A d e rn a r L o p e s , S a n d ra M o rin i, L e o n a rd o J o s é V ie ira , A n to n io T a lv a n e d e O liv e ira
C h o n d ro sa rc o m a se c o n d a ry to h e re d ita ry m u Itip Ie
e x o sto sis tre a te d b y e x te n d e d in te rn a I h e m ip Ie v e c to m y
Pelvie Surgery, Pathology Department
and Surgery Residents,
Hospital A.C. Camargo, Antonio Prudente Foundation
- São Paulo, Brazil
T h e a u th o rs re p o rt o n th e ca se o f a 2 8 -ye a r-o ld p a tie n t w ith e xte n sive ch o n d ro sa rco m a o f th e le ft isch iu m a n d p u b is in vo lvin g h ip jo in t, skin , a n d so ft tissu e o f th e g lu te a l re g io n , se co n d a ry to h e re d ita ry m u ltip le e xo sto sis su b m itte d to a n e xte n d e d in te rn a i E n n e kin g typ e 1 1a n d 1 1 1h e m ip e lve cto m y. N o p ro sth e sis o r a rth ro d e sis w a s u se d . A fe w ye a rs a g o , p a tie n ts w ith e xte n sive tu m o rs like th is o n e w e re tre a te d w ith in te rilio a b d o m in a l a m p u ta tio n , re su ltin g in a lo ss o f q u a lity o f Iife .T w o ye a rs a fte r th e lim b -p re se rvin g su rg e ry, th is p a tie n t w a s d ise a se fre e , w ith g o o d fu n ctio n a l re su lts, in clu d in g b ip e d a l a m b u la tio n w ith su p p o rt.
U N IT E R M S : C h o n d ro sa rco m a . O ste o ch o n d ro m a . S u rg e ry. P ro sth e sis. Ilia c b o n e . O ste o ch o n d ro m a to sis.
IN T R O D U C T IO N
H
B o y e r in 1 8 1 4 ,e r e d ita r y m u ltip leIis a d o m in a n te x o s to s is ( H M E ) ,a u to s o m a ld e s c r ib e din h e r ite db yd is o r d e r c h a r a c te r iz e d b y th e p r e s e n c e o f m u lti p I e
o s te o c h o n d r o m a . T r a n s f o r m a tio n o f H M E r a n g e s f r o m 1 0
-3 8 p e r c e n t, a n d in s o lita r y o s te o c h o n d r o m a f r o m 1 - 1 6
p e r c e n t. 2 S u r g e r y is th e tr e a tm e n t o f c h o ic e f o r
o s te o c h o n d r o m a a s w e ll a s f o r c h o n d r o s a r c o m a . T h is
r e p o r t p r e s e n ts a c a s e o f lo c a lly a d v a n c e d c h o n d r o s a r c o m a ,
s e c o n d a r y to H M E . A lth o u g h a c a n d id a te f o r a n
in te r ilio a b d o m in a l a m p u ta tio n , th e p a tie n t's lim b w a s
A d d re s s fo r c o rre s p o n d e n c e :
A d e rn a r L o p e s M O , P h .D . H o s p ita l A . C . C a m a rg o
D e p a rta m e n to d e C iru rg ia P é lv ic a R u a P ro t. A n to n io P ru d e n te , 2 1 1
S ã o P a u lo /S P - B ra s il - C E P 0 1 5 0 9 -0 1 0
p r e s e r v e d w ith a n in te r n a I e x te n d e d h e m ip e lv e c to m y ,
w ith o u t u tiliz a tio n o f p r o s th e s is o r a r th r o d e s is f o r th e
r e c o n s tr u c tio n . T h e p a tie n t p r e s e n te d g o o d o n c o lo g ic a l,
e s th e tic a n d f u n c tio n a l r e s u lts tw o y e a r s p o s ts u r g e r y .
C A S E R E P O R T
A 2 8 - y e a r - o ld w h ite m a le w a s f ir s t s e e n o n A p r il1 0 ,
1 9 9 4 , r e p o r tin g a tu m o r in th e le f t g lu te a l r e g io n f o r th e
p r io r f iv e y e a r s . H e h a d b e e n s u b m itte d to a p r e v io u s
r e s e c tio n e ls e w h e r e , a n d h a d s u f f e r e d a lo c a l r e c u r r e n c e .
