|Year : 2014 | Volume
| Issue : 3 | Page : 172-175
Idiopathic tumoral calcinosis
Sarang Rathod, Neha Jindal, Gurjit Singh, Iqbal Ali
Department of General Surgery, Padmashree Dr. Dnyandeo Yashwantrao Patil Hospital and Research Centre, Pimpri, Pune, Maharashtra, India
|Date of Web Publication||8-Dec-2014|
Dr. Neha Jindal
Department of Surgery, Padmashree Dr. Dnyandeo Yashwantrao Patil Hospital and Research Centre, Sant Tukaram Nagar, Pimpri, Pune - 411 018, Maharashtra
Source of Support: None, Conflict of Interest: None
Tumoral calcinosis is a disease characterized by peri-articular deposition of calcium phosphate mostly around major joints of the body. A 60-year-old female was suffering from painful swelling over the left greater trochanteric area for past 4 months. No similar lesion was detected on any other part of the body. Thorough biochemical and radiological work up was done followed by excision of swelling. Pathology revealed Tumoral calcinosis. Patient is being followed up regularly for any recurrence.
Keywords: Phosphatemia, tumoral calcinosis, trochanter
|How to cite this article:|
Rathod S, Jindal N, Singh G, Ali I. Idiopathic tumoral calcinosis. Arch Int Surg 2014;4:172-5
| Introduction|| |
Tumoral calcinosis is an uncommon condition resulting in deposition of calcium in soft tissue especially around large joints. It has been found in patients in Africa but rarely reported from other countries. 
This condition was described by Giard in 1898, Duret in 1899, and Teutschländer in 1935 but the term "Tumoral calcinosis" was first coined by Inclan in 1943.  It does not show any abnormalities in calcium metabolism. Its etiology remains uncertain. However, its diagnosis and treatment is simple provided surgeon is familiar with this condition. Boulman and colleagues classified soft-tissue calcifications into 5 categories: Metastatic, tumoral, dystrophic, calciphylaxis, and idiopathic. 
Metastatic calcification has an abnormality in calcium metabolism and occurs as firm, subcutaneous nodules near large joints. These nodules involve normal tissues and are associated with conditions such as hyperparathyroidism, milk-alkali syndrome, and hypervitaminosis D.
Tumoral calcification is a rare familial disorder in which there is increased phosphorus and normal to decreased calcium levels. It occurs as large, subcutaneous calcium deposits near joints or pressure areas.
Dystrophic calcification includes calcinosis and occurs in the presence of normal metabolism as subcutaneous nodules, plaques, or extensive deposits in tissues "damaged" by trauma, infection, lupus, scleroderma, or dermatomyositis.
Calciphylaxis is found in patients with chronic renal failure along with abnormalities in serum calcium phosphate levels. Small-vessel vasculopathy with intimal fibrosis and thrombosis leads to tissue ischemia and necrosis.
Idiopathic calcification usually occurs in the presence of normal calcium metabolism as asymptomatic, subcutaneous nodules in healthy individuals.
Another study suggests that, pathological process of calcification in tumoral calcinosis can be further divided into dystrophic, metastatic, idiopathic, and iatrogenic types. 
Smack DP et al. have divided Tumoral calcinosis into following three subtypes: 
- Primary normo-phosphatemictumoral calcinosis: In patients with normal serum phosphate and serum calcium levels and no evidence of disorders previously associated with soft tissue calcification,
- Primary hyper-phosphatemic tumoral calcinosis: In patients with an elevated serum phosphate level, a normal serum calcium level, and no evidence of disorders previously associated with soft tissue calcification; and
- Secondary tumoral calcinosis: In patients with a concurrent disease capable of causing multiple soft tissue calcification and familial occurrence.
Minor repeated trauma and tissue injury seem to play a role in the calcifying process; it probably serves as a trigger mechanism that leads to a chain of events, beginning with hemorrhage, fat necrosis, fibrosis, collagenisation, and ending with collagenolysis and ultimately massive calcification.  Localized tumors even though multiple in a healthy patient skeleton and complete absence of any other abnormality is the deciding factor in tumoral calcinosis. The autosomal dominant mode of transmission is the accepted mode of inheritance; however, autosomal recessive mutation especially to genes GALNT3  and FGF23  have been identified as the mode of transmission in recent discoveries.
Other study suggests that familial forms of tumoral calcinosis are linked to mutations of various genes that include GALNT3, FGF-23, and Klotho.This study also concluded that Tumoral calcinosis can occur in patients without positive familial history.  Sokolov et al. have recommended molecular genetic analysis of GALNT3 and FGF23 gene for exact diagnosis and genetic counseling of patients in familial tumoral calcinosis. 
Pathogenesis of the Tumoral calcinosis lesions has been explained by three theories which are as follows:
- Repetitive trauma leading to reparative dysfunction,
- Peri-articular forces dissecting histiocytic aggregates that initiate osteoclastic activity, and
- Hemorrhage from micro trauma, causing an exaggerated reparative response. 
Both elbows and buttock regions were site of involvement in an 11-year-old girl  whereas in our patient, the hip was the site of involvement.
