
==== Front
Clin Case Rep
Clin Case Rep
10.1002/(ISSN)2050-0904
CCR3
Clinical Case Reports
2050-0904
John Wiley and Sons Inc. Hoboken

10.1002/ccr3.9236
CCR39236
CCR3-2024-03-0854-IV.R1
Nephrology
Transplantation
Chronic Diseases
General Medicine
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Case Image
Case report of tumoral calcinosis in a peritoneal dialysis patient with tertiary hyperparathyroidism and systemic lupus erythematosus
Jayanatha et al.
Jayanatha Kalpa https://orcid.org/0000-0002-8214-5021
1 2 kalpa.jayanatha@auckland.ac.nz

Idrus Irfan 3
Sapsford Mark 4
Tutone Viliami 3
Lam Michael 3
1 School of Medicine, Faculty of Medical and Health Sciences University of Auckland Auckland New Zealand
2 Middlemore Hospital, Health New Zealand Auckland New Zealand
3 Department of Renal Medicine Middlemore Hospital Auckland New Zealand
4 Department of Rheumatology Middlemore Hospital Auckland New Zealand
* Correspondence
Kalpa Jayanatha, School of Medicine, Faculty of Medical and Health Sciences, University of Auckland, Auckland, New Zealand.
Email: kalpa.jayanatha@auckland.ac.nz

09 9 2024
9 2024
12 9 10.1002/ccr3.v12.9 e923611 6 2024
28 3 2024
23 6 2024
© 2024 The Author(s). Clinical Case Reports published by John Wiley & Sons Ltd.
https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.

Key Clinical Message

Tumoral calcinosis (TC) is a rare condition characterized by dystrophic calcinosis. TC in end stage kidney disease is associated with severe hyperparathyroidism. It is radiologically characterized by multilobulated cystic calcifications in periarticular regions without erosive arthropathy or osseus destruction. Secondary TC may necessitate medical or surgical parathyroidectomy for symptom control.

Plain radiography of bilateral hands revealing innumerable periarticular soft tissue calcifications without bony erosions.

endocrinology & metabolic disorders
nephrology
radiology & Imaging
rheumatology
surgery
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cover-dateSeptember 2024
details-of-publishers-convertorConverter:WILEY_ML3GV2_TO_JATSPMC version:6.4.8 mode:remove_FC converted:09.09.2024
Jayanatha K , Idrus I , Sapsford M , Tutone V , Lam M . Case report of tumoral calcinosis in a peritoneal dialysis patient with tertiary hyperparathyroidism and systemic lupus erythematosus. Clin Case Rep. 2024;12 :e9236. doi:10.1002/ccr3.9236
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pmc1 INTRODUCTION

A 28‐year‐old female presented to hospital with impaired mobility due to severe pain in the elbows, wrists, hands, and feet. Her past medical history included end stage kidney disease (ESKD) secondary to lupus nephritis, for which she was receiving peritoneal dialysis (PD). The patient had previously received several courses of prednisone for polyarthralgia; however multiple joint aspirates had excluded crystalline and inflammatory arthropathies.

Examination revealed numerous firm, nodular swellings over the dorsal surface of the hands and wrists, and extensor surfaces of the elbows. Her C‐reactive protein was elevated (119 mg/L; normal range [NR] 0–5) with unremarkable double‐stranded DNA (7 EU; NR 0–20) and complement levels (C4 0.9 g/L; NR 0.2–0.6 and C3 0.9 g/L; NR 0.8–1.8). Her urate had been suppressed for 12 months (0.33 mmol/L; NR 0.16–0.42). She had tertiary hyperparathyroidism (PTH 105 pmol/L; NR 1.7–6.9 pmol/L) with elevated albumin‐adjusted calcium (2.60 mmol/L; NR 2.10–2.55), phosphate (2.48 mmol/L; NR 0.75–1.50), and calcium‐phosphate product (79.96 mg2/dL2; NR < 55).

Her PD clearances were acceptable (total Kt/V 1.695, nPCR 0.9 g/kg/day, creatinine clearance 40.87 L/week/1.73m2). Her bilateral hand radiographs revealed innumerable periarticular soft tissue calcifications without bony erosions (Figure 1). Dual‐energy computer tomography (CT) revealed widely distributed amorphous and “cloud‐like” calcifications consistent with tumoral calcinosis (TC) in both hands (Figure 2 and Figure 3). There was no evidence of urate deposition or tophi on CT.

