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Brazilian Journal of Nephrology

Print version ISSN 0101-2800On-line version ISSN 2175-8239

J. Bras. Nefrol. vol.39 no.2 São Paulo Apr./June 2017

http://dx.doi.org/10.5935/0101-2800.20170037 

Case Report

Reversal of uremic tumoral calcinosis by optimization of clinical treatment of bone and mineral metabolism disorder

Mariana Espiga Maioli1  2 

Vinicius Daher Alvares Delfino1  2 

Amanda Carolina Damasceno Zanuto Guerra1  2 

Luiz Fernando Kunii1  2 

Raquel Ferreira Nassar Frange2 

1Universidade Estadual de Londrina (UEL).

2Instituto do Rim de Londrina.

Abstract

Tumoral calcinosis is an uncommon type of extraosseous calcification characterized by large rubbery or cystic masses containing calcium-phosphate deposits. The condition prevails in the periarticular tissue with preservation of osteoarticular structures. Elevated calcium-phosphorus products and severe secondary hyperparathyroidism are present in most patients with uremic tumoral calcionosis (UTC). Case report of an obese secondary to chronic glomerulonephritis, undergoing continuous ambulatory peritoneal dialysis (CAPD) reported the appearance of painless tumors in the medial surface of fifth finger and left arm. Tumoral calcinosis was confirmed by left biceps biopsy. Poor adherence to CAPD. The patient was transferred to the "tidal" modality of peritoneal dialysis and after was treated by hemodialysis, despite the persistence of severe hyperparathyroidism progressive reduction of UTC until near to its complete disappearance. Nowadays, one year after patient received deceased-donor kidney transplantation, he presents with an improvement in secondary hyperparathyroidism. UTC should be included in the elucidation of periarticular calcification of every patient on dialysis. Relevant laboratory findings such as secondary hyperparathyroidism and elevated calcium- phosphorus products in the presence of periarticular calcification should draw attention to the diagnosis of UTC.

Keywords: calcium metabolism disorders; nephrology; phosphorus metabolism disorders

Introduction

Tumoral calcinosis is an uncommon type of extraosseous calcification characterized by large rubbery or cystic masses containing calcium-phosphate deposits.1 The condition prevails in the periarticular tissue with preservation of osteoarticular structures. Genetic causes and metabolic disorders are associated with their appearance.2 The tumoral calcinosis that occurs in chronic kidney disease (CKD) patients is known for uremic tumoral calcinosis (UTC).

In this condition, calcifications can also contain hydroxyapatite (Ca5 (PO4) 3OH).1,3 Although, most often described in hemodialysis (HD) patients (0.5- 1.2% prevalence),2 UTC may also occur in patients undergoing peritoneal dialysis. Elevated calcium-phosphorus products and severe secondary hyperparathyroidism are present in most patients with UTC.4

Common sites of involvement are the blood vessels, periarticular region, heart, lungs, kidneys, gastric mucosa, central nervous system, breast and eyes. Clinical treatment for UTC includes dietary phosphorus restriction, non-calcium phosphate binders, calcimimetics, optimal control of hyperparathyroidism, and intensive hemodialysis with low calcium dialysate. Surgical excision of tumoral calcinosis, parathyroidectomy, and kidney transplantation are recommended for persistent or refractory UTC.1,5

Case report

An obese (Body Mass Index 38.5) young white man of 22 years with CKD secondary to chronic glomerulonephritis, undergoing continuous ambulatory peritoneal dialysis (CAPD) for uremia management, reported, after 6 months of treatment, the appearance of painless tumors in the medial surface of fifth finger and left arm (biceps region).

Laboratory results: serum calcium 8.5 mg/dL (reference: 8.4 to 10.2 mg/dL), serum albumin 3.6 mg/dL (reference: 3.4 to 5.2 mg/dL), serum phosphorus 11.1 mg/dL (reference: 2.5 to 4.5 mg/dL), and intact PTH (iPTH) 1867 pg/mL (reference: 15 to 65 pg/ml), serum uric acid 8.7 mg/dL (reference: 3.6 to 7.7 mg/dL).

Computed tomography of the left arm and radiography of the right hand (Figure. 1) showed periarticular, irregular and multilobular calcifications. Tumoral calcinosis was confirmed by left biceps biopsy: lesion characterized by the presence of abundant giant cells scattered throughout the proliferation of fibroblasts, with associated areas of hemorrhage and extensive calcifications. Ultrasonography of anterior cervical region showed no nodules in the four parathyroid glands.

Figure 1 Right hand X-ray: Multiple calcifications around the phalanges and the phalangeal-metacarpal transition of 5th right hand finger. 