We report a case of tumoral calcinosis in an elderly female where plain radiograph and histopathology clinched final diagnosis.
| Case Report|| |
A 60-year-old female presented with the history of a hard swelling on the left greater trochanter for past 4 months. The swelling was initially 1 cm in diameter, which gradually increased in size and became painful during the course of time. There was no history of trauma over the site of swelling or similar illness in other members of the family. No similar swelling was detected in any other part of the body. Local examination revealed a solitary well outlined, hard, and mobile swelling of size 6 cm × 4 cm over the left greater trochanter. All movements of left hip joint were full and painless. Serum calcium, phosphorus, alkaline phosphatase, and uric acid levels were within normal limits. Anti-nuclear antibodies were not detected. Radiograph revealed small linear clusters of soft tissue calcification over the left greater trochanter [Figure 1]. Ultrasound examination of the swelling revealed lesion in subcutaneous tissue with calcific specks, suggestive of tumoral calcinosis. On Fine Needle Aspiration Cytology, diagnosis of tumoral calcinosis was suspected.
|Figure 1: The arrow showing small linear clusters of soft tissue calcifi cation over the left greater trochanter in plain radiograph|
Click here to view
The mass, which was entirely in the subcutaneous plane, was excised completely, and the wound healed by primary intention. Histopathological report of the excised tissue revealed that dermis had prominent fibrosis and there were basophilic deposits [Figure 2] of calcium forming islands in the dermis. No inflammation/granulomas were seen. The diagnosis was confirmed as tumoral calcinosis. There was no recurrence of swelling after 6 months of follow-up.
|Figure 2: The arrow showing basophilic deposits of calcium forming islands in the dermis|
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| Discussion|| |
Tumoral calcinosis is a distinct but rare entity in which there is deposition of calcium in peri-articular soft tissue. In the idiopathic form, tumoral calcinosis occurs mainly in the first three decades of life  whereas in our case, the patient was into the sixth decade of her life. It has been reported in a child as young as 4 years old.  However, in this case, serum calcium levels were normal but levels of serum phosphorus and alkaline phosphatase were raised. Our case falls in the idiopathic category since serum calcium and serum phosphorus levels were normal. Hence, it can be grouped under subtype primary normophosphatemic tumoral calcinosis. An aberrant tissue response to local trauma may be a cause of this type of tumoral calcinosis. 
In a retrospective study in two institutions over a period of 10 years, amongst 9 patients, the most affected site was the elbow, with the hip coming at second position.  Other conditions such as secondary calcinosis, calcinosis universalis, calcinosis circumscripta, soft tissue chondroma, pseudogout, and calcareous tendinitis need to be differentiated from tumoral calcinosis. Plain radiographs in tumoral calcinosis are often diagnostic, showing multiple areas of well-circumscribed, nodular masses with fibrous septae, giving a "cobblestone" or "chicken-wire" appearance. Films exposed with a horizontal beam may show the "sedimentation sign" due to mineral portion pooling dependently, creating a fluid calcium level.  In our patient, plain radiograph of the region showed small clusters of soft tissue calcification over the left greater trochanter [Figure 1].
Other imaging modalities such as CT Scan, MRI, and bone scintigraphy are undertaken in specific clinical settings in which radiologists play a critical role in selecting appropriate tests that can result in conclusive diagnosis. 
Microscopically, tumoral calcinosis is divided into two histological types, active and inactive stages. The active stage is more frequent and is easy to identify.  In the active stage, a large central calcified granular area is present, bordered by an inflammatory infiltrate with predominant epithelioid histiocytes mixed with several lymphocytes and scattered giant multinuclear macrophages. The cellular infiltrate is limited by thick fibrous septa. Inflammatory infiltrate is absent in the Inactive stage. Only calcified deposits and dense collagenous tissue are observed. In our case, basophilic deposits of calcium were seen in dermis with no granular areas placing it into inacative type. Histopathology shows calcium deposits in the dermis with or without surrounding giant cell reaction.  In our patient, basophilic deposits of calcium were seen in the dermis with no inflammatory granuloma forming response [Figure 2].
Symptomatic treatment is the natural choice, as the cause of the disease is unknown. Only one case of spontaneous regression has been noted.  Medical treatment with use of calcitonin, diphosphonates, steroids, phenylbutazone, and radiation therapy have proved to be unsuccessful.  Complete surgical excision of tumoral calcinosis is considered to be the optimum treatment. The recommended management for tumoral calcinosis is surgical excision. Complete excision of mass is required to prevent recurrence. Medical treatment using agents that decrease serum phosphate levels have limited use in the management of tumoral calcinosis. Alternative management techniques using steroid and radiation therapy were proposed but they did not consistently prevent recurrence.  Hence, surgical treatment is the only curative approach.
Spontaneous regression does not occur. Indications for surgical removal of skin lesions are pain, recurrent infection, ulceration, and functional impairment. It is important to exclude other causes of calcinosis after evaluating with biochemical parameters and imaging studies. Pain was the indication for surgery in our case. Patient was followed up for 6 months and there was no recurrence.
| Conclusion|| |
Tumoral calcinosis is a rare entity. Hence, the surgeon must be aware of this possibility. It needs thorough evaluation to exclude other causes of soft tissue calcification. Once the diagnosis is established, complete surgical excision is the only option for cure and prevention of recurrence.
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[Figure 1], [Figure 2]