R icardo E desio A m orim S antos D iniz, Jose G oldenberg,
Jose C arlos M edina de C arvalho, C arlos E duardo S ales
G om es, E velin D iana G oldenberg, A ngelo S em entille
Lym phom a of unknow n origin located in paravertebral
m uscles: an unusual cause of low back pain in children
T h e a u th o rs re p o rt a c a s e o f a n a d o le s c e n t w ith a p o o rly d iffe re n tia te d ly m p h o m a o f u n k n o w n o rig in lo c a te d a t p a ra v e rte b ra l m u s c le s , w h o s e p re s e n ty s y m p to m w a s lo w b a c k p a in .
U N IT E R M S : L y m p h o m a . B a c k p a in .
IN T R O D U C T IO N
L
o w
u n u su al
b ack
sy m p to m .
p ain
in ch ild ren
It is rarely
an d
o f a p sy ch o g en ic
ad o lescen ts
is an
n atu re
an d its cau se is u su ally
id en tified .
E tio lo g y
m ay b e in fectiv e,
in flam m ato ry ,
trau m atic,
d ev elo p m en tal
d iso rd ers,
d isc p ath o lo g y
o r n eo p lasic
d isease (7 ,8 , 1 4 , 1 6 ).
N eo p lasic
cau ses
o f lu m b ar
p ain in ch ild h o o d
m ay
b e
b en ig n
(o steo id
o steo m a,
b en ig n
o steo b lasto m a,
an eu ry sm al
b o n e
cy st, eo sin o p h ilic
g ran u lo m a);
m alig n an t
p rim ary
tu m o rs
(E w in g s
sarco m a,
o steo g en ic
sarco m a);
m etastatic
tu m o rs
(n eu ro b lasto m a,
W ilm s
tu m o r);
leu k em ia
o r sp in al co rd tu m o rs
(g lio m as,
d erm o id
cy sts,
lip o m a,
in tram ed u llary
cy st) (1 6 ).
N o n -H o d g k in 's
ly m p h o m as
rep resen t
7 to
1 0 % o f
th e
m alig n an t
tu m o rs
in th e first an d seco n d
d ecad es
o f
life (9 ), an d are o n e o f th e m ain m alig n an cies
in ch ild h o o d ,
su rp assed
o n ly
b y
leu k em ia
an d cen tral
n erv o u s
sy stem
tu m o rs
(1 7 ). In v o lv em en t
o f
sk eletal
m u scu latu re
is v ery
A ddress
for correspondence:
R icardo
E desio A m orim
S antos D iniz
H ospital A na C osta de S antos
U niversidade
Federal de S ao P aulo
D isciplina
de R eum atologia
R ua B otucatu,
740 - V ila C lem entino
S ao P aulo -
S P -B rasil-
C E P 04023.062
u n u su al,
m ain ly
w h en
it is th e
p rim ary
site
w h ich
h as
b een d escrib ed
in o n ly a few cases
(5 ,6 ,1 0 ,1 1 ,1 3 ,1 5 ).
In
th is
rep o rt
th e
au th o rs
d escrib e
a p o o rly
d ifferen tiated
ly m p h o cy tic
ly m p h o m a
in th e p arav erteb ral
m u scu latu re
o f an ad o lescen t
w h o se
p resen tin g
sy m p to m
w as lo w b ack
p ain
asso ciated
w ith
n eu ro lo g ic
sig n s
o f
n erv e ro o t co m p ressio n .
C A S E R E P O R T
L .S .P .,
a 1 2 y ear
o ld
cau casian
b o y ,
w ith
a tw o
-m o n th
h isto ry
o f co n tin u o u s
lu m b ar
p ain
w h ich
w as
ag g rav ated
o n
stan d in g
an d reliev ed
b y rest. T h irty
d ay s
later th e p ain w o rsen ed
w ith rad iatio n
to w ard s
th e rig h t
th ig h , rig h t leg , left fo o t an d left leg . P ain w as o f a b u rn in g
n atu re
an d h e h ad p ro g ressiv e
d ifficu lty
in w alk in g .
A t
th is stag e
h e w as ad m itted
fo r
ex am in atio n
to elu cid ate
th e d iag n o sis.
T h ere w as n o m en tio n
o f fev er, w eig h t lo ss,
sw eatin g
o r lo cal in flam m ato ry
sig n s.
O n th e ex am in atio n
h is g en eral
co n d itio n
w as fair,
b reath in g
w as n o rm al,
h e w eig h ed
4 8 k g an d
its h eig h t
w as
1 .5 5
cm ;
B P :
1 1 0
x
8 0
m m H g .
