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[JOURNAL OF HEAD & NECK PHYSICIANS
AND SURGEONS]
Case report
TUMORAL CALCINOSIS-LIKE LESION IN THE NASAL
SEPTUM IN END-STAGE RENAL DISEASE
M!"# P$%$&&' I"()#& M*$ V$&&**#&
1
' C&* S&+
2
' P#,*- R.$)
3
' O/* L0.#%"#
1
'
C*$+"-2& L-3#)-C-*"$4-
5
Department of Otorhinolaryngology, Department of Pathology, Department of Radiology
Department of Nephrology.
Puerta de Hierro University Hospital, Madrid Spain
I%"*-,.6"$-%
A tumoral alinosis!li"e
lesion or metastati
alifiation is a pathologi
ondition harateri#ed $y
deposits of alium%
phosphate rystals &ithin
other&ise normal soft tissue
as a result of
hyperphosphatemia &ith or
&ithout onurrent
hyperalemia. 'here is a
hereditary and an a(uired
possi$le etiology )*+
Metastati alifiation is a
&ell "no& ompliation of
end%stage renal disease,
ho&ever the presene of
these deposits presented
linially as a nasal lesion is
e,tremely rare )-, .+
C+# R#3-*"
A /0!year%old male &ith
end%stage renal disease, a
history of hroni renal
failure and aute myoardial
infartion 1&ith stent
implantation2, presented
himself &ith a s&elling of
the anterior nasal septum.
Sine May -333 he &as
treated &ith hemodialysis.
'he hemodilutor that is used
during these proedures is
fragmented heparin. During
Cite: Mayte P, Ithzel M, Clara S, Pedro R, Omar L, Cristobal LC, Tumoral calcinosis like lesin
in the nasal septum in end sta!e renal disease, "ournal o# $ead and %eck physicians and
sur!eons, &'()*(+(,:(-.&/
19
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[JOURNAL OF HEAD & NECK PHYSICIANS
AND SURGEONS]
4uly -33*, the patient slo&ly
developed a painless
s&elling in the nasal septum
that progressed more rapidly
over the last day of that
month. On e,amination, the
s&elling &as a round lesion
of appro,imately - m in
diameter and affeted the
artilaginous anterior septum
in $oth sides. 'he remaining
nasal septum did not sho&
any a$normalities. On the
surfae the lesion had &hite
points and &as slightly
flutuant upon palpation.
Although the patient did not
have fever, a nasal a$sess
&as suspeted and the lesion
&as e,plored under loal
anesthesia 1lidoaine -5
adrenaline *6*333.3332. A
aseous material &as
evauated from multiloular
tissue. 7ultures of this
material remained sterile.
Radiologi studies &ith 7'
san revealed a foal lesion
in the anterior nasal septum
&ith multiple, rounded
opaities separated $y
radioluent lines in a laminar
pattern. 18igure *a2
'he tissue from the $iopsy
sho&ed a entral mass of
amorphous and alified
material inside a dense
fi$rous tissue and $ordered
$y a nodular proliferation of
marophages and
multinulear ells. 18igure
*$2
9a$oratory findings
presented a severe seondary
hyperparathyroidism &ith
hyperphosphatemia $ut no
evidene of inreased
alium levels: a
'ehnetium%;; parathyroid
san sho&ed a hyperplasia of
$oth inferior parathyroid
glands.
Radiologial imaging of
large $ones revealed no
evidene of alinosis
involving either their soft or
periartiular tissues.
'he patient had a (ui"
reovery after the operation
and intravenous vitamin D
&as administered to ontrol
hyperparathyroidism and
later on a parathyroidetomy
&as performed. During
Cite: Mayte P, Ithzel M, Clara S, Pedro R, Omar L, Cristobal LC, Tumoral calcinosis like lesin
in the nasal septum in end sta!e renal disease, "ournal o# $ead and %eck physicians and
sur!eons, &'()*(+(,:(-.&/
20
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I/JUNE-DEC 2013
[JOURNAL OF HEAD & NECK PHYSICIANS
AND SURGEONS]
follo&%up one year later, the
patient presented a minimal
lesion &ithout any signs of
ne& deposits in the septal
area. At present the patient is
alive &ithout nasal
symptoms.