D e a m b u la tio n w a s h in d e r e d b y r e s tr ic tio n o f h ip jo in t
m o v e m e n ts . U p o n p h y s ic a l e x a m in a tio n , th e p a tie n t
e x h ib ite d a n e x te n s iv e , h a r d tu m o r a l m a s s w ith a f lu te d
s u r f a c e m e a s u r in g 3 0 x 2 8 e m , in v o lv in g th e g lu te a l g r o o v e
a n d e x te n d in g to th e p e r in e u m w ith c o m p r e s s io n o f th e
p e n ile r o o t ( F ig .1 ) . U p o n r e c ta l e x a m in a tio n , th e tu m o r
f ille d o u t th e is c h io r e c ta l c a v ity , w ith c o m p r e s s io n o f th e
FIGURE 1 -L o c a lly a d v a n c e d c h o n d ro s a rc o m a .
left lateral w all o f th e rectu m . T h e lesio n in v o lv ed b o n e
an d w as attach ed to th e sk in n ear th e g lu teal g ro o v e at th e
m o st p ro m in en t p art o f th e tu m o r, an d at th e lev eI o f th e
p rev io u s su rg ical scar.
A C T scan sh o w ed a larg e tu m o r w ith in terio r
calcificatio n s lo cated in th e left g lu teal reg io n , d estro y in g
th e isch iu m an d th e p u b is an d ex ten d in g to th e acetab u lar
reg io n w ith ex ten siv e so ft tissu e in v o lv em en t (F ig . 2 ). T h e
rem ain in g p h y sical ex am in atio n sh o w ed n eg ativ e resu lts.
T h e rem ain in g lab o rato ry an d clin icaI ex am in atio n s,
in clu d in g h em o g ram , b io ch em ical tests, ch est C T ,
ab d o m in al u ltraso n o g rap h y an d E C G w ere n o rm al.
A fam ily h isto ry rev ealed th at th e p atien t's fath er,
th ree o u t o f fo u r b ro th ers, an d o n e sister p resen ted m u lti p Ie
b o n e ex o sto sis, as d id h is tw o ch ild ren , a g irl an d a b o y
(F ig . 3 ).
Gn
A p ril 2 7 , 1 9 9 4 , th e p atien t w as su b m itted toan ex ten d ed in tern aI E n n ek in g3 ty p e 1 1 an d 1 1 1
,. an d _
=
p atien ts w ith o steo ch o n d ro m ato sista
=case w ith tran sfo rm atio n fo r ch o n d ro sarco m aFIGURE 3 - H e re d ita ry h is to ry p o in tin g o u t th e p a tie n t w ith tra n s fo rm a tio n o f th e o s te o c h o n d ro m a .
FIGURE 2 -
T e
s c a n s h o w in g tu m o r w ith c a lc ific a tio n s d e s tro y in g is c h iu m a n d p u b is , w ith e x te n s io n to s o ft tis s u e .h em ip elv ecto m y , th e rad ical rem o v al o f th e en tire tu m o r,
in clu d in g th e attach ed sk in an d su rro u n d in g so ft tissu e.
T h e isch ial an d ilio p u b ic b ran ch es an d th e acetab u lu m w ith
th e fem u r's h ead an d n eck w ere also rem o v ed (F ig . 4 ). N o
p ro sth esis w as u sed fo r resto ratio n , b u t a m etal th read w as
u sed to attach th e fem u r to th e rem ain in g iliac b o n e (F ig .5 ).
F ' A P 5 1• • •0 1 7 1 7 d
FIGURE 4 -S u rg ic a l s p e c im e n re s u ltin g fro m e x te n d e d re s e c tio n .
L O P E S , A .; M O R IN I, S .; V IE IR A , L .J .; O L IV E IR A , A .T . - C h o n d ro s a rc o m a s e c o n d a ry to h e re d ita r m u ltip le e x o s to s is tre a te d b y e x te n d e d in te rn a i h e m ip le v e c to m y
1 4 4 2
F IG U R E 5 - X -ra y s h o w in g lo s s o f b o n e m a s s , m e ta l th re a d a tta c h in g th e fe m u r to th e ilia c b o n e .a n d ilio -fe m o ra l p s e u d o
a rth ro s is a s a re s u lt o f fib ro s is .
F IG U R E 6 - C o n d ro s a rc o m a w ith c e l! v a ria tio n in s h a p e , s iz e ,
b in u c le a tio n a n d a ty p ic a l (H E - 4 0 0 x ).
a c e ta b u la r c a v ity a n d h e a d a n d n e c k o f th e fe m u r (F ig . 4 ).
