IC \L
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1
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.
REPORT
A d e m a r L o p e s , B e n e d it o M a u r o R o s s i, C la u d io R e g is S a m p a io S ilv e ir a , A n t o n io C o r r e a A lv e sC h o r d o m a : r e tr o s p e c tiv e a n a ly s is o f 2 4 c a s e s
A.
C .Camargo Hospital, Antonio Prudente Founfation - Sao Paulo, Brazil
Introduction: C hordom a is a rare and slow -grow ing tum or, w ith local aggressiveness and preferential localization in the vertebral
colum n. O bjective: The m ain objective of this study is to evaluate natural history and results of treatm ent of chordom as. M
ethodol-ogy: This is a retrospective study from 1953 to 1993. M aterial and m ethods: The age ranged from 2 to 86 years (m ean=34.5). Tw elve patients w ere m ale and 12 fem ale. The localization of the tum or w as: 20 in the sacral region, 3 in head and neck and one out of the
spine. R esults: The treatm ent, alone or com bined, w as surgery, radiation therapy and chem otherapy. The survival rate for patients
w ith lesions in the sacrum ranged from 4 to 119 m onths, since the date of the sym ptom s. The 5-year overall survival w as 4.2% .
C onclusion: C hordom a is a rare and slow grow ing tum or, w ith a very difficult approach by surgery due to its preferential location in the
sacrum and poor therapeutic results w ith radiation therapy or chem otherapy, m ainly in patients w ith advanced disease.
U N ITE R M S : C hordom a, sacral tum or, notochord, bone tum or, treatm ent.
IN TR O D U C TIO N
C
h o r d o m a w a s f i r s t d e s c r i b e d b y V i r c h o w i n 1 8 5 7u n d e r t h e n a m e o f " e c c h o r d o s i s p h y s a l i p h o r a " .'7 I n 1 8 9 4 , R i b b e r t d e f i n e d c h o r d o m a a s a m a l i g n a n t t u m o r a r i s i n g f r o m e m b r y o n i c r e m a i n s o f t h e n o t o c h o r d .'2
T h e p r e f e r e n t i a l l o c a l i z a t i o n o f c h o r d o m a i s i n t h e v e r t e b r a l c o l u m n , f r o m t h e s p h e n o - o c c i p i t a l s y n c h o n d r o s i s t o t h e c o c c y x , m o r e f r e q u e n t l y i n t h e s a c r o c o c c y g e a l r e g i o n Y
T h e m a j o r i t y o f c h o r d o m a s a r e s l o w g r o w i n g w i t h l o c a l p r o l i f e r a t i o n a n d h i g h a g g r e s s i v e n e s s a n d i n f i l t r a t i o n o f t h e a d j a c e n t o r g a n s a n d s t r u c t u r e s ( F i g . 1 ) . S u r g e r y i s t h e o n l y c u r a t i v e t r e a t m e n t 7 .X .l J .1 3w h e n a d e q u a t e m a r g i n s a r e o b t a i n e d . T h e r e i s a l o w r a t e o f m e t a s t a s e s f o r t h i s t y p e o f t u m o r .7
A d d r e s s f o r c o r r e s p o n d e n c e : A d e m a r L o p e s
D e p a r t a m e n t o d e C ir u r g ia P e lv ic a
H o s p it a l A. C.C a m a r g o - F u n d a 9 a O A n t o n io P r u d e n t e R u a P r o f . A n t o n io P r u d e n t e , 2 1 1
S a o P a u lo / S P - B r a s il- C E P 0 1 5 0 9 - 0 1 0
Figure 1 - Fem ale patient w ith a large tum or bulging the posterior region of the pelvis.
f o r a 2 1 - m o n t h p e r i o d ; t h e s e c o n d w a s s u b m i t t e d
e x c l u s i v e l y t o r a d i a t i o n t h e r a p y , d y i n g d u e t o t h e d i s e a s e
a f t e r I I m o n t h s , a n d t h e t h i r d w a s t r e a t e d w i t h s u r g e r y
a l o n e a n d h a s b e e n d i s e a s e f r e e f o r a 3 - m o n t h p e r i o d .
