A case of tumoral calcinosis in hemodialysis

Abstract

Dear Editor, Tumoral calcinosis is a rare syndrome which presents as painless, firm, tumor-like masses around the joints leading to pain, and limitation of joint movements. Commonly affected joints are shoulders, elbows, and hip joints. Spinal, temporomandibular joint, metacarpals, and metatarsal involvement have also been described. A 50-year-old female on twice weekly maintenance hemodialysis for the last 3 years presented with complaints of pain and restriction of movements at right hip joint and knee. On examination, range of movements was restricted at the hip joint. On palpation, there were subcutaneous hard nodules in the right hip region. Plain radiographic images of the pelvis were obtained and they showed irregular periarticular masses [Figure 1a]. Plain radiographs of hand, skull, and shoulder joins showed similar lesions [refer to adjoining Figure 1b-d]. On metabolic work, she had parathyroid hormone level of 162 pg/mL, phosphorus of 8.5 mg/dL, calcium of 9.8 mg/dL, albumin of 3.5 g/dL, and Vitamin D level of 29 ng/mL. She was poorly compliant with dietary restrictions and taking phosphate-binding medications. Diagnosis of secondary tumoral calcinosis was made. The patient was managed with phosphate-restricted diet, phosphate-binding medication, and increasing the dialysis frequency from twice weekly to thrice weekly.Figure 1: X-ray images showing (a) irregular peri articular masses around the hip joint. Similar findings were seen in X-ray images of (b) hand (c) skull and (d) shoulder joints as wellTeutschlaender initially described this disease in his studies in 1930’s and it came to be known as Teutschlaender disease.[1] The term tumoral calcinosis was coined by Inclan in 1943.[2] Smack et al. retrospectively studied 122 cases of tumoral calcinosis and proposed a classification system.[3] They classified it as (1) primary normophosphatemic type, (2) primary hyperphosphatemic type, and (3) secondary tumoral calcinosis. Secondary causes include renal insufficiency, hyperparathyroidism, or hypervitaminosis D, with renal insufficiency being the most common identifiable condition. Even though soft-tissue calcifications are common in hemodialysis patients, the prevalence of periarticular masses is low. Most of cases in chronic kidney disease are thought to occur due to the presence of hyperparathyroidism although it may occur in its absence as well. The calcification process results from elevated calcium–phosphorus product with hyperphosphatemia being the most important component. Diagnosis is based on characteristic imaging findings. Imaging typically shows amorphous, cystic, and multi-lobulated deposits in periarticular areas.[4] Computed tomography may show cystic loculi with fluid levels giving rise to sedimentation signs. Treatment of secondary tumoral calcinosis in the setting of renal failure is predominantly medical therapy. Calcium- and phosphate-restricted diets and phosphate binders are used as first-line management. Intravenous pamidronate is also shown to be effective.[5] Partial or total parathyroidectomy can be considered in refractory cases in case hyperparathyroidism is the cause.[6] Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initial will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.