Questioned, the patient admitted poor adherence to CAPD (with 3.5 mEq/L calcium concentration in dialysate), sometimes doing only one or two exchanges a day. The patient was transferred to the "tidal" modality of peritoneal dialysis in order to increase the efficiency of uremia treatment, while waiting for AVF maturation for starting HD.

With the "tidal" modality, there was an improvement in laboratory levels of bone metabolism: serum calcium 8.0 mg/dL, serum phosphorus 7.8 mg /dL and iPTH of 300.5 pg/mL. After a short stay in "tidal" CAPD, the patient was treated for 2.5 years by HD (three sessions per week, four hours per session, with 3.0 mEq/L calcium in the dialysis bath). During this period, he made use of sevelamer, calcitriol and calcium carbonate (the latter two when calcium x phosphorus product allowed it).

After 3 months on hemodialysis, it was observed, despite the persistence of severe hyperparathyroidism (calcium 8.9 mg/dL, P 7.6 mg/dL and PTH 1840 pg/mL), progressive reduction of UTC until near to its complete disappearance (Figure. 2). In November 2014, the patient received deceased-donor kidney transplantation (immunosuppression: tacrolimus, mycophenolate mofetil, and prednisone).

Figure 2 Right hand X-ray after 4 months of the transference of the patient to hemodialysis showing markedly reduction of UTC. 

Nowadays, three months after surgery, he presents with a serum creatinine of 0.9 mg/dL and calcium, phosphorus and iPTH values of 9.4 mg/dL; 1.7 mg/dL and 368.8 pg/mL, respectively. Since there was improvement in hyperparathyroidism by normalization of renal function, no hypercalcemia, moderate hypophosphatemia, thus was vitamin D nutritional supplemented by low serum.

Since no abnormal parathyroid glands was identified at their usual position at ultrasound, it was planned to observe for a few months more the possibility of reversal the secondary hyperparathyroidism, as suggested in the Literature,6 instead considering parathyroidectomy as a first approach of treatment.6

Discussion

UTC should be included in the elucidation of periarticular calcification of every patient on dialysis. The diagnosis is often not done due to its rarity and the difficulty of clinical recognition. In patients on peritoneal dialysis, there is a report of adherence to dialysis, as exemplified in this case.5

Relevant laboratory findings such as secondary hyperparathyroidism and elevated calcium-phosphorus products in the presence of periarticular calcification should draw attention to the diagnosis of UTC. Treatment involves dialysis improvement, and appropriate control of calcium and phosphorus. Parathyroidectomy and kidney transplantation may be required for its management.

References

1 Chu HY, Chu P, Lin YF, Chou HK, Lin SH. Uremic tumoral calcinosis in patients on peritoneal dialysis: clinical, radiologic, and laboratory features. Perit Dial Int 2011;31:430-9. DOI: http://dx.doi.org/10.3747/pdi.2009.00250Links ]

2 Olsen KM, Chew FS. Tumoral calcinosis: pearls, polemics, and alternative possibilities. Radiographics 2006;26:871-85. PMID: 16702460 DOI: http://dx.doi.org/10.1148/rg.263055099Links ]

3 Floege J. When man turns to stone: extraosseous calcification in uremic patients. Kidney Int 2004;65:2447-62. DOI: http://dx.doi.org/10.1111/j.1523-1755.2004.00664.xLinks ]

4 Carvalho M, de Menezes IA, Riella MC. Massive, painful tumoral calcinosis in a long-term hemodialysis patient. Hemodial Int 2011;15:577-80. DOI: http://dx.doi.org/10.1111/j.1542-4758.2011.00581.xLinks ]

5 Raju DL, Podymow T, Barre P. Tumoral calcinosis in a peritoneal dialysis patient. Kidney Int 2006;70:1887. DOI: http://dx.doi.org/10.1038/sj.ki.5001752Links ]

6 Triponez F, Clark OH, Vanrenthergem Y, Evenepoel P. Surgical treatment of persistent hyperparathyroidism after renal transplantation. Ann Surg 2008;248:18-30. PMID: 18580203 DOI: http://dx.doi.org/10.1097/SLA.0b013e3181728a2dLinks ]

Received: July 01, 2016; Accepted: August 30, 2016

Correspondence to: Mariana Espiga Maioli. Instituto do Rim de Londrina. Rua Engenheiro Omar Rupp, nº 100, Jardim Ipiranga, Londrina, PR, Brazil. CEP: 86015-360, E-mail: mariana.espigamaioli@gmail.com

Creative Commons License This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.