N o rm al
card io p u lm o n ary
au scu ltatio n .
H is ab d o m en
w as p ain less,
w ith o u t
o rg an o m eg aly
o r p alp ab le
m asses.
P erip h eral
D IN IZ , R .E A S . - L y m p h o m a o f u n k n o w n o rig in lo c a te d in p a ra v e rte b ra l m u s c le s : a n u n u s u a l c a u s e o f lo w b a c k p a in c h ild re n
lym phadenopathy
w as not observed.
Pulses w ere present
and sym m etric.
The patient had difficulty in w alking, crural
paraparesis
and decreased
patellar
and ankle jerk reflexes.
Lasegue
w as positive
at 30° on the left and 40° on the
right and
he had generalized
hypoaesthesia
of the low er
lim bs.
H em oglobin
w as
13 g/dl, W BC
count
w as 8,000
leucocyte/m m 3
w ith norm al differential
distribution
count
and
516.000
platelet/m m 3.
ESR
w as 34 m m /lst
hour
(W estergreen).
Calcium
and phosphorus
serum
levels,
alkaline
phosphatase,
protein
electrophoresis
and
urinalysis
w ere norm al.
Chest X -ray
w as
norm al.
X -ray
of the lum bosacral
spine
show ed
bony
sclerosis
of
the
left side of the L3 body
w hich
had a large transversal
process and an obscure
left pedicle (fig. 1). Com putorized
tom ography
(CT) (L2-S 1) show ed hypertrophia
of the left
process
and vertebral
body
of L3 w ith
thickening
and
sclerosis
of left pedicle. The rachi canal w as not stenosed.
D uring
surgery
a m ass
w as observed
englobing
the nerve
root bilateraly.
The initial
biopsy specim en
confirm ed
the
presence
of m alignant
neoplasm
and
the tum oral
m ass
w as excised.
The pathologic
report show ed
fragm ents
of
fibrom uscular
tissue containing
solid blocks of neoplastic
lym phocytic
cells. The nuclei
w ere hyperchrom atic
w ith
few m itoses (fig.3), and a poorly differentiated
lym phocytic
lym phom a
infiltrating
the skeletal
m uscle w as diagnosed.
Bony
fragm ents
obtained
at operation
did not show
invasion
by tum or.
O nce the diagnosis
w as m ade, the patient underw ent
intensive
investigation
in order
to identify
a possible
prim ary
focus including
abdom inal
ultrasonography
(U S);
com putorized
tom ography
of the skull, thorax, abdom en
and pelvis; bone m arrow
biopsy
(iliac crista) all of w hich
w ere norm al.
The patient w as treated w ith system ic
chem otherapy
and local radiotherapy,
and after 11 m onths of follow
up
the patient w as free of any clinical m anifestation
of disease.
D IS C U S S IO N
Skeletal
m uscle
involvem ent
by lym phom a
m ay
occur by m etatastic
dissem ination,
by direct invasion from
a lym phnode
or adjacent
bone, or m ore rarely due to a
prim ary
extranodal
lesion (1).
M uscular
involvem ent
w ith
clinical
or radiologic
expression
is .not com m on,
being
found by tom ography
in only 5% of
the 400
cases of non-H odgkin
lym phom a
in the G lazer et al. series
(4) and in 5.4% of the 110 cases
F ig u r e 1 - X - r a y o f lu m b a r s p in e s h o w in g b o n e s c le r o s is o f th e le ft s id e o f th e L 3 v e r te b r a l b o d y , w h ic h h a d a la r g e tr a n s v e r s a l p r o c e s s .
analyzed
by
N ew al
(12).
H ow ever
m icroscopic
involvem ent
can be m ore
frequent,
especially
in those
cases
w ith
w idespread
system ic
disease
as show n
by
Buerger
and
M onteleone
(2) w ho
noted
m icroscopic
m etastatic
foci in 40%
of
the patients
w ho
underw ent
m ultiple
m uscular
biopsies.
O n the other hand, the prim ary
lym phom a
in skeletal
m uscle seem s to be very unusual.
Freem an
et al. (3) in his
series of 1,467 extranodal
lym phom a
cases observed
that
only
6% had their origin
in connecti ve tissue,
although
they did not m ention m uscular involvem ent.
A m ong 7,000
lym phom as
seen at the M ayo Clinic during a period of 10
years
Travis
et
al.
(15)
found
isolated
m uscular
involvem ent
in only eight cases (0.11 % ).