D$+6.++$-%
'umoral alinosis is a
ongenital or a(uired defet
in alium meta$olism that
in the first ase is inherited
aording to a dominant or
reessive pattern. <t is
harateri#ed $y tumor%li"e
periartiular deposits of
alium phosphate that are
found foremost in the
regions of the hip, shoulder
and el$o& )/+. Some other
sites of metastasi alium%
phosphate deposition that
have $een reported are
"idneys, lungs, liver, vessels,
stomah, visera , dura,
myoard, pleura,
on=untiva, voal ords and
$reasts )-+. <n ase of $eing
an a(uired defet, one of
the possi$ilities that have to
$e onsidered is it $eing a
rare ompliation of dialysis
)/+.
Morphologially idential
periartiular lesions may $e
enountered in patients &ith
hroni renal disease and
seondary
hyperparathyroidism, $ut
most of the patients &ith
these lesions are older than
those &ith tumoral alinosis
)*, /+. 'hey are desri$ed as
enapsulate masses,
multilo$ulated of different
possi$le si#es oupied $y a
reamy dense yello&ish
material of alium
omponents. Primary
lesions are usually of smaller
si#e than the ones found in
patients on dialysis.
'hree types of tumoral
alinosis have $een
desri$ed. 'ype * is a
tumoral alinosis not related
to disorders of phosphate or
alium meta$olism. <s is
also "no& as primary
normophosphatemi tumoral
alinosis. <t usually affets
young people, $eing only a
single lesion &ith lo&
Cite: Mayte P, Ithzel M, Clara S, Pedro R, Omar L, Cristobal LC, Tumoral calcinosis like lesin
in the nasal septum in end sta!e renal disease, "ournal o# $ead and %eck physicians and
sur!eons, &'()*(+(,:(-.&/
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[JOURNAL OF HEAD & NECK PHYSICIANS
AND SURGEONS]
reurrene rate after
omplete e,ision. 'ype - is
thought to involve a defet in
phosphate resorption
produing elevated serum
phosphate &ith normal
alium levels. 'his type
"no&n also as a familial
hyperphosphatemi tumoral
alinosis may affet teeth,
$lood vessels, ranium and
diaphysis and reurrene is
not rare. Patients &ith type .
tumoral alinosis have an
underlying disease suh as
hroni renal failure &ith
seondary
hyperparathyroidism,
hypervitaminosis D, mil"%
al"ali syndrome and $one
destrution, &hih lead to
soft tissue alifiation )/+.
'here are also tumoral
alinosis!li"e lesions and
vasular alifiations
assoiated &ith
hyperphosphatemia in
patients &ith end%stage renal
disease undergoing dialysis
)/, >+. 'he presene of
alium phosphate deposits
in dialysis patients &ith end%
stage renal disease indiates
that plasma phosphate levels
e,eed the preipitation level
as a onse(uene of
hyperparathyroidism.
?esides hyperphosphatemia
&ith a onse(uent inrease in
the alium%phosphate
produt 1@ 032 and seondary
hyperparathyroidism, it may
also $e due and a dynami
osteopathy preipitated $y
e,essive vitamin D and
alium supplementation,
e,posure to aluminum or
parathyroidetomy )A+.
8ormation of alium%
phosphate deposits an also
$e failitated $y loal fators
suh as tissue hypo,ia,
elevation of tissue pH,
presene of uremi to,ins
and loal trauma )-+. <n this
ase the patient an $e
regarded as suffering from
hroni uremia that an also
elevate susepti$ility for
infetions.
Repeated miro%trauma an
ontri$ute to the formation
of the alium%phosphate
deposits in the s"in, or in the
Cite: Mayte P, Ithzel M, Clara S, Pedro R, Omar L, Cristobal LC, Tumoral calcinosis like lesin
in the nasal septum in end sta!e renal disease, "ournal o# $ead and %eck physicians and
sur!eons, &'()*(+(,:(-.&/
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[JOURNAL OF HEAD & NECK PHYSICIANS
AND SURGEONS]
nasal vesti$ule 1nose%
pi"ing2 )/, 0+. Up to no&
this ase report is only the
third e,ample &ith nasal
septum involvement in end%
stage renal disease )-,.,/+.
'here is ho&ever a report of
a alifiation of the nasal
artilage in an infant &ho
&as e,posed to &arfarin
throughout pregnany and
had &arfarin em$ryopathy. <t
supports the hypothesis that
&arfarin inhi$its Bitamin C%
dependent protein that
prevent the alifiation of
artilage )>+. <n our ase,
fragmented heparin is used
in hemodialysis, ho&ever
heparin does not interfere
&ith vitamin C dependent
proteins.
