Uremic Tumoral Calcinosis (UTC) is a rare complication of chronic kidney disease on dialysis, characterized by large periarticular calcification. Among some cases, hypercalcemia with no conventional etiologies has been reported. We present a case of UTC in which hypercalcemia occurred after parathyroidectomy and introduction of low-calcium containing dialysate. Work-up of hypercalcemia did not reveal any conventional etiology, but hypercalcemia resolved after debulking of the tumor. This change in serum calcium gives us an insight into the mechanism of hypercalcemia, occasionally seen among cases with UTC

Bornemann, Michael

Fujiyoshi, Akira

Ng, Roland

eVols at University of Hawaii at Manoa

Improved hypercalcemia afterdebulking of Uremic Tumoral Calcinosisin a parathyroidectomized patientThis paper was presented at the2004 Annual Scientific Meeting ofthe Hawaii Chapter ot AmericanCollege of Physicians. The HawaiiMedical Journal published a twopart series of papers presented atthe annual meeting in the September and November 2004 issues.A Thy- ,er-. 0 ra:. r:,-r”‘.l-o-- P “-‘‘“r’4 B’ 7 -.9Correspondence to:Akira Fujivoshi MD1356 Lusitana Street 7th. (Ocr.Honolu.iu, Hi 96813Ph: 808-586-2910Fax: 808-586-7486E-mail: afujivoshi@aoi.comAbstractfiromic TumoraiCaicinosis (UT is a rare comphcatrorofchronic kidneydisease on dialysis, charactenhedbvlarge periarticular calcification. Among some noses,hypercalcemia with no conventional etiologies hasbeen reported. We present a case of UTC in whichhypercaicemia occurredafterparathyroidectomyandintroduction of/ow-calcium containing dia/ysate. Work-up of hypercalcemia did not re veal any conventionaletiology but hyoercalcemia resolved after debuikingof the tumor This change in serum calcium givesus an insight into the mechanism of hypercalcemia.occasionally seen among cases with UTC.IntroductionFTC is a complication of chronic kidney disease ondialysis. Its prevalence appears to have increasedrecently Although histologically benign. FTC isgenerally progressive. may result in significant pain.i mniobilization. skin ulceration’. secondar infectionand e en death’h The pathouenesis of FTC is notuI Iv understood Among some cases. h\ percalcemiaith no conventional etioioeies has been reported. Itis h\pothesized. in those cases, that FTC can he anendogenous suppls of calcium. mobilizing calciuminto serum. To our knowledge, our case is the linereport in the English literature to describe post—exci-sional improvement of hvpercalceniia in FTC.We review the literature regarding the palhogenesisof FTC. its association with hvpercalcemia. andtreatment.Case Reportear—old multiethnic—Ha w’auan woman beganpertoneal dial’ sis in 1993 because of proeresslonof idiopathic elomerulonephritis. She rece\ ed ali ing—related renal transplant in I 997. Hoes e ci. shesuffered a rejection in 1999. and subsequentl startedon heniodial As 3hr3Omin Dialysate: Ca 2.5mEq!L.K 2iimEq/L Dialyzer MCA 160, Blood Flos’ Rate350cclminutej with the (Allowing medications: calcium carbonate ig =400mg of elemental calcium)orally. 3 times a day, calcitriol I meg intravenousl -3 limes a week on each ED, (subsequently switchedto or ul form) Epogen If) 000 units suheutanLoushtimes a week on each HI). Three days after, laboratorydata were as follows: total calcium (Ca) 7.5 mg/dL,phosphate (P)7.4mg!dL, albumin 3.5 g/dL.She was discovered to have a large calcified massaround the right hip in December 2001 (Figure 1) butrefused further evaluation. The laboratory resultsone month prior were as follows: intact parathyroidhormone (iPTH) 390pg!mL (reference range 12.6-53.Spg/rnU. K 5.9mEq/L, Cl 96mEq/L, HCOr 2OmEq/L BEN 92mg/dL, Cr lO.2mg/dL, Ca 10.1 mg/dL, P7.3mg/dL, albumin 3.8 gIdL. alkaline phosphatase(ALK-P) 147 U/L (reference range 39-I l7U/L).Because of increased CaxP product, calcitriol wasdiscontinued, and calcium carbonate was switched tosevelainer 800mg. orally, 3 times a day. Diet restriction. especially phosphate restriction was discussed.Repeat renal transplantation was suggested to thepatient as an option, but she was reluctant becauseof her pre’ious reaction to steroids (i.e. palpitations.and demonic dreams). She was not always compliant to medications and diet restriction. As a result.her indices for sec ondarv hvperparathvroidism didnot improve as represented in the following: i P’l’H306 pv/mL. Ca 9.2 mg/dL. P 7.