FIGURE 1 Plain radography of bilateral hands.

FIGURE 2 Computer tomography (CT) scan of left hand. (A) Coronal view of left hand in soft tissue window. (B) Coronal view of left hand in bone window.

FIGURE 3 Computer tomography (CT) of right hand. (A) Coronal view of right hand in soft tissue window. (B) Coronal view of right hand in bone window.

She transitioned to alternative‐day hemodialysis (HD) with low calcium dialysate and an in‐patient total parathyroidectomy was performed with histology subsequently revealing parathyroid hyperplasia. Her post‐operative PTH was 1.6 pmol/L (NR 1.7–6.9 pmol/L), phosphate 1.99 mmol/L (NR 0.75–1.50), albumin‐adjusted calcium 2.10 mmol/L (NR 2.10–2.55), and calcium‐phosphate product 51.82 mg2/dL2 (NR <55). The patient's polyarthralgia, periarticular nodular swellings, and functional status improved post‐operatively.

2 DISCUSSION

TC is a rare condition characterized by periarticular calcific masses that cause pain and limitation of joint mobility. The calcification process is multifactorial and likely initiated by elevated calcium‐phosphate product. 1 A pathogenesis‐based classification of TC has been proposed. 2 Primary normophosphataemic TC is characterized by normal serum calcium and phosphate levels. It is associated with autosomal recessive mutations in the SAMD9 gene. 1 , 2

Primary hyperphosphataemic TC is characterized by normal serum calcium with high serum phosphate levels and it is associated with reduced urinary phosphate excretion due to autosomal recessive mutations in the FGF23, GALNT3, or KLOTHO genes. 1 , 2 Secondary TC is characterized by severe hyperparathyroidism with elevated calcium‐phosphate product in ESKD. 1 , 2

Irregular, multilobulated, and cystic calcifications located in periarticular regions are typical findings on plain radiographs. 1 , 2 Erosive arthropathy and osseus destruction by these calcified masses are typically absent. 2 Treatment strategies should be based on the subtype. The treatment for secondary TC includes dietary modification, minimisation of calcium‐based phosphate binders and intensification of HD with low calcium dialysate to optimize serum calcium‐phosphate product. 2 , 3

Parathyroidectomy should be considered for severe secondary TC. 1 , 3 Early parathyroidectomy, high preoperative serum alkaline phosphatase levels, and low postoperative serum phosphate levels are associated with remission of soft tissue masses. 3 Surgical excision of soft tissue masses is not recommended due to the high risk of postoperative complications and the high probability of recurrence. 2

AUTHOR CONTRIBUTIONS

Kalpa Jayanatha: Conceptualization; methodology; project administration; supervision; visualization; writing – review and editing. Irfan Idrus: Writing – original draft. Mark Sapsford: Supervision; writing – review and editing. Viliami Tutone: Supervision; writing – review and editing. Michael Lam: Supervision; writing – review and editing.

CONFLICT OF INTEREST STATEMENT

None declared.

FUNDING INFORMATION

No funding was received for this manuscript.

CONSENT

The authors confirm that the patient has provided signed written consent for the publication and dissemination of this case report.

ACKNOWLEDGMENTS

We would like to thank the patient for kindly providing written consent for the publication of this case report. Open access publishing facilitated by The University of Auckland, as part of the Wiley ‐ The University of Auckland agreement via the Council of Australian University Librarians.

DATA AVAILABILITY STATEMENT

Data sharing is not applicable to this article as no new data were created or analyzed in this study.
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REFERENCES

1 Fathi I . Review of tumoral calcinosis: a rare clinico‐pathological entity. World J Clin Cases. 2014;2 :409‐414. doi:10.12998/wjcc.v2.i9.409 25232542
2 Smack DP , Norton SA , Fitzpatrick JE . Proposal for a pathogenesis‐based classification of tumoral calcinosis. Int J Dermatol. 1996;35 :265‐271. doi:10.1111/j.1365-4362.1996.tb02999.x 8786184
3 Wang J , Zeng M , Yang G , et al. Effects of parathyroidectomy on tumoral calcinosis in uremic patients with secondary hyperparathyroidism. BMC Surg. 2019;19 :133. doi:10.1186/s12893-019-0603-8 31510980