A t th e c u t su rfa c e , th e tu m o r p re se n te d a w h ite c o lo r,
lo b u la te d a sp e c t, b rittle a n d e x te n d in g fro m th e
su b c u ta n e o u s tissu e a n d to th e d e stro y e d b o n e .
M ic ro sc o p ic e x a m in a tio n d isc lo se d a m o d e ra te ly
d iffe re n tia te d c h o n d ro sa rc o m a (F ig . 6 ) in v o lv in g th e sk in
a t th e le v e I o f th e su rg ic a l sc a r, a d ja c e n t m u sc le a n d b o n e .
N o n e o p la sia w a s fo u n d o n th e su rg ic a l m a rg in s. T h e
p a tie n t is a liv e a n d fre e o f d ise a se w ith p o sitiv e e sth e tic
a n d fu n c tio n a l re su lts, in c lu d in g d e a m b u la tio n w ith
su p p o rt (F ig . 7 ).
D IC C U S S IO N
S e c o n d a ry c h o n d ro sa rc o m a is re la te d to a p rio r b o n e
le sio n , su c h a s e ith e r so lita ry o r m u ltip le o ste o c h o n d ro m a .
B o n e e x o sto sis a p p e a rs p re d o m in a n tly in m a le s in e a rly
c h ild h o o d , a n d th e tra n sfo rm a tio n in to sa rc o m a s o c c u rs
in a d u lth o o d , b e tw e e n 2 0 -4 0 y e a rs.
R a p id g ro w th , p a in a n d c a lc ific a tio n s a re sig n s o f
m a lig n a n t tra n sfo rm a tio n . In H M E , h a lf o f th e o ffsp rin g o f
th e a ffe c te d p a tie n t b e a r th e g e n e a n d p re se n t e x o sto sis.
T h e h e re d ita ry h isto ry (F ig . 3 ) o fth is p a tie n t's fa m ily sh o w s
th e re la tiv e s w ith o ste o c h o n d o rm a to sis, in c lu d in g th is c a se
in w h ic h m a lig n a n t tra n sfo rm a tio n o c c u rre d . T h e stu d y o f
th e g e n e in v o lv e d in H M E , a s w e ll a s its p o ssib le m u ta tio n s,
m ig h t b e o f m a jo r sig n ific a n c e fo r fa m ily p la n n in g a n d
th e ra p e u tic c o u n se lin g , a s a lre a d y o c c u rs w ith o th e r
h e re d ita ry d ise a se s su c h a s fa m ilia l c o lo n p o ly p o sis.
P a tie n ts w ith th is sy n d ro m e m u st b e c a re fu lly
fo llo w e d , a n d a n y e x o sto sis p re se n tin g sig n s o f
F IG U R E 7 - P a tie n t w ith b ip e d a l s u p p o rt tw o y e a rs a fte r s u rg e ry . tra n sfo rm a tio n s o r fu n c tio n a l d iso rd e r re se c te d .
T h e p a tie n t h a d a g o o d p o sto p e ra tiv e re c o v e ry a n d sta rte d
d e a m b u la tio n w ith c ru tc h e s 1 5 d a y s a fte r su rg e ry , b e in g
d isc h a rg e d o n th e d a y 1 9 .
T h e su rg ic a l sp e c im e n m e a su re d 2 6 x 2 4 x 2 4 e m ,
in c lu d in g sk in , m u sc le s a n d b o n e stru c tu re o f th e
C h o n d ro sarco m a seco n d ary to o steo ch o n d ro m ato sis is a lo w -g rad e m alig n an cy , an d resectio n w ith ad eq u ate m arg in s sig n als a g o o d p ro g n o sis.4
R ecen tly , g reat p ro g ress h as b een m ad e in th e treatm en t o f m alig n an t b o n e tu m o rs o f th e p elv ic g ird le w ith lim b -sav in g su rg ery p ro ced u res. In tern aI h em ip elv ecto m y is little k n o w n w ith in o u r .su rg ical co m m u n ity . T h is p ro ced u re can su b stitu te so m e cases o f in terilio ab d o m in al am p u tatio n w ith o u t im p airm en t o f th e o n co lo g ical m arg in s, an d im p ro v e q u ality o f life.