$nig!clL in December2002.Her pain around the right hip ‘Jowly progressed tothe point where she opted for total pai-athvroidectonivin an attempt to obtain s’ mptomatic relief. Despitethe operation performed in February 2003. her predials ‘Js serum calcium level remained high: Ca II .0mg/dl.. P X.4mg/dL. albumin 3.1 g/dL. ALK—P 17,3[IL. Therefore. beginning Feb..i-tr’ 2003. calciumconcentratIon ofdialvsate xsas loss eredfrom 2.5mEq/I.to 7.UniEq/’L. 3hr 30mm. Dial’sate: Ca 2.OmEq/L.1.)ial icr F70\R. Blood Flow Rate 400cciminuteHoss es ci. the Inass reniained unchanged. and herserumcalcium appeared to es en increase to 11.0 mg/dL..\t that point, an extensive workup was completedfor her hvpercalcernia. The results, including serums itamin I) levels, parathyroidhormonerelated peptide.serum protein electrophoresis and hone scan. wereunrevealing I TahleI). Hypercalcemia appeared to hedue to mobilization of calcium from the large ealciME:t:000LJ000N.AL,. our or., roAr :.:r’122lied mass. Serum calcium levels taken from differentvenous sites laded todemonstrate differences betweenthe sites Table IIn June 2003 the patient presented with a complaintof rght hi p pain after a f’al I and ss as subsequently admuted for pain control. MRI showed a 27x 4x 17 cmcalcified mass with fluid—filled loculations. which ischaracteristic for LTC’, in the soft tissues of the righthip and proximal femur (Ficures 2. 3, Compared tothe pres ious film in 2001. the mass was essentiall\unchanged. The patient continued to have intractablepain refractory to opioids. She elected to undergo anexcision of the mass because of the pain. Pre-operativecore needle biopsy showed calcification and benignsoft tissue fragments with no evidence of neoplasm.In J ulv 2003. excision and debulking of the masswas performed. Because of too much intermingline of’the soft tissue with the mass, a clean dissection couldnot he performed. Histological findings showed calcification’. surrounded by libro—fattv tissue and skeletalmuscle withotit es idence of neoplasm. Eleven daysafter the surger the patient was discharged in goodcond it i OiLTwenty-three days after the surgery, however, thepatient presented with fever, chills and increased painat the surgical site. Foul-smelling, purulent materialwas draining from the incision site. She was admittedand intravenous antibiotics were started. Her regularmedications were not changed. DcThridements of theright thigh were carried oul on multiple occasionsbecause of non—healing wound infection. Woundcultures a’rew Pmreitv mirabth.s and Enlemcouciisspecies. Despite vigorous treatment, her svoundnever healed. On post—operative day #67. she diedfrom sudden cardiopulmonary arrest. .-\utops\ wasnot performed. Her post-operative calcium levelshad dropped l’rom I 0.Xmg/dL (post-operative #1) to8.Smg/di. (post-operative #34L Her serum albuminlevels remained stable 2.9g/dL(post-operative day#8),32g/dL(post-operative #23). 