T h e p a t i e n t w i t h t u m o r i n t h e r e t r o p e r i t o n e u m w a s
s u b m i t t e d e x c l u s i v e l y t o c h e m o t h e r a p y , d y i n g I I m o n t h s
a f t e r a d m i s s i o n .
F r o m t h e t o t a l o f 2 4 c a s e s , e i g h t ( 3 3 .4 % ) h a v e
d e v e l o p e d m e t a s t a s e s i n t h e f o l l o w i n g s i t e s : l u n g - 5 ; l i v e r ,
I y m p h o n o d u s a n d b o n e - o n e c a s e e a c h . T h e 5 - y e a r o v e r a l l
s u r v i v a l i s s h o w n i n F i g u r e 2 .
C h o r d o m a i s a r a r e n e o p l a s m e v e n i n a c a n c e r c e n t e r .
T h e p r e s e n t s e r i e s o f 2 4 c a s e s r e p r e s e n t e d 0 .0 2 % o f a l l
7 9 ,9 1 7 n e w c a n c e r c a s e s s e e n a t t h e A .C .C a m a r g o H o s p i t a l
( 1 9 5 3 - 1 9 9 3 ) . A m o n g t h e 1 ,0 3 0 c a s e s o f b o n e t u m o r s i n
t h e s a m e p e r i o d , c h o r d o m a s c o r r e s p o n d e d o n l y t o 2 .2 % .
T h e c h o r d o m a c a n a r i s e f r o m a l l a x i a l s k e l e t o n , b u t
v a r i o u s a u t h o r s s h o w t h a t 5 0 % b e g i n i n t h e s a c r u m , 3 5 %
i n t h e s p h e n o - o c c i p i t a l r e g i o n a n d 1 5 % i n t h e
v e r t e b r a e .:U.7.1
'J T h e l o c a l i z a t i o n o u t o f t h e s p i n e i s r a r e .')
I n t h i s s e r i e s , t h e m a j o r i t y o f t h e t u m o r s w e r e i n t h e s a c r a l
r e g i o n , b u t w e m u s t r e m e m b e r t h a t b e f o r e t h e i n t r o d u c t i o n
o f c o m p u t e r i z e d t o m o g r a p h y , i t w a s d i f f i c u l t t o o b t a i n t h e
d i a g n o s i s o f s p h e n o - o c c i p i t a l l e s i o n s .
T h e t i m e e l a p s e d b e t w e e n t h e i n i t i a l s y m p t o m s a n d
t h e t r e a t m e n t i s u s u a l l y v e r y l o n g , w h i c h i s a c h a r a c t e r i s t i c
o f a s l o w - g r o w i n g t u m o r . P r e s e n c e o f m a s s a n d s a c r a l p a i n ,
w i t h r e c t a l a n d u r i n a r y b l a d d e r d y s f u n c t i o n a r e t h e m a i n
s y m p t o m s o f t h e d i s e a s e i n t h e s a c r o c o c c y g e a l r e g i o n .l
)
T h e d i g i t a l r e c t a l e x a m i n a t i o n i s a v e r y i m p o r t a n t
p r o p e d e u t i c m e t h o d f o r d i a g n o s i s o f s a c r a l t u m o r s .
I n s p h e n o - o c c i p i t a l l e s i o n s t h e m a i n s i g n s a n d
s y m p t o m s a r e p a i n , m a s s - i n v a d i n g n a s o p h a r y n x ,
F ig u re 2 - F iv e -y e a r o v e ra ll s u rv iv a l
o
to
N
,...
