriole s o r capillarie s di spl ayi ng a
c on tin uou s e ndo theli al l ayer w it h
p l u mp e ndo the l i al c e l l s p ro tr ud i ng
i nto the capillary lumen .The calcif ied
c ompone nt c on si s t s of ro unde d o r
lo bulated basophili c ceme ntum-li ke
masse s, trabeculae of o steoid or bo ne
o r co mbi na tion s of bo th , t he m a jo ri ty
of
bony
t r abeculae
in
ce men too s sifyi ng fi bro ma are thi n , single , and
sep ar ate wi th o steo bla stic ri mmi ng .
Tre at me nt
of
o ss ifyi ng
fi bro ma
i nvolve s the co mple te re mov al of
le sio n by cu ret tage, en ucleatio n , o r
exci sion. Co mple te exci sio n of the
t u mor ha s be co me a ne ce ssit y sin ce it
i s no to rious fo r re cu rr en ce 2 0 .
Juvenile Ossifying Fibroma
Juvenile
( agg ressive)
o s sifyi ng
fi bro ma was used in 2 n d edition of
WHO classifi cation of odontogeni c
t u mor of ch ild ren to de sc ri be a le sio n
a ffec tin g the jaw s unde r the a ge of 15
ye ars.
De fin it ion
The second edi tion of the WHO
classifi cation of o dontoge nic tumo rs
de fine s juve nile ( agg re ssive) ossifyi ng
fi bro ma as a n actively g rowi ng le sion
c on si st ing of cell ric h fib ro us ti ssue
c on tai ning b a nd s of c ell ula r o steoi d
wi tho ut o st eobla sti c r i mming toge the r
wi th tr abec ul ae o f mo re ty pic al bo ne .
Gi an t ce ll s may al so be pre se nt.
Cl assifi catio n
I t i s the t er m use d t o de s cribe two
d istin c t hi sto pa thologi c va ri an t s of
o s sifyi ng fi bro ma of t he c ra niof aci al
skele ton –
¾ psammo matoid
juvenile
o s sifyi ng fi bro ma
¾ t r abe cula r ju veni le o s sifyi ng
fi bro ma 1 6 .
J uv e n i l e
a ct i v e
o s sif y i ng
f i bro ma
a ffec t s p redo mi na ntly pa tie nt s i n t he
fi r st two de cade s of life , t he mea n age
of o cc u rren ce be ing 3 to 2 3 yr s 1 6 .
No si gnif icant sexual pre dilectio n i s
seen i n any of the two fo rms 1 2 .
Psammo ma toid
juve nile
ossifying
fi bro ma o ccurs overwhel mingly i n the
sino na sa l a nd o rbit al bo ne s of the
skull, whereas trabe cular juvenile
o s sifyi ng fi bro ma i s p redo mi nan tly a
g na thi c l e s i o n a f f e c ti ng the j a w s , w i t h
a predilectio n fo r maxilla. In the
mandi ble , the tumor occurs more
commo nly in the ramus than i n the
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67
bo dy of ma ndi ble 1 2 . The JOF is ofte n
cha ra c terized by a p rog re ssive and
so meti me s rapid expansio n of the
affected area. T he cli ni cal di ag nos ti c
c ha ra c teris ti c s s ug g e sti v e o f J OF a re
t he pa tie n t s a g e , r ap i d i nc re a se i n
le sio n si ze an d ab se nce of pa in ,
p are s the sia a nd b ru i t .
When the
o rbi tal bone s and pa ra na sal sin u se s
a re
i nvolve d ,
t he
p at ien ts
ma y
de velop propto si s, exophthal mos, and
bulbar
displace ment 1 2 , 1 6
Rarely ,
i nt ra cr ania l e x ten si o n ha s re su l te d i n
meni ngi ti s 1 2 , 1 6 . The le sio n ex hi bit s a
p ri ma ry
r ad i o l u ce nt
q uali ty
with
varying
amounts
of
inte rnal
radiopaci ty
,refle cting
degree
of
mine rali zation. So me lesions contai n
n u me ro u s
u n i f o r m,
r o und ,
o f te n
l a m i na ted s t r uc tu re s d e s cr i b e d a s
o s sic les or ps a mmoma like bo die s .
Foci o f mul tinucleated g iant cell s may
also be present.
References
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