tumoral calcinosis-like lesion in the nasal septum in end-stage renal diseasesis

8/12/2019 TUMORAL CALCINOSIS-LIKE LESION IN THE NASAL SEPTUM IN END-STAGE RENAL DISEASEsis

1/12

VOLUME I/ ISSUE

I/JUNE-DEC 2013

[JOURNAL OF HEAD & NECK PHYSICIANS

AND SURGEONS]

Case report

TUMORAL CALCINOSIS-LIKE LESION IN THE NASALSEPTUM IN END-STAGE RENAL DISEASE

M!"# P$%$' I"()# M*$ V$**#1 ' C* S+2' P#,* R.$)3' O* L.#%"#'

C*$+" L#)-C*"$45

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

etremely rare )-, +

C+# R#*"

A /0!year%old male &ith

end%stage renal disease, ahistory 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


2/12

VOLUME I/ ISSUE

I/JUNE-DEC 2013


AND SURGEONS]

4uly -33*, the patient slo&ly

developed a painless

s&elling in the nasal septumthat progressed more rapidly

over the last day of that

month. On eamination, the

s&elling &as a round lesion

of approimately - 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 eplored under loal

anesthesia 1lidoaine -5

adrenaline *6*333.3332. Aaseous 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 alifiedmaterial 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 &ithhyperphosphatemia $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 noevidene 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



sur!eons, &'()*(+(:(-.&/

20


3/12

VOLUME I/ ISSUE

I/JUNE-DEC 2013


AND SURGEONS]

follo&%up one year later, the

patient presented a minimal

lesion &ithout any signs ofne& 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 thatin the first ase is inherited

aording to a dominant or

reessive pattern.


4/12


5/12


6/12

VOLUME I/ ISSUE

I/JUNE-DEC 2013


AND SURGEONS]

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 eaer$ate the

seondary

hyperparathyroidism if largeamounts 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+.


7/12

VOLUME I/ ISSUE

I/JUNE-DEC 2013


AND SURGEONS]

the hypothesis of its mode of

ation is that its molar

solu$ility $eing more thanthat 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 meaningfulpatient outomes. Strategies

to regress or stop the

progress of etraosseous

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 eploration

an avoid unneessary

anti$ioti treatment andhospitali#ation. Some

ompliations may inlude

fistula formation and

infetion, &hih may

produe systemi symptoms.

SUMMARY

Ftraosseous tissue

alifiation in patients &ithadvaned 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.


8/12

VOLUME I/ ISSUE

I/JUNE-DEC 2013


AND SURGEONS]

alifiation. Radiologi

imaging help onfirm this

ondition. Ade(uate ontrolof serum alium%phosphate

levels &ill diminish the ris"

of formation or reurrent

deposits in these patients.

REFERENCES

1. Sharon E. Eeiss, 4ohn R.

Gold$lum. Soft 'issue

'umors. /thed. St. 9ouis

2. ?eerens A4, Stel HB,

Middel&eerd M4.Metastati

aliumphosphate

deposition in the

mem$ranous nasal septumin end%stage renal disease.

Rhinology, 0,*;;;,*%

*.

3. Ala&i 8, 8reedman PD.Metastati alifiation of

the nasal septum

presenting as an intraoral

mass6 a ase report &ith arevie& of the literature.

Oral Surg Oral Med Oral

Pathol Oral Radiol Fndod,;*, -33*,pp ;%;.

4. Cim S, C&on , Par" 7 etal. 'umoral alinosis in

the nose in a patient &ithsleroderma6 an unusual

site for a rare tumor. 47raniofa Surg. 4ul:-/1/2,-3*, pp */%*//

5. Ho&e AM, 9ipson AH, deSilva M, Ouvrier R,

Ee$ster ES. Severe

ervial displasia and nasal

artilage alifiationfollo&ing prenatal

&arfarin eposure. AM 4

Med Genet,0*, *;;0,;*%

;.

6. ounes M, ?elghali S,Irour%Hassen S, et al.

7omplete reversal of

tumoral alinosis aftersu$total

parathyroidetomy in a

hemodialysis patient.

4oint ?one Spine. Ot:0>1>2,-33, pp3%3;.

7. 7ofan 8, GarJa S,7om$alia A, et al. Uremi

tumoral alinosis in

patients reeivinglongterm hemodialysis

therapy. 4 Rheumatol.

8e$:-1-2,*;;;, pp 0;%>.