1 0 090
80
7 0
60
5 0
4 0 3 0 2 0 1 0 o
o
I n t h e 2 0 c a s e s w i t h t u m o r i n t h e s a c r a l r e g i o n , t h e
t r e a t m e n t a n d o v e r a l l s u r v i v a l w e r e : a ) r a d i a t i o n t h e r a p y
a l o n e ( 5 c a s e s ) - 5 t o 5 0 m o n t h s ( m e a n - 1 8 .8 ) ; b ) r a d i a t i o n
t h e r a p y a n d s u r g e r y ( 4 c a s e s ) - I t o 8 8 m o n t h s ( m e a n
-2 7 .8 ) ; c ) r a d i a t i o n t h e r a p y , c h e m o t h e r a p y a n d s u r g e r y ( 4
c a s e s ) - 6 t o 4 5 m o n t h s ( m e a n - 2 4 .8 ) ; d ) s u r g e r y a l o n e ( 3
c a s e s ) - I I t o 1 7 m o n t h s ( m e a n - 1 4 ) ; e ) c h e m o t h e r a p y a n d
s u r g e r y ( I c a s e ) - 2 7 m o n t h s ; f ) c h e m o t h e r a p y a n d r a d i a t i o n
t h e r a p y ( I c a s e ) - 3 1 m o n t h s . T w o p a t i e n t s d i d n o t r e c e i v e
a n y t r e a t m e n t d u e t o t h e a d v a n c e d s t a g e o f t h e d i s e a s e .
I n t h e 3 c a s e s w i t h t u m o r s i n t h e h e a d a n d n e c k r e g i o n
( a g e s 1 0 , I I a n d 2 1 y e a r s o l d ) , t h e f i r s t w a s t r e a t e d w i t h
s u r g e r y a n d r a d i a t i o n t h e r a p y a n d h a s b e e n d i s e a s e f r e e
R E S U L T S
M A T E R IA L S A N D M E T H O D S
A s e r i e s o f 2 4 p a t i e n t s w i t h c h o r d o m a s w a s
i d e n t i f i e d i n t h e H o s p i t a l f i l e s ( 1 9 5 3 - 1 9 9 3 ) , w i t h a g e s
r a n g i n g f r o m 2 t o 8 6 y e a r s ( m e a n = 3 4 .5 ) , 1 2 m a l e s a n d
1 2 f e m a l e s ; 2 2 w e r e w h i t e . T h e a n a t o m i c a l l o c a t i o n w a s :
s a c r u m - 2 0 c a s e s ( 8 3 .4 % ) , 8 w i t h e x t e n s i o n t o t h e c o c c y x
a n d 5 i n v a d i n g t o t h e l u m b a r c o l u m n ; h e a d a n d n e c k - 3
c a s e s ( 1 2 .4 % ) i n t h e c l i v u s r e g i o n ; r e t r o p e r i t o n e u m - I
c a s e ( 4 .2 % ) . A s u m m a r y o f c l i n i c a l d a t a , t r e a t m e n t a n d
f o l l o w - u p i s s h o w n i n T a b l e 1 .
W h e n u l t r a s o n o g r a p h y , c o m p u t e r i z e d t o m o g r a p h y
a n d m a g n e t i c r e s o n a n c e w e r e n o t a v a i l a b l e , t h e d i a g n o s i s
o f t u m o r s i n t h e s a c r a l r e g i o n w e r e m a d e t h r o u g h d i g i t a l
r e c t a l e x a m i n a t i o n a n d b a r i u m e n e m a . I n t h e a b s e n c e o f
i m a g i n g e x a m s , t h e d i a g n o s i s o f c h o r d o m a s o f o t h e r
l o c a t i o n s w e r e v e r y d i f f i c u l t d u e t o t h e l a c k o f o t h e r
t r u s t w o r t h y m e t h o d s .
