Rare disease

RosaiDorfman disease presenting as a kidney tumour

Smarajit Roy
1
, Edward Kweka
2
, Preeti Chaudhri
3
and Jorge Clavijo
4

1
E! "S #ru$t, %rology, ull Royal &n'irmary, ull, %3 2J(, %K
2
")* "S #ru$t, Radiology, +P,- o$.ital, *rim$/y, +"33 201, %K
3
Pathlink, Pathology, )in2oln County o$.ital, )"2 34!, %K
4
")* "S #ru$t, %rology, +P,- o$.ital, *rim$/y, +"33 201, %K
Corre$.onden2e to5
roy$marajit6yahoo72om
S%881R!
1n 91:year:old Cau2a$ian lady $tudied 'or anaemia .re$ented

with a large $olid enhan2ing
ma$$ ari$ing 'rom the hilum o'

the le't kidney on 2om.uted tomogra.hy $2an7 1 la.aro$2o.i2

le't radi2al ne.hre2tomy wa$ done through a retro.eritoneal

a..roa2h7 8i2ro$2o.y and
hi$to2hemi$try 2la$$i'ied it a$ Ro$ai;+or'man

di$ea$e though there wa$ neither
lym.hadeno.athy anywhere nor

lym.ho2yto$i$7 &t wa$ a very unu$ual urologi2al .re$entation

o' a rare di$ea$e7

01CK*R,%"+
-e want to re.ort a 2a$e on i$olated .re$entation o' Ro$ai;+or'man

di$ea$e, whi2h i$ a rare
di$ea$e and rarely .re$ent$ without

any lym.hadeno.athy7 ,nly one $u2h 2a$e re.ort, where
Ro$ai;+or'man

di$ea$e .re$ented without any lym.hadeno.athy and any a$$o2iated

urogenital tra2t .athology, wa$ 'ound 'ollowing an e<ten$ive

$ear2h o' the internet7

C1SE PRESE"#1#&,"
1n 91:year:old, non:$moker Cau2a$ian lady .re$ented with e.i$ode$