3.2g/dL(post-operative#65).DiscussionTumoral calcinosis ) TC ii characterited h\ massivecalcium—phosphate deposits. usuall periarticularin location. The etioloev ma\ be divided into tw icateL’ories: prunarv and secondar- Primary TC i’.postulated to he an autocoinal recessive disorderSecondar\ TC. known as ureinic TC ) [TC. is wellrecognized in patients vs ith chronic kidney diseaseon dialsis.The exact pathogenesis of [TC is not fully uuderstood, The following factors are associated withUTC-’’: I tincreased calcium-phosphate product(CaxP), 2i mpaired mineralization of the hone (renalosteod stroph ) .3)secondary h\ perparathyroidism.4)aluminuin overload, 5) local factors, such as tissueCosyntropin Stimulaton TestSerum Cortisol levelsmco:dLiTablel — Findings on Workup for HynercalcemiaReset Reference RangeCa img/dU 13 8.4-10.2Phos imge’dLt 5.6 2.6-5.4Albume udL 3.5 3.5-5.0VtamnD (25-OH ngmLi <5 20-57ViraminD (1,25(OH2 pc.rnL) 9 15-75serum Aluminum rncgL;r 6 <30PTHrP (pmol/L) 0.7 <1.3intact PTH (pg/mL) <1 7-5321.3 baselinel28.5 30mim2-2427,5 (60mimSerum Ca levels from the different sitesmg!dLiRight antecubital veinRight femoral vernLeft temoral vein11.311.38.4-10.211.68.4-10.28.4-10.2Serum Protein Electrophoresis: NormalBone scan No tumor123Figures 23.— MRI showed a calcified mass with fIud filled loculations in the soft tissues of the right hipand proximal femur.injury, hematoma, infection, and injection. There arerecent cases lacking evidence of hyperparathyroidism Therefore. many authors considcr increasedCaxP to he the most iniportant predisposing factor forrecent cases of ETC.The prevalence of ETC appears to have increasedto 3 in I NU-972 1mm 1) .)i in the period of 1968-I 98ii’. Some authors speculate that this increase maxbe due to increased use of calcium—based phosphorushinders and vitamin D, and long—term use ofdialysatewith relatively high Ca concentration, all of whichcould result in positive calcium balanceUHypercalcernia is freqLientlv observed in ETC5 7As in our case. hypercalcemia in sonic ETC casescannot be attributed to conventional enologics suchas hvperparathvroidism. excess of vitamin D. ma—lignancv. or granulomatous diseases. It appears thatETC itself can cause lix pcrcalceniia independentof parathx roid hormone. or ‘ itamin D status. Thepostulated mechanism is increased release of calciumfrom UTC in the presence of adynaniic bone disease.in whichcondition mineralization of calcium intoboneis impaired . This hypothesis is supported by thefollowing facts: I) Animal models demonstrated thatcalcium resorption from soft tissuecalcificationcausessignificant lix percalcenna in parathsroidectomizedrats with normal and abnormal renal fnnction-. 2Transient increase in serum calcium and phosphate2as well as urinarx excretion ofcalcium and phosphate.during regressions ofEiC in cases on dialx sis or withrenal transplants .3) Induction of a negative calciumbalance appears to further facilitate mobilization ofcalcium from UTC 2 Interestingly, it appears that themore aggressively a negative balance of calcium isinduced. the faster ETC regresses hut also the morehkelv se\ crc temporal hx percalcemiani des elopIn retrospect. the worsened hvpercalcemia in our caseafter total parathyroidectom and introduction of lowcalcium—dialvsate max he due to the same mechanism.although so e did not obsero e any appreciable decreasein the size of ETC during the course.To our knowledge. our case is the first report in theEnglish literature to describe post-excisional improvement of hpercalcemia in ETC. Medications cannotexplain this change. because calcium—containingmedications and vitamin F) so crc discontinued duringthe hospitalitation prior to surgery, vet hvpercalce—inia persisted. In contrast, her serum calcium fellsigniticantlx afler the debulking. We cannot excludeother possibilities for this drop. ‘itch as decreased oralintake posh iperatively resulting in lower calcium andphosphorus intake, Mitigating against this possibility is the fact that her serum albumin levels had beenstable postoperatively.Treatment of UTC is not established Six casesof successt ul medical treatments so ith complete orFiaj arathyroidectomy,-2 UTC Discovered] -_1 /1V Iz’ A ‘.. 