8. Hiramatsu R, U$ara ,

Hayami N, et al.

Ourrene of ne& $one%li"e tissue formation in



sur!eons, &'()*(+(:(-.&/

26
http://www.ncbi.nlm.nih.gov/pubmed?term=Younes%20M%5BAuthor%5D&cauthor=true&cauthor_uid=18693124http://www.ncbi.nlm.nih.gov/pubmed?term=Belghali%20S%5BAuthor%5D&cauthor=true&cauthor_uid=18693124http://www.ncbi.nlm.nih.gov/pubmed?term=Zrour-Hassen%20S%5BAuthor%5D&cauthor=true&cauthor_uid=18693124http://www.ncbi.nlm.nih.gov/pubmed?term=Cofan%20F%5BAuthor%5D&cauthor=true&cauthor_uid=9972973http://www.ncbi.nlm.nih.gov/pubmed?term=Garc%C3%ADa%20S%5BAuthor%5D&cauthor=true&cauthor_uid=9972973http://www.ncbi.nlm.nih.gov/pubmed?term=Combalia%20A%5BAuthor%5D&cauthor=true&cauthor_uid=9972973http://www.ncbi.nlm.nih.gov/pubmed?term=Hiramatsu%20R%5BAuthor%5D&cauthor=true&cauthor_uid=23142362http://www.ncbi.nlm.nih.gov/pubmed?term=Ubara%20Y%5BAuthor%5D&cauthor=true&cauthor_uid=23142362http://www.ncbi.nlm.nih.gov/pubmed?term=Hayami%20N%5BAuthor%5D&cauthor=true&cauthor_uid=23142362http://www.ncbi.nlm.nih.gov/pubmed?term=Belghali%20S%5BAuthor%5D&cauthor=true&cauthor_uid=18693124http://www.ncbi.nlm.nih.gov/pubmed?term=Zrour-Hassen%20S%5BAuthor%5D&cauthor=true&cauthor_uid=18693124http://www.ncbi.nlm.nih.gov/pubmed?term=Cofan%20F%5BAuthor%5D&cauthor=true&cauthor_uid=9972973http://www.ncbi.nlm.nih.gov/pubmed?term=Garc%C3%ADa%20S%5BAuthor%5D&cauthor=true&cauthor_uid=9972973http://www.ncbi.nlm.nih.gov/pubmed?term=Combalia%20A%5BAuthor%5D&cauthor=true&cauthor_uid=9972973http://www.ncbi.nlm.nih.gov/pubmed?term=Hiramatsu%20R%5BAuthor%5D&cauthor=true&cauthor_uid=23142362http://www.ncbi.nlm.nih.gov/pubmed?term=Ubara%20Y%5BAuthor%5D&cauthor=true&cauthor_uid=23142362http://www.ncbi.nlm.nih.gov/pubmed?term=Hayami%20N%5BAuthor%5D&cauthor=true&cauthor_uid=23142362http://www.ncbi.nlm.nih.gov/pubmed?term=Younes%20M%5BAuthor%5D&cauthor=true&cauthor_uid=18693124


9/12

VOLUME I/ ISSUE

I/JUNE-DEC 2013


AND SURGEONS]

uremi tumoral alinosis.?one. 8e$: >-1-2, -3*,

pp/%.

9. Cetteler M, Rothe H,

CrKger ' et al.

Mehanisms andtreatment of etraosseous

alifiation in hroni

"idney disease. Nat Rev

Nephrol, 4ul *;:01;2,-3**, pp >3;%>*

10. eh SM, H&ang S4,

7hen H7. 'reatment of

severe metastatialifiation in

hemodialysis patients.

Hemodial


10/12

VOLUME I/ ISSUE

I/JUNE-DEC 2013


AND SURGEONS]



sur!eons, &'()*(+(:(-.&/

28

1 1


11/12

VOLUME I/ ISSUE

I/JUNE-DEC 2013


AND SURGEONS]

F$7.*# 18Soft tissue mass &ith etensive areas of alifiation

that affets the anterior septal artilage 1Aial 7' san2

F$7.*# 18 Histopathologi eamination &ith a tumoral

alinosis in ative phase 1hematoylin and eosin stain -332

C%$6" $%"#*#+" 9 N$

A6:%;#,7##%"+- N$

S.*"- N$

C**#+%,%6#

tumoral calcinosis-like lesion in the nasal septum in end-stage renal diseasesis

Documents