Radiologi imaging may
support the linial
diagnosis. Plain radiographs
sho& a alified
homogeneous mass, &ith a
multilo$ulated appearane.
<n a 7' san a septated mass
&ith fluid levels may $e
visi$le and in MR< sans,
signal is lo& on '* images
and mi,ed on '- images
&ith $oth high signal related
to edema and lo& signal
from alifi deposits.
Histologially, it is desri$ed
as alified material
surrounded $y
multinuleated giant ells
and mononulear ells &hih
are 7DAD and tartrate%
resistant aidi phosphatase
1'RAP2 and reeptor
ativator of nulear "appa
$eta 1RANC2 positive )D+.
Some differential diagnosis
inludes6 osteosaromas,
ondrosaromas, synovial
saromas, myositis
ossifians, dermatomyositis,
alinosis irunsripta,
alifi tendinitis and
heterotopi ossifiation )/,A+.
Heterotopi alifiation
may $e found aompanying
tumoral alinosis )D+.
Ris" fators for progressive
alifiation in patients &ith
hroni "idney disease may
inlude age, duration of
dyalisis, inflammation,
dia$etes mellitus, early
Cite: Mayte P, Ithzel M, Clara S, Pedro R, Omar L, Cristobal LC, Tumoral calcinosis like lesin
in the nasal septum in end sta!e renal disease, "ournal o# $ead and %eck physicians and
sur!eons, &'()*(+(,:(-.&/
23
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[JOURNAL OF HEAD & NECK PHYSICIANS
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hyperphosphatemia and an
inappropriate alium load
);+.
'reatment must $e aimed on
reduing the 7a , P produt.
One option inludes the use
of lo&%alium dialysate to
help learing the alifi
deposits, $ut this measure
may e,aer$ate the
seondary
hyperparathyroidism if large
amounts of alium are lost
over long periods. A lo&%
phosphate diet, stopping the
vitamin D, and alium
supplements may also have
some positive results )0,*3+.
<f peritoneal dialysis is the
treatment $eing reeived it
should $e hanged to
hemodialysis, &hih has less
ris" of having this
ompliation. One of the
most important treatment
measures is performing a
su$total parathyroidetomy,
&hih is onsidered one of
the first options &hen there
is a severe
hyperparathyroidism.
Another surgial possi$ility,
&ith no dou$t, inludes a
renal transplantation ausing
reversal of alinosis &ith no
ris" of reurrene. ?ut in
our ase as &ell as in other
ases of small deposits, the
omplete e,ision is the
treatment of hoie, even
though loal reurrene is
not unommon )D+.
Monitoring the 7a , P and
P'H levels is vital in patients
undergoing dialysis )A+. <t is
important to onsider that
moreover, surgial trauma
may stimulate further
alifiation )/+.
Ee &ould li"e to mention
other therapeuti onepts
for e,traosseous alifiation
that are emerging. Sodium
thiosulfate is a linially
esta$lished helator antidote
against yanide into,iations
and it is also used to treat a
deleterious disease &hih
auses severe pain and
neroti soft%tissue
ulerations due to
alifiation of $lood vessels
and adipose tissue "no&n as
aliphyla,is );,*3+. One of
Cite: Mayte P, Ithzel M, Clara S, Pedro R, Omar L, Cristobal LC, Tumoral calcinosis like lesin
in the nasal septum in end sta!e renal disease, "ournal o# $ead and %eck physicians and
sur!eons, &'()*(+(,:(-.&/
24
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[JOURNAL OF HEAD & NECK PHYSICIANS
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the hypothesis of its mode of
ation is that its molar
solu$ility $eing more than
that of alium phosphate
may potenially interrumpt
alium phosphate
preipitaion in to soft tissue.
Nevertheless, its mode of
ation and optimal
administration route are still
$eing studied.
7alimimetis ontrol
suessfully seondary
hyperparathyroidism in
patients &ith end stage renal
disease and also lo&er serum
levels of $oth phosphate and
alium. Still there are
studies $eing held on to
prove &hether alimimetis
are assoiated &ith
improvement of meaningful
patient outomes. Strategies
to regress or stop the
progress of e,traosseous
alifiation are &arranted.