Q U I' t h e r a p e u t i c a p p r o a c h f o r c h o r d o m a s i s c u r a t i v e
s u r g e r y f o l l o w e d b y a d j u v a n t r a d i a t i o n t h e r a p y a n d / o r
c h e m o t h e r a p y . T h i s c o n d u c t w a s n o t a l w a y s p o s s i b l e i n
o u r s e r i e s b e c a u s e 1 3 o u t 2 4 c a s e s w e r e p r e v i o u s l y
s u b m i t t e d t o v a r i o u s t y p e s o f t r e a t m e n t w i t h r e s i d u a l o r
r e c u r r e n t d i s e a s e , a n d t h e m a j o r i t y o f t h e i n t a c t c a s e s h a d
a d v a n c e d t u m o r s . I n t w o c a s e s , d u e t o t h i s r e a s o n , n o
t r e a t m e n t w a s p o s s i b l e .
R a d i a t i o n t h e r a p y , c o m b i n e d o r a l o n e , w a s t h e
t r e a t m e n t i n 1 6 ( 6 6 .7 % ) o f t h e c a s e s , b e i n g 9 ( 3 7 .5 % )
a s s o c i a t e d w i t h s u r g e r y a n d 5 ( 2 0 .9 % ) w i t h c h e m o t h e r a p y .
C h e m o t h e r a p y w a s t h e e x c l u s i v e t r e a t m e n t i n t h e c a s e o f
t h e r e t r o p e r i t o n e a l t u m o l '.
L O P E S ,A .; R O S S I, 8 .M .; S IL V E IR A , C .R .S . & A L V E S , A .C . - C h o rd o m a : re tro s p e c tiv e a n a ly s is o f 2 4 c a s e s
Table I
Characteristics of the patients, treatment and follw-up
# A g e (y r.) S e x D u ra tio n o f s y m p to m s S y m p to m s S ite A d m is s io n s ta g e P re v io u s tre a tm e n t In s titu tio n tre a tm e n t F o llo w -u p
S S m 9 6 m o . lo c a l p a in s a c ra l liv e r m e ta s ta s e s b io p s y n o d e a d a fte r
n a rro w fe c e s a d v a n c e d s a c ra l 1 3 m o .
re c ta l/b la d d e r tu m o r
d y s fu n c tio n
2 2 2 m o . s a c ra l tu m o r s a c ra l in g u in a l b io p s y R T (3 6 G y ) d e a d a fte r
lo s s o f w e ig h t ly m p h o n o d u s S m o .
a d v a n c e d s a c ra l tu m o r
3 2 2 4 m o . s a c ra l tu m o r a n d s a c ra l lu n g m e ta s ta s e s s u rg e ry R T (3 6 G y ) lo s t a fte r
p a in a d v a n c e d s a c ra l C T (a c t) 8 m o . w ith
tu m o r d is e a s e
4 2 8 7 2 m o . s a c ra l p a in w ith s a c ra l lu n g m e ta s ta s e s s u rg e ry s u rg e ry d e a d a fte r
Irra d ia tio n to a d v a n c e d s a c ra l R T (3 6 G y ) 6 m o .
th ig h s a n d le g s tu m o r C T (a c t)
S 4 0 m 6 0 m o . s a c ra l p a in w ith s a c ra l a d v a n c e d s a c ra l s u rg e ry R T (S O G y ) d e a d a fte r
irra d ia tio n to tu m o r C T (c y c + S F ) 4 0 m o .
th ig h s a n d le g s
6 6 2 m 8 4 m o . s a c ra l tu m o r a n d s a c ra l a d v a n c e d s a c ra l s u rg e ry C T d e a d a fte r
p a in re c ta l/b la d d e r tu m o r (S F U + a c t+ 2 7 m o .
d y s fu n c tio n v in )
7 2 S S m o . s a c ra l tu m o r a n d s a c ra l a d v a n c e d s a c ra l s u rg e ry R T (3 6 G y ) d e a d a fte r
p a in tu m o r 1 0 m o .
8 4 4 m 2 4 m o . s a c ra l a n d le g s a c ra l s a c ra l tu m o r s u rg e ry R T (S O G y ) d e a d a fte r
p a in 8 8 m o .