5.7‘/I /—zz- - Staw Lower Ca-contained, Dialysatete (Ca 2 OmEq!L,Oct Ci - 0i 2Fi a ow a ‘0 a r rum in r i Cof vai b w r in in I II win in in dIN II c I r I Ii 4Di d p ilfi slk:nP.124significant rctsi’ession of L’TC have been reporte&- ‘ In theseeases, combined modalities were used to induce a neoatie CaxPbalance d.c. extended diaI sis time. includin2 daily nocturnal homehemodial sis. use ol Inch-performance membrane dialvzers. use oflow calcium dialvsate in most cases. Recression of L’TC has alsobeen reported in those ss ho receis ed renal transplants. \oneof these cases has reported recun’ence of k’TC or treatment-relatedmorh di t or mortal i t\ after i he i’ecression Thus, treatment ‘a ith theaho\ e calcium—reduction reonnens appears to be sale and durablees en thouch reported ii uinhers are small.In summar. [IC. althoueh rare, is one of the potentially life—threatening complications of lon—tcrm dialysis. The prevalenceis apparently increasine. This may he related to recent therapeuticmodalities causine positive calcium balance. UTC itself may causehypercalcemia independent of parathyroid hormone. Given itslikely progression to a debilitating condition, aggressive medicaltreatment targeting negative calcium balance is recommended beforecontemplating surger. particularly when the UTC is too large forcomplete excision.ReferencesHsu SC H ,,na aJ 5un Jilt diana (AC T CC Lv’ SY T mama] c h”orn vvvi1eds,,m Isv an den ‘rc uc’ na hvre’riaa a va, mcd,akis p .tit !yeohrol Dial TransplantAU 2” 15i8i’22.12ci,a”a = F Ace a Si”astu ‘ mn-en, ai ass ‘‘ iarri 1.’— ‘aV,n ‘i sKi al,‘v-9”amo.’ ,,io —v, “v. a sri 1,.’, Nephrol Dial Tran oC,-it 1 O,i 6 2°”’u°a Km,, d”l’ Saa T°’e as, A A voLcnc’rn,, j, “ ‘ “- ‘ n sctin’ j La Am J 5idne Ds, Lla 9002 ‘ E’vTB I a .‘ co’. ad ‘ ‘‘n ‘i ‘ ‘v rni — — -, —-‘ Nepiirol DialTi’a”vplnt 1’9 ‘‘-- J ‘ ‘ O 1.-’-’ EF Ha GO S ‘- ,‘,,,“9’ Skeletal Radio! ‘d.. 1 45 n’i.UaC ‘ 02 ‘d U’ ‘,, ‘ ‘‘- a’ C/inRadio, - --n 1, k b L Kr’ 2, il C. F ,‘ ‘vi “K ‘, via ‘t “AmJKidn yDi Iv 0/ia 4 F’“by B T, r’ .1 ,4H H rn ‘(“F L “a MB a 0t n A ft r’ DJ P r a‘1ira, ‘, ‘i,ft,i,n’e a ,. r ,, r”m, Ai p 5 atm’S u nyp p r thyr i ma_I J Neal Med J ‘1’ D il’ 16 riasD Ncr’onSA c:pts,y]E Pro_ar piflgfl a ifii’tio’ioftamraa’ ma let J Deirnatol Ap’ 1 ‘9o v5’4, 2 vi /MaG, r C Ba’ ‘J L,’ a K Av, an A R pA dat, n f/urn in aft r a tr a ,J nt/i a Cliii Neptire! Jan i°Q f’( ,54 ‘8K’ iyrn S Tmo’ nH Nasa(ar-iaM Km uviYSak 0 u’ tu,tr tarn ftamr/inc usrr CAPD aamhn’-K ,‘ ‘ft ft modi (pat w’L L,w a ium da/y t Blood Pert(1)4 38C’! a F a S naba ‘a A Camp, t JM Opp nh rn iF A rn A Ur m tumor a ia y tt 0, r ‘via L”at’rra a’eodi p i 1” r py J Rh em to! F H 1999c ‘2/ 9 aSSApotli T i ne], .1 C’ri ,od”s,il,douC Fount P 04/ A Rot aion tm iv turn rii ‘f/ft i fts, 1 a, a’ a ti t ft r r trn at ilft d d turn di ly / vdma, r /ion C/in Nephrol 0 19Q8 50i4i24, 114 WaId H Y,nnoa A P000vt r MM R rption t ft a u (et,’atrnn p th’ m ft a] ‘ ofayp rca/c n-i Mine I trolyt Metab 1 86 12)2! 10(anon 0 ZI t rK S Lmor Ic (moat iou, rnr pu rep]vt”Ditl t I ClinNephrolJun 000 53(6/ 489JjsBestMedicaIcarodayJ.Jucted here in Hawai’i that willtv ofvour patient’s care.J trials appl the latest medical)rlnation to develop interventions that[1 hec ime tomorrows standard.Clinical trials cover the continuum ofpatient care, not just treatments—fri )in“Vuntion to tollow—up, and support.• Hawai’i insurers, including Medicare and HMSA, will coithe costs ofclinical trials.• The latest most acuirate information on clinical trials, jflHawai’i or around the orid, i’ in ailable b contaotin theNational Cancer IflstitMtL’s Cancet Intormation Sers ice it1 800 4 CANCER, on the web at n.s,anccr.so.Nowyou know.vU’ IHAU’IAH MED/CAL JOURNAL. 61 MAE 2001125

Improved hypercalcemia after debulking of uremic tumoral calcinosis in a parathyroidectomized patient.

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Improved hypercalcemia after debulking of uremic tumoral calcinosis in a parathyroidectomized patient.

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