In o u r case, an ex ten d ed in tern aI h em ip elv ecto m y w ith o u t u tilizatio n o f arth ro d esis o r p ro sth esis w as
1 4 4 3
p erfo rm ed as p rev io u sly d escrib ed . In th is case, as w ell as in o th ers w e h av e stu d ied ,5 fib ro sis fo rm ed in th e stu m p o f th e am p u tated fem u r, so ft tissu e an d /o r rem ain in g iliac b o n e (F ig . 7 ) six m o n th s after su rg ery . T h is allo w ed th e p atien t n o n su p p o rted d eam b u latio n u n d er lo ad , an d g reater ease in sittin g d o w n b ecau se o f th e lim b 's flex ib ility aro u n d th e fib ro u s area; th is d o es n o t o ccu r w h en p ro sth esis is u sed . F u rth er, w ith th e su rg ical tech n iq u e u sed in th is case, w e av o id ed th e co m p licatio n s d u e to in fectio n freq u en tly rep o rted w ith p ro sth esis u se.6
R E S U M O
O s a u to re s d e s c re v e m o c a s o d e u m p a c ie n te d e 2 8 a n o s , c o m e x te n s o c o n d ro s s a rc o m a d o ís q u io e p ú b is e s q u e rd o s e n v o lv e n d o a rtic u la ç ã o c o x o -fe m u ra l, p a rte s m o le s e p e le d a re g iã o g lú te é l, s e c u n d á rio à o s te o c o n d ro m a to s e m ú ltip la fa m ilia r e s u b m e tid o
'a h e m ip e lv e c to m ia in te rn a tip o E n n e k in g ll e 1 1 1a la rg a d a . N ªo s e u s o u n e n h u m tip o d e p ró te s e o u a rtro d o s e ,: H á p o u c o s a n o s , p a c ie n te s c o m tu m o re s d e s s a e x te n s ã o e ra m s u b m e tid o s ,a a m p u ta ç ã o in te rílio a b d o m in a l c o m p e rd a d o m e m b ro e p io ra d a
q u a lid a d e d e v id a . D o is a n o s 'a p ó s a c iru rg ia p r~ s ~ rv a d o ra d º m e m b ro , o p a c ie n te e s tá liv re d a d o e n ç a , c o m b o m re s u lta d o fu n c io n a l, in c lu s iv e , c o m a p o io b ip o d á lic o a u )(ilia d o P o ~ ~ u P Q rte .;
REFERENCES
1 . B o y er A . T raite d es m alad ies ch iru rg icales, V o 1 .3 . P aris: V e.
M ig n eret, 1 8 1 4 :5 9 4 .
2 ~ M an k in H J, C an tley K P , L ip p iello L . et aI: T h e b io lo g y o fh u m an
ch o n d ro sarco m a. I. D escrip tio n o f th e series, g rad in g , an d
b io ch em icaI an aIy ses. J B o n e Jo in t S u rg 1 9 8 0 ;6 2 : 1 6 0 -7 6 .
3 . E n n ek in g W F , D u n h am W K . R esectio n an d reco n stru ctio n
fo r p rim ary n eo p lasm s en v o lv in g th e in n o m in ate b o n e. J
B o n e Jo in t S u rg 1 9 7 8 ;6 0 :7 3 1 -4 6 .
4 . L o p es A , F ig u eired o M T A , G en til F C . C o n d ro ssarco m a
lo calm en te av an çad o d o o sso ilíaco tratad o p o r am p u tação
in terilio ab d o m in al e rep arad o co m retalh o s m io cu tân eo
ân tero -m ed ial d a co x a: R elato d e u m caso . R ev B ras O rto p
1 9 8 9 ;2 4 :2 5 5 -5 9 .
5 . L o p es A , P en n a V , R o ssi B M , C h u n g W T , T an ak a M H .
H em ip elv ecto m ia to tal in tern a n o tratam en to d e tu m o res
m alig n o s d a reg ião p élv ica. R ev B ras O rto p 1 9 9 4 ;2 9 : 1 1 -2 2 .
6 . H u th JF , E ck ard t JJ, P ig n atti G , E ilb er F R . R esectio n o f
m alig n an t b o n e tu m o rs o f th e p elv ic g irg le w ith o u t ex trem ity
am p u tatio n . A rch S u rg 1 9 8 8 ;1 2 3 : 1 1 2 1 -2 4 .
L O P E S , A .; M O R IN I, 5 .; V IE IR A , L .J .; O L IV E IR A , A .T . - C h o n d ro s a rc o m a s e c o n d a ry to h e re d ita r m u ltip le e x o s to s is tre a te d b y e x te n d e d in te rn a i h e m ip le v e c to m y