o' .er re2tal /leeding
'ollowed /y anaemia7 She wa$ known to

have hy.erten$ion, i$2haemi2 heart di$ea$e, a2=uired
hy.othyroidi$m,

gout, a2tini2 kerato$i$, diverti2ulo$i$, 8eniere>$ di$ea$e

and hearing
im.airment7 She had a .a$t hi$tory o' a..ende2tomy

and 2hole2y$te2tomy7 er medi2ation$
were5 allo.urinol, ome.ra?ole,

atenolol, rami.ril and ):thyro<ine7 #here wa$ 're$h /lood on

.er re2tal e<amination /ut other 2lini2al e<amination$ were

normal7

&"@ES#&*1#&,"S
Colono$2o.y revealed no a2tive .athology7 0lood te$t revealed

rai$ed $erum 2al2ium A2793
mmolBlC and rai$ed erythro2yte $edimentation

rate AESRC A29 mm a'ter the 'ir$t hourC7 %rine
2ytology revealed

no malignant 2ell$ /ut rai$ed white 2ell 2ount, though mi2ro/iologi2al

analy$i$ o' her urine revealed no a2tive in'e2tion7

1$ a .art o' the inve$tigation 'or anaemia, an a/dominal ultra$ound

$2an wa$ done that
in2identally di$2overed a $.a2e:o22u.ying

le$ion in her le't kidney mea$uring a/out 3D mm x
3E mm x 3E

mm7

#he 2om.uted tomogra.hy AC#C $2an revealed a large A3E mm x

43 mm diameterC $olid ma$$
ari$ing 'rom the hilum o' the le't

kidney A 'ig$ 1 and 2C7 #he ma$$ had a well de'ined margin
and

2au$ed di$.la2ement o' the /ran2he$ o' the renal ve$$el$ without

en2a$ement7 %ni'orm
.o$t:&@ 2ontra$t enhan2ement wa$ demon$trated7

#here were no enlarged lo2o:regional
lym.hadeno.athy and the

.eri:renal 'at wa$ 2lear7 #he 2ontralateral kidney wa$ normal7

"o
lym.hadeno.athy or meta$ta$i$ wa$ dete2ted in the 2he$t or

a/domen7

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Figure 1 Com.uted tomogra.hy $2an a/domen 15 23 $ .o$t:inje2tion $erie$5 a large 3E mm
$olid ma$$ 2entrally within the le't kidney, whi2h o/literate$ the renal hilum and di$.la2e$ the
renal ve$$el$H the margin$ are well de'ined and the attenuation o' the ma$$ i$ homogenou$
mea$uring 3F % Ae<.e2ted non:enhan2ed ti$$ue mea$ure 4E %CH there i$ no hydrone.hro$i$
and e<trarenal $o't ti$$ue$ are normal7

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Figure 2 Com.uted tomogra.hy $2an a/domen 25 DE $ .o$t:inje2tion $erie$5 the 2entral renal
ma$$ ha$ 2ontinued to enhan2e to /etween FE and IE % and the enhan2ement ha$ remained
homogenou$7

#he /one $2an did not $how any a2tive 'o2u$ o' meta$ta$i$7

+&JJERE"#&1) +&1*",S&S
Renal tumour, mo$t likely renal 2ell 2ar2inoma7

#RE1#8E"#
)a.aro$2o.i2 le't radi2al ne.hre2tomy wa$ done through a retro.eritoneal

a..roa2h
1
7 #hree
tro2ar$ were u$ed and the /agged $.e2imen wa$

retrieved /y minimal e<ten$ion o' the
anterior;in'erior

tro2ar in2i$ion without mor2ellation7 Re2overy wa$ unevent'ul7

,%#C,8E 1"+ J,)),-:%P
#he .atient>$ re2overy wa$ unevent'ul and $he wa$ ready

to go home a'ter D day$7
%n'ortunately $he $u''ered an a2ute

myo2ardial in'ar2tion within 4 week$ a'ter $urgery and
died

2 week$ later7

8a2ro$2o.i2ally the kidney mea$ured 13E mm x FE mm x 3E mm7

#he e<ternal $ur'a2e wa$
unremarka/le7 #he 2ut $ur'a2e $howed

an endo.hyti2 'le$hy white tumour in the mid kidney7
#he tumour

did not /rea2h the 2a.$ule and mea$ured 3E mm x D3mm x 13mm7

8i2ro$2o.y $howed a well 2ir2um$2ri/ed le$ion 2om.o$ed o' a

$heet o' hi$tio2yti2 2ell$ with
a/undant granular eo$ino.hili2

2yto.la$m and well de'ined 2yto.la$mi2 /order$ A 'ig$ 3 and
4C7

#he nu2lei were too varia/le in $i?e, round, ve$i2ular and with

$mooth nu2lear 2ontour$7 1
.rominent /a2kground o' 2hroni2 in'lammation,

.redominantly lym.ho.la$mo2yti2 with
inter$.er$ed lym.hoid 'olli2le$,

wa$ $een7 #here were e<ten$ive em.eri.ole$i$ Ahi$tio2yte$
with

intra2yto.la$mi2 lym.ho2yte$C7 "o 8i2haeli$;*utman /ody

wa$ 'ound7

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Figure 3 8i2ro$2o.y $lide 15 a 2lu$ter o' hi$tio2yte$ with the 2hara2teri$ti2 em.eri.ole$i$7

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Figure 4 8i2ro$2o.y $lide 25 di''u$e $heet$ o' hi$tio2yte$ in a /a2kground o' mi<ed
in'lammatory 2ell in'iltrate7