9 6 3 m 7 6 m o . la rg e s a c ra l s a c ra l lu n g m e ta s ta s e s s u rg e ry C T d e a d a fte r
tu m o r a n d p a in a d v a n c e d s a c ra l R T (? G y ) (c y c + S F U + 4 S m o .
tu m o r M T X )
1 0 1 0 3 6 m o . n e c k p a in ,c o u g h c liv u s la rg e c e rv ic a l b io p s y tra c h e o s to -m y d e a d a fte r
c ra n ia l n e rv e s tu m o r w ith R T (7 0 G y ) 1 1 m o .
d y s fu n c tio n (III, re s p ira to ry
IV ,V I,V II,X II) o b s tru c tio n
1 1 6 9 m 4 m o . s a c ra l p a in a n d s a c ra l lu n g m e ta s ta s e s n o R T (6 0 G y ) d e a d a fte r
re c ta l a d v a n c e d s a c ra l 2 0 m o .
d is fu n c tio n tu m o r
1 2 4 2 m 2 4 m o . s a c ra l p a in a n d s a c ra l a d v a n c e d s a c ra l b io p s y R T (3 0 G y ) d e a d a fte r
tu m o r b la d d e r tu m o r 1 2 m o .
is fu n c tio n
1 3 2 4 S 'm o . s a c ra l p a in a n d s a c ra l lu n g m e ta s ta s e s s u rg e ry n o d e a d a fte r
lo s s o f w e ig h a d v a n c e d s a c ra l R T (S O G y ) 1 m o .
tu m o r
1 4 S 4 m 2 4 m o . s a c ra l tu m o r a n d s a c ra l a d v a n c e d s a c ra l b io p s y n o lo s t a fte r
p a in , lo s s o f tu m o r 1 m o . w ith
w e ig h d is e a s e
1 S 8 6 7 m o . s a c ra l p a in s a c ra l s a c ra l tu m o r n o R T (S O G y ) d e a d a fte r
S O m o .
1 6 3 9 m 4 m o . s a c ra l tu m o r a n d s a c ra l b o n e m e ta s ta s e s b io p s y R T (4 0 G y ),C T d e a d a fte r
p a in , w ith irra d i- s a c ra l tu m o r R T (4 0 G y ) (v in + a d r+ c y c + 3 1 m o .
a tio n to th ig h s c is + d ti)
1 7 6 1 8 m o . s a c ra l tu m o r a n d s a c ra l a d v a n c e d s a c ra l s u rg e ry n o d e a d a fte r
p a in tu m o r 1 4 m o .
1 8 3 7 m ? s a c ra l p a in s a c ra l a d v a n c e d s a c ra l n o p a llia tiv e lo s t a fte r
tu m o r s u rg e ry 1 7 m o .
1 9 2 3 m 1 6 8 m o . s a c ra l tu m o r a n d s a c ra l a d v a n c e d s a c ra l b io p s y n o lo s t a fte r
p a in , le g p a in , tu m o r R T (6 0 G y ) 7 m o .
re c ta l/b la d d e r w ith d is e a s e
d y s fu n c tio n
2 0 1 1 m 1 2 m o . n e c k p a in c liv u s c e rv ic a l tu m o r n o s u rg e ry d is e a s e fre e
R T (4 S G y ) a fte r
2 1 m o .
2 1 4 6 4 6 m o . s a c ra l tu m o r s a c ra l s m a ll s a c ra l n o s u rg e ry lo s t, d is e a s e
tu m o r fre e a fte r
1 1 m o .
2 2 2 1 1 2 m o . n e c k p a in ,c o u g h c liv u s la rg e c e rv ic a l s u rg e ry s u rg e ry d is e a s e fre e
c ra n ia l n e rv e s tu m o r w ith a fte r
d y s fu n c tio n (V I, re s p ira to ry 3 m o .
IX ,X ,X I,X II) o b s tru c tio n
2 3 1 8 2 m o . a b d o m in a l p a in o u t o f re tro p e rito n e a l b io p s y C T (v b l+ c is + d e a d a fte r
s p in e tu m o r b le ) 1 1 m o .