9oading the system &ith
alium and phosphate may
have very negative outomes
and $e of real danger, so the
ation of novel drugs and
other interventions that target
learane mehanisms must
$e profoundly analy#ed and
studied );+.
Although our patient has a
high ris" of developing
metastati alium deposits,
the presene of this
alinosis in the nasal
septum &as a surprise. An
early and ade(uate diagnosis
$y radiology and e,ploration
an avoid unneessary
anti$ioti treatment and
hospitali#ation. Some
ompliations may inlude
fistula formation and
infetion, &hih may
produe systemi symptoms.
SUMMARY
F,traosseous tissue
alifiation in patients &ith
advaned hroni "idney
disease undergoing dialysis
is a omple,, highly
prevalent proess.
Pathologial alifiation
sites may vary, $ut the
loali#ation in the nasal
septum is very rare. <t is
aused $y inreased alium
phosphate produt in the
serum, leading to soft tissue
Cite: Mayte P, Ithzel M, Clara S, Pedro R, Omar L, Cristobal LC, Tumoral calcinosis like lesin
in the nasal septum in end sta!e renal disease, "ournal o# $ead and %eck physicians and
sur!eons, &'()*(+(,:(-.&/
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alifiation. Radiologi
imaging help onfirm this
ondition. Ade(uate ontrol
of serum alium%phosphate
levels &ill diminish the ris"
of formation or reurrent
deposits in these patients.
REFERENCES
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th
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*.D.
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8e$:-A1-2,*;;;, pp .0;%
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8. Hiramatsu R, U$ara H,
Hayami N, et al.
Ourrene of ne& $one%
li"e tissue formation in
Cite: Mayte P, Ithzel M, Clara S, Pedro R, Omar L, Cristobal LC, Tumoral calcinosis like lesin
in the nasal septum in end sta!e renal disease, "ournal o# $ead and %eck physicians and
sur!eons, &'()*(+(,:(-.&/
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uremi tumoral alinosis.
?one. 8e$: >-1-2, -3*.,
ppAD/%ADD.
9. Cetteler M, Rothe H,
CrKger ' et al.
Mehanisms and
treatment of e,traosseous
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"idney disease. Nat Rev
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10. Heh SM, H&ang S4,
7hen H7. 'reatment of
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Hemodial <nt, Apr:*.1-2,
-33;, pp *A.%*A0
FIGURES
Cite: Mayte P, Ithzel M, Clara S, Pedro R, Omar L, Cristobal LC, Tumoral calcinosis like lesin
in the nasal septum in end sta!e renal disease, "ournal o# $ead and %eck physicians and
sur!eons, &'()*(+(,:(-.&/
27
VOLUME I/ ISSUE
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[JOURNAL OF HEAD & NECK PHYSICIANS
AND SURGEONS]
Cite: Mayte P, Ithzel M, Clara S, Pedro R, Omar L, Cristobal LC, Tumoral calcinosis like lesin
in the nasal septum in end sta!e renal disease, "ournal o# $ead and %eck physicians and
sur!eons, &'()*(+(,:(-.&/
28
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F$7.*# 18 Soft tissue mass &ith e,tensive areas of alifiation
that affets the anterior septal artilage 1A,ial 7' san2
F$7.*# 128 Histopathologi e,amination &ith a tumoral
alinosis in ative phase 1hemato,ylin and eosin stain ,-332
C-%0&$6" -0 $%"#*#+" 9 N$&
A6:%-;&#,7#/#%"+- N$&
S.33-*"- N$&
C-**#+3-%,%6#<
Cite: Mayte P, Ithzel M, Clara S, Pedro R, Omar L, Cristobal LC, Tumoral calcinosis like lesin
in the nasal septum in end sta!e renal disease, "ournal o# $ead and %eck physicians and
sur!eons, &'()*(+(,:(-.&/
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Mayte Pinilla
7LSan Restituto
0-7, A%/ -D3.;
Madrid, Spain
Phone6 M ./ A-;D*3*./
Cite: Mayte P, Ithzel M, Clara S, Pedro R, Omar L, Cristobal LC, Tumoral calcinosis like lesin
in the nasal septum in end sta!e renal disease, "ournal o# $ead and %eck physicians and
sur!eons, &'()*(+(,:(-.&/
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