&mmunohi$to2hemi$try revealed that the hi$tio2yti2 2ell$ were

.o$itive 'or C+D9, C+1D3 and
S1EE, /ut negative 'or 2ytokeratin$

and C+117 #he 2om/ined hi$tology and
immunohi$to2hemi$try 'avoured

the diagno$i$ o' Ro$ai;+or'man di$ea$e rather than
granulomatou$

.yelone.hriti$, /enign hi$tio2yti2 .roli'eration or malako.lakia7

+&SC%SS&,"
Clini2ally, the .atient did not have $igni'i2ant lym.hadeno.athy

or lym.ho2yto$i$7 &n'e2tiou$
aetiology o' the ma$$ wa$ al$o

e<2luded /y inve$tigation$7 #he age o' the .atient wa$ not a

ty.i2al age grou. 'or .re$entation o' Ro$ai;+or'man di$ea$e7

#he ESR wa$ rai$ed, a$ i$ $een
/oth in renal malignan2y and

hi$tio2yto$i$7

Radiologi2ally $ome o' the C# 'eature$ were unu$ual 'or a kidney

tumour7 #he$e in2luded a
well de'ined margin, uni'orm 2ontra$t

enhan2ement and ve$$el di$.la2ement a$ o..o$ed to
en2a$ement7

0ut $everal renal tumour$ al$o .re$ent with the$e 'eature$7

Jew o' the mi2ro$2o.i2 'eature$ were unu$ual or une<.e2ted 'or

Ro$ai;+or'man di$ea$eK'or
e<am.le, .o.ulation o'

$.indle 2ell$, the low degree o' nu2lear .leomor.hi$m and the

$heet$
o' 'oamy hi$tio2yte$7 Phago2yto$i$ o' the lym.ho2yte$

wa$ mu2h le$$ and thi$ .erha.$ wa$
due to le$$ availa/le lym.ho2yte$

in the kidney than the lym.h node$7 #he literature on renal

Ro$ai;+or'man di$ea$e Awithout lym.hadeno.athyC i$ $2ar2e,

$o the range o' mor.hologi2
variation i$ not well e$ta/li$hed7

#he aetiology o' thi$ di$ea$e i$ not known7 #he hi$tio2yti2

.roli'eration i$ .oly2lonal and
there'ore non:neo.la$ti27 &t

i$ u$ually $el':limiting with a tenden2y 'or $.ontaneou$ re$olution7
Some 2a$e$ $how more aggre$$ive /ehaviour with .er$i$ten2e and

rarely death7 %$ually thi$
di$ea$e i$ limited to lym.h node$,

/ut e<tra:nodal 2a$e$ have /een re.orted
2
7 &n 2on2lu$ion,
renal

Ro$ai;+or'man di$ea$e i$ a rare e<tra:nodal 'orm o' $inu$

hi$tio2yto$i$ with u$ually
ma$$ive lym.hadeno.athy, /ut it 2an

o22ur in i$olation a$ in thi$ 2a$e7 &n thi$ .atient it
mimi2ked

a malignant renal neo.la$m on 2lini2al and radiologi2al e<amination,

/ut wa$
'inally 2la$$i'ied on .athologi2al e<amination7

)E1R"&"* P,&"#S
@ery rare .re$entation o' a very rare di$ea$e
+r J " E/le and +r 1 ,ra?i, +e.artment o' Pathology, &ndiana.oli$,

%S17 1$ Ro$ai +or'man
+i$ea$e i$ a very rare di$order and the

.re$entation i$ very unu$ual, the $.e2imen wa$ $ent to
+r E/le

and +r ,ra?i to get a $e2ond o.inion a/out the diagno$i$ and

they al$o agreed with
the diagno$i$7

Competing interests: none7

REJERE"CES
17 *ill, &S, L Ra$$weiler, JJ7 Retro.eritoneo$2o.i2 renal $urgery5 our a..roa2h7 %rology
1IIIH 345 F34;97M8edlineN
27 1'?al, 8, 0ae?:*iangre2o, 1, al Ja$er, 1", L ,nuora, @C7 %nu$ual /ilateral renal
hi$tio2yto$i$7 E<tranodal variant o' Ro$ai:+or'man di$ea$e7 1r2h Pathol )a/ 8ed
1II2H 11D5 13DD;F7M8edlineN