2 4 2 3 m 1 8 m o . le g p a in , re c ta l s a c ra l a d v a n c e d s a c ra l p a llia tiv e R T (S O G y ) d e a d a fte r
d y s fu n c tio n tu m o r s u rg e ry 1 2 m o .
m -m a le , f-fe m a le , R T -ra d ia tio n th e ra p y , C T -c h e m o th e ra p y , a c t-a c tin o m y c in , c y c -c y c lo p h o s p h a m id e , v b l-v in b la s tin e
S F U .-S flu o ro u ra c il, v in -v in c ris tin e , M T X -m e th o tre x a te , a d r-a d ria m y c in , c is -c is p la tin , d ti-d a c a rb a z in e , b le -b le o m y c in
~~~~"'''''''''w''''''':'''~''''''''':-m:-:*~w''''''''''''''''''''' . ~lo'-" ~-:-::m: .~~~:t::::r~&::m::~~~:::::&:::::::&."%iW"~~~~~W~
S a o Paulo Medical Journal/RPM 1 1 4 (6 ): 1 3 1 2 -1 3 1 6 , 1 9 9 6 L O P E S ,A .; R O S S I, 8 .M .; S IL V E IR A , C .R .S . & A L V E S , A .C . - C h o rd o m a :
so m etim es
w ith o b stru ctio n
o f air p assag e an d d y sfu n ctio n
o f cran ial
n erv es.lI
T h e m etastases
rate fo u n d in o u r series (3 3 .4 % )
is
w ith in th e ran g e d escrib ed
in th e literatu re.4 .1 I.lJ.1 1 I
C o n v en tio n al
x -ray , co m p u terized
to m o g rap h y
an d
m ag n etic
reso n an ce
are v ery im p o rtan t
in th e d iag n o sis
an d to p lan th e b etter treatm en t. In th e h isto p ath o lo g y ,
th e
p resen ce o f larg e cells w ith lo b u lar arran g em en t
an d in
tra-cy to p lasm atic
m u cu s,
called
p h y salip h o ra
cells,
is
ch aracteristic
o f ch o rd o m as
(F ig . 3 ).
S u rg ery
w ith ad eq u ate
m arg in s is th e b est av ailab le
treatm en t
fo r ch o rd o m as,
b u t th is is n o t alw ay s p o ssib le
d u e to th e ad v an ced
stag e
o f th e d isease
an d d ifficu lt
access
5(F ig . 4 ).
T h e ex ten t o f cu rativ e su rg ery is a d ilem m a, b ecau se
th e to tal rem o v al
o f th e tu m o r m an y tim es im p lies rectal
an d u rin ary b lad d er
im p airm en t.
T h e
resu lts
o f rad iatio n
th erap y
alo n e
are
n o t
satisfacto ry ,
h o w ev er
is in d icated
in th e ab sen ce o f b etter
th erap eu tic
o p tio n s 1 I.1 1 I
an d th e d o sag e v aries fro m 7 0 to 8 0
G y .".1 6
T h e
ch em o th erap y
(P Y B
sch em e
- cisp latin ,
v in b lastin e,
b leo m y cin )
w as u sed in o u r retro p erito n eal
case o f ch o rd o m a
an d w as u n su ccessfu l.
A zzarelli rep o rted
o n e case
w ith
co m p lete
rem issio n
w ith ch em o th erap y
(P Y B sch em e)
th at allo w ed
su rg ical treatm en t.
H o w ev er,
th is p atien t h ad lo cal recu rren ce
3 m o n th s later.