The few cases present
in the Iiterature
(5,10,11,13)
are indicative
of the rarity of this lesion,
as w ell
as the
S a o P a u lo M e d ic a l J o u r n a l/R P M 1 1 3 ( 4 ) : 9 5 3 - 9 5 6 , 1 9 9 5 D IN IZ , R .E .A .S . - L y m p h o m a o f u n k n o w n o r ig in lo c a te d in p a r a v e r te b r a l m u s c le s : a n u n u s u a l
955
Figure 2 -
C o m p u to riz e d to m o g ra p h y o f L 3 s h o w in g h y p e rtro p h iao f th e le ft p ro c e s s a n d v e rte b ra l b o d y , w ith th in c k e n in g a n d
s c le ro s is o f th e le ft p e d ic le .
d ifficu lty
in
co n firm in g
th is
d iag n o sis.
C y to lo g ic
ex am in atio n ,
electro n
m icro sco p y
an d
im m u n o
-h isto c-h em ical
m ark ers
are freq u en tly
n eed ed to accu rately
d istin g u ish
ly m p h o m a
fro m
o th ers sm all cell tu m o rs w h ich
can in v o lv e
th e m u scle
(5 ,6 ).
In m o st o f th e rep o rted cases o f p rim ary
in v o lv em en t
th e affected m u scles are in th e lo w er lim b s (5 ,6 ,1 5 )
lead in g
to an in crease
o f th e so ft tissu es,
d u e to tu m o r
m ass o r
ed em a,
lo calized
p ain an d rarely p aresth esia.
T h e
in v o l
v em en t
o f
p so as
an d
p arav erteb ral
m u scu latu re
as o b serv ed
in th e p resen t case is o ften related
to an ad jacen t
ly m p h ad en o p ath y
o r to a p rim ary
b o n y
lesio n
(1 ).
H o w ev er,
th e p ath o lo g ic
stu d y
o f th e b o n y
sp ecim en s
o b tain ed
d u rin g
th e
su rg ery
as w ell
as a
p o st-o p erativ e
in v estig atio n
u sin g
C T an d
U S d id n o t sh o w
ev id en ce
o f a p rim ary
b o n e tu m o r.
A lth o u g h
b o n y in v o lv em en t
h ad b een su g g ested
b y
th e rad io lo g ical
fin d in g s,
th is w as n o t co n firm ed
b y th e
Figure
3 - P a th o lo g y . H E : fra g m e n ts o f fib ro m u s c u la r tis s u e c o n ta in in t b lo c k s o f n e o p la s tic ly m p h o c y tic c e lls .h isto lo g ical
stu d y m ad e o f th e p ed icle
an d v erteb ral
b o d y .
T h e
o b serv ed
alteratio n s
w ere
a p ro b ab ly
resu lt
o f an
in crease
in th e
lo cal
irrig atio n
d u e
to
th e
ad jacen t
n eo p lasm .
T h e
fin d in g
o f a p rim ary
ly m p h o m a
in sk eletal
m u scle
can b e ex p lain ed
b y th ree
h y p o th esis:
th e tu m o r
m ig h t
h av e
arisen
fro m
an ad en o p ath y
w h ich
w as n o t
lo cated
o r w h ich
h as
su ffered
seco n d ary
reg ressio n ,
h em ato g en ic
o rig in
fro m
an
u n id en tified
fo cu s,
o r
o rig in ated
fro m in tram u scu lar
ly m p h o id
stru ctu re.
In th e d escrib ed
case
th e rap id
p ro g ressio n
o f th e
p ain ,
th e
n eu ro lo g ical
in v o lv em en t
an d
th e
rad io lo g ic
ex am in atio n
su g g ested
a n eo p lastic
cau se. H o w ev er,
o n ly
th e h isto lo g ic
tests
p ro v id ed
th e d iag n o sis
an d en ab led
treatm en t.
T h is
v ery
u n u su al
case
rein fo rces
th e
fact
th at
b ack ach e
in
ch ild ren
an d
ad o lescen ts
sh o u ld
b e
in v estig ated
v ery
th o ro u g h ly .
RESUMO
a s a u to re s d e s c re v e m u m c a s o d e lin fo m a Iin fo c ftic o p o u c o d ife re n c ia d o , lo c a liz a d o n a m u s c u la tu ra p a ra v e rte b ra llo m b a r e m
a d o le s c e n te d o s e x o m a s c u lin o te n d o lo m b a lg ia c o m o a p re s e n ta 9 a o c 1 fn ic a in ic ia l.
D IN IZ , R .E .A .S . - L y m p h o m a o f u n k n o w n o rig in lo c a te d in p a ra v e rte b ra l m u s c le s : a n u n u s u a l c a u s e o f lo w b a c k p a in c h ild re n
956
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