2A 5 0 % fiv e-y ear
su rv iv al
rate is rep o rted
b y S m ith
in p atien ts
w ith sacral lesio n s.1 4 In th e Q u een E lizab eth
H o sp ital
series n o d ifferen ce
w as fo u n d b etw een p atien ts
su b m itted
to p alliativ e
rad iatio n th erap y an d th o se w ith o u t
treatm en t.4 T h e sam e au th o r rep o rted a 5 -y ear su rv iv al rate
b etw een 5 0 % an d 6 0 % fo r cases su b m itted to su rg ery w ith
ad eq u ate
In arg in s fo llo w ed
b y rad iatio n
th erap y , 2 5 % fo r
cases su b m itted
to p alliativ e
rad iatio n
th erap y
an d 1 5 %
fo r p atien ts
su b m itted
to p alliativ e
su rg ery .4
K eisch
rep o rted a 5 -y ear su rv iv al rate o f 1 0 0 % fo r cases su b m itted
to su rg ery
w ith ad eq u ate
m arg in s
an d rad iatio n
th erap y ,
Table 2
Five-year overall survival reported by some authors
for chordomas
Figure
3 - C h o r d o m a c e lls w it h c y t o p la s m a t ic v a c u o liz a t io n . C la s s ic a l p h y s a lif e r o u s la r g e c e ll o n t h e r ig h t s id e ( h e m a t o x y lin -e o s in s t a in m a g n if ic a t io n x 4 0 0 ) ./
Figure 4 -
N e c r o p s y s p e c im e n o f a la r g e s a c r o c o c c y g e a l c h o r d o m a in v a d in g a d ja c e n t s t r u c t u r e s o f t h e p e lv is , d e s t r o y in g e q u in e t a il.A u t h o r
K e is c h e t a l( 1 9 9 1 )
R ic h e t a l( 1 9 8 5 )
S m it h e t a l( 1 9 8 7 )
C h e t iy a w a r d a n a ( 1 9 8 4 )
A z z a r e lli e t a l( 1 9 8 8 )
T h is s e r ie s
# c a s e s
2 1
4 8
6 0
69
3 3
2 4
5y r ( % )
74
5 0
5 0
4 0
30
4 .2
an d 6 8 % fo r cases su b m itted
o n ly to su rg ery w ith ad eq u ate
m arg in s.
loT h is ex cellen t resu lt
in d icates
th e n ecessity
o f
co m b in ed
treatm en t
an d early
d iag n o sis.
T h e fiv e y ear
o v erall
su rv iv al
rep o rted
b y so m e au th o rs
are sh o w n
in
T ab le 2 .
L O P E S , A . ; R O S S I , 8 . M . ; S I L V E I R A , C . R . S . & A L V E S , A . C . - C h o r d o m a : r e t r o s p e c t iv e a n a ly s is o f 2 4 c a s e s
T he 4.2% five year survival obtained
in our series is
an indication
of the advanced
stage of the disease
and
recurrence
after previous
inadequate
treatm ent.
Since chordom as
are a slow -grow ing
and rare tum ors,
m ultidisciplinary
approach and early diagnosis are required
for adequate
treatm ent.
RESUMO
In tro d u c;:a o : 0 co rd o m a
e
u m tu m o r ra ro e d e cre scim e n to le n to , co m a g re ssivid a d e lo ca l e lo ca liza ya o p re fe re n cia l n a co lu n a ve rte b ra l. O b je tivo : 0 o b je tivo p rin cip a l d e ste e stu d oe
a n a lisa r a h ist6 ria n a tu ra l e o s re su lta d o s d e tra ta m e n to d o s co rd o m a s. M e to d o lo g ia : E stee
u m e stu d o re tro sp e ctivo re a liza d o e n tre 1 9 5 3 e 1 9 9 3 . M a te ria l e M e to d o s: A id a d e va rio u d e 2 a 8 6 a n o s (m e d ia = 3 4 ,5 ). D o ze p a cie n te s e ra m d o se xo m a scu lin o e 1 2 d o se xo fe m in in o . A 1 0 ca liza Q a o d o tu m o r e ra : re g ia o sa cra l e m 2 0 ca so s, ca b e ya e p e sco yo e m 3 ca so s e fo ra d a co l u n a e m u m ca so . R e su lta d o s: 0 tra ta m e n to , so zin h o o u co m b in a d o , fo i ciru rg ia , ra d io te ra p ia e q u im io te ra p ia . A so b re vid a p a ra p a cie n te s co m le s6 e s n o sa cro va rio u d e 4 a 1 1 9 m e se s, d e sd e a d a ta d o s sin to m a s. A so b re vid a g lo b a l e m 5 a n o s fo i d e 4 ,2 % . C o n clu sa o : C o rd o m ae
u m tu m o r ra ro e d e cre scim e n to le n to , co m u m a a b o rd a g e m te ra p e u tica ciru rg ica d iffcil d e vid o a su a lo ca liza ya o p re fe re n cia l n o sa cro e re su lta d o s p o b re s co m ra d io te ra p ia o u q u im io te ra p ia , p rin cip a lm e n te e m p a cie n te s co m d o e n ya a va n r;a d a .REFERENCES
I. A m endola M A , P over E , M cL achey K D C hordom a: role of
radiation therapy. R adiology 1986; 158:839-43.
2. A zzarelli A , Q uagliuolo V , C erasolli S et al C hordom a:
natural history and treatm ent results in 33 cases. J S urg O ncol
1985;37: 185-91.
3. B ethke K P , N eifeld JP , L aw rence Jr. W D iagnosis and m anagem ent
of sacrococcygeal chordom a. J S urg O ncol 1991; 48: 232-8.
4. C hetiyaw ardana A D . C hordom a: results of treatm ent. C lin
R adiol 1984;35: 159-61.
5. C hetty R , L evin C V , K alan M R C hordom a: a 20-year
clinicopathologic review of the experience at G roote S chuur
H ospital, C ape T ow n. J S urg O ncol 1991 ;46:261-4.
6. D ahlin D C , M acC arty C S . C hordom a: a study of fifty-nine
cases. C ancer, 1952;5: 1170-8.
7. G entil F C , C oley B L S acrococcygeal chordom a. A nn S urg
1948; 127:432-55.
8. H effelfinger M J, D ahlin D C , M acC arthy C S et al.
C hordom as and cartilaginous tum ors at the skull base.
C ancer 1973;32:410-20.
9. H iginbotham N L , P hillips R F , F arr H W et al. C hordom a:
thirty-five-year study at M em orial H ospital. C ancer
1967;20: 1841-50.
10. K eisch M E , G arcia D M , S hibuya R B . R etrospective
long-term follow -up analysis in 21 patients w ith chordom a of
various sits treated at a single institution. J N eurograf
1991 ;75:374-7.
II.P earlm an A W , S igh R K , H oppenstein R et al C hordom a:
com bined therapy w ith radiation and surgery: case repO lt and
new operative approach. B ull H osp Joint D is 1972;33:47-57.
12. R ibbert H . U eber die E cchordosis physalifora
spheno-occipitalis. Z entralbl A llg P athol 1894;5:457-61.
13. R ich T A , S chiller A , S uit H D , M ankin H J. C linica and
pathologic review of 48 cases of chordom a. C ancer
1985;56:182-7.
14. S m ith J, L udw ig R L , M arcove R C S acrococcygeal
C hordom a. S keletal R adiol 1987; 16: 37-44.
15. S undaresan N , G alich JH , C hu F C H et al. S pinal chordom as.
J N eurosurg 1979;50:312-9.
16. T ew fik H H , M cG innis W L , N ordstrom D G et al.
C hordom a: evaluation of clinical behavior and treatm ent
m odalities. Int J R adiat O ncol B ioi P hys 1977;2:959-62.
17. V irchow R L K . U ntersuchungen liber die E ntw ickenlung
des S chadelgrundes in gesunden und krakhaften
Z ustande und liber den E influs derselben auf
S chadelform , G esichtsbildung und gehirnbau - B erlim ,
G . R eim er, 1957. p.128.
18. V olpe R , M azabraud A A clinicopathologic review of 25
cases of chordom a. A m J S urg P athol 1983;7: 161-70.
20. W elinger C L . R achidial C hordom a: III R eview of the
literature since 1960. R ev R hum M al O steoartic
